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MORFEA / ESCLERODERMIA LINEAL
HISTORIA:
La palabra morfea viene viene del griego "morphe" que significa "apariencia o "forma", termino que se utiliza en la literatura médica para referirse a condiciones de piel caracterizada por endurecimiento o fibrosis, el cual se produce por un aumento de la síntesis de colágeno, el cual se acumula en la piel, provocando la patología. Algunas escuelas dermatológicas la denominan también esclerodermia localizada.
Pero realmente existe una gran diferencia entre la MORFEA y la ESCLERODERMIA LOCALIZADA y es que en la MORFEA CLÁSICA, solo hay afectación de la piel, no hay afectación de órganos internos, tampoco se encuentran en la mayoría de los casos anticuerpos antinucleares, ni afectación sistémica, detalles que explicaré mas adelante.
El término "MORFEA se hizo popular en el siglo XIX por medio del médico Addison quien describió una monografía titulada "Keloid Scleriasis, Morphoea" en 1854. (Morfea, Esclerosis y Queloide). A partir de ese año comenzó a utilizarse el término "MORFEA" para referirse a las lesiones de piel localizadas y caracterizadas por induración y/ o fibrosis cutánea, y que como dije algunos la consideran una variante de la esclerodermia sin afectación de órganos internos.
La confusión se presta porque hay una variante de la MORFEA llamada MORFEA PROFUNDA la cual cual si tiene compromiso de órganos internos y esta si esta considerada un variante de la ESCLERODERMIA LOCALIZADA.
DEFINICIÓN, TIPOS Y CARACTERÍSTICAS:
La morfea, también conocida como esclerodermia localizada, es un trastorno inflamatorio crónico del tejido conectivo que se caracteriza por fibrosis de la piel y el tejido subcutáneo. La etiología no se conoce por completo, pero se cree que implica predisposición genética, desregulación vascular y desequilibrios del sistema inmunológico, con posibles desencadenantes ambientales.
La morfea, también conocida como esclerodermia localizada, es un trastorno inflamatorio crónico del tejido conectivo que se caracteriza por fibrosis de la piel y el tejido subcutáneo. La etiología no se conoce por completo, factores probablemente involucrados en esta patología: predisposición genética, factores ambientales, y alteraciones del sistema inmunológico.
MORFEA CLÁSICA:
La morfea se presenta con mayor frecuencia en los niños y adolescentes pero también puede presentarse en la edad adulta. La presentación clínica mas frecuente es la morfea lineal caracterizada por endurecimiento de la piel y placas hiperpigmentadas de color pardo o marfil de aparición progresiva, por los general sin otra sintomatología.
Para algunas escuelas dermatológicas la MORFEA como tal se diferencia de la esclerodermia localizada, porque NO TIENE SÍNTOMAS SISTÉMICOS, y no hay anticuerpos antinucleares ni anticuerpos específicos de la esclerodermia como el anti SCL70, y otros.
MORFEA PROFUNDA:
De modo que cuando hay manifestaciones EXTRA CUTÁNEAS que incluyen: artritis, síntomas neurológicos y complicaciones oftalmológicas que se presentan en hasta el 20% de los pacientes, o la llamada MORFEA PROFUNDA que es una variante de esta enfermedad donde hay compromiso de la fascia, el músculo e incluso el hueso, estas estarían incluidas en el espectro de las ESCLERODERMIAS LOCALIZADAS.
Un estudio ha destacado los distintos perfiles de expresión genética en la morfea en comparación con la esclerosis sistémica, haciendo hincapié en el papel de la desregulación inmunitaria de los linfocitos T, esto apoya la ESCUELA dermatológica que afirma lo anteriormente expuesto.
MORFEA GENERALIZADA: Las placas de morfea pueden ocupar gran parte del cuerpo con síntomas mucocutáneos, pero por lo general no hay síntomas sistémicos, o anticuerpos típicos de la esclerodermia:
La MORFEA también puede presentar síntomas mucocutáneos siendo las lesiones orales más comunes en pacientes más jóvenes con morfea lineal, y las lesiones genitales más frecuentes en mujeres mayores postmenopáusicas con morfea generalizada. Aquí hay que repetir que, si hay síntomas EXTRACUTANEOS con anticuerpos anti SCL70 estaríamos en presencia de ESCLERODERMIA LOCALIZADA o GENERALIZADA.
Con el descubrimiento de la BORRELIA BURGDORFERI, comenzaron a aparecer algunos estudios donde en lesiones de MORFEA se encontraron anticuerpos específicos para Borrelia, incluso en los CÓDIGOS ICD11 para enfermedad de LYME (ocasionada por esta bacteria), la MORFEA aparece en ese espectro, pero esta situación es un debate total hoy día, pues hay estudios donde NO SE HA ENCONTRADO relación entre MORFEA y BORRELIA BURGDORFERI, sobre todo en estados Unidos, y otros estudios predominantemente en EUROPA donde si se han reportado haber encontrado ADN específico de Borrelia en lesiones de MORFEA, gran de tema en discusión hoy día.
TRATAMIENTOS:
El tratamiento de esta patología es un reto dermatológico, y lo que se busca con ellos es mejorar la elasticidad de la piel, reducir el endurecimiento cutáneo, y mejorar la estética fundamentalmente, empleando para ello:
A.-Tratamientos tópicos: Humectantes, calcipotriene, tacrolimus, fórmulas magistrales a base de urea, ácido salicílico y Dimetilsulfóxido (DMSO), láser y fototerapia.
B.-Tratamientos Sistémicos: Corticosteroides y metotrexato en casos inflamatorios, Micofenolato de Mofetilo, Ciclosporina,y antibióticos donde se ha demostrado relación con Borrelia burgdorferi. Entre los nuevos tratamientos están fármacos biológicos como abatacept, un anticuerpo de fusión contra CTLA-4, o tocilizumab, un anticuerpo totalmente humanizado contra IL-6R, en el tratamiento de esta patología.
C.-Tratamientos complementarios: En casos de desfiguración o atrofia facial, se ha utilizado relleno con ácido hialurónico e inyecciones de grasa autóloga para mejorar los resultados estéticos.
D.-La clásica vitamina E: Casi ni mencionada en los estudios ha demostrado ser MUY ÚTIL tanto en la MORFEA como LA ESCLERODERMIA.
Aquí encuentras el enlace donde se relaciona la ESCLERODERMIA LOCALIZADA con BORRELIA BURGDORFERI LA ENFERMEDAD DE LYME CLASIFICACIÓN Y CÓDIGOS 2019 (CLICK)
Saludos,,,
Dr. José Lapenta.
ENGLISH
HISTORY:
The word morphea comes from the Greek "morphe" meaning "appearance" or "form", a term used in medical literature to refer to skin conditions characterized by thickening or fibrosis, which is produced by an increase in collagen synthesis, which accumulates in the skin, causing the pathology. Some dermatological schools also call it localized scleroderma.
But there is really a big difference between MORPHEA and LOCALIZED SCLERODERMA and that is: CLASSIC MORPHEA, only the skin is affected, there is no affectation of internal organs, nor are antinuclear antibodies found in most cases, nor is there systemic affectation, details that I will explain later.
The term "MORPHEA" became popular in the 19th century through the doctor Addison who described a monograph entitled "Keloid Scleriasis, Morphoea" in 1854. (Morphea, Sclerosis and Keloid). From that year on, the term "MORPHEA" began to be used to refer to localized skin lesions characterized by induration and/or cutaneous fibrosis, and which, as I said, some consider it a variant of scleroderma without involvement of internal organs.
The confusion arises because there is a variant of MORPHEA called DEEP OR MORPHEA PROFUNDA which does involve internal organs and is considered a variant of LOCALIZED SCLERODERMA.
DEFINITION, TYPES AND CHARACTERISTICS:
Morphea, also known as localized scleroderma, is a chronic inflammatory disorder of the connective tissue characterized by fibrosis of the skin and subcutaneous tissue. The etiology is not fully understood, but it is believed to involve genetic predisposition, vascular dysregulation, and immune system imbalances, with possible environmental triggers.
Morphea, also known as localized scleroderma, is a chronic inflammatory disorder of the connective tissue characterized by fibrosis of the skin and subcutaneous tissue. The etiology is not fully understood, with genetic predisposition, environmental factors, and immune system disorders probably involved in this pathology.
CLASSICAL MORPHEA:
Morphea occurs most frequently in children and adolescents, but can also occur in adulthood. The most frequent clinical presentation is linear morphea, characterized by thickening of the skin and hyperpigmented brown or ivory-colored plaques that appear progressively, usually without other symptoms.
For some dermatological schools, MORPHEA as such is different from localized scleroderma, because it DOES NOT HAVE SYSTEMIC SYMPTOMS, and there are no antinuclear antibodies or specific antibodies for scleroderma such as anti SCL70, an others.
DEEP OR MORPHEA PROFUNDA:
Therefore, when there are EXTRA CUTANEOUS manifestations that include: arthritis, neurological symptoms and ophthalmological complications that occur in up to 20% of patients, or the so-called DEEP MORPHEA, which is a variant of this disease where there is involvement of the fascia, muscle and even bone, these would be included in the spectrum of LOCALIZED SCLERODERMA.
A study has highlighted the different gene expression profiles in morphea compared to systemic sclerosis, emphasizing the role of immune dysregulation of T lymphocytes, this supports the dermatological SCHOOL that states the above.
GENERALIZED MORPHEA: Morphea plaques may occupy a large part of the body with mucocutaneous symptoms, but there are usually no systemic symptoms, or antibodies typical of scleroderma:
With the discovery of BORRELIA BURGDORFERI, some studies began to appear where specific antibodies for Borrelia were found in MORPHEA lesions, even in the ICD11 CODES for LYME disease (caused by this bacteria), MORPHEA appears in that spectrum, but this situation is a total debate today, since there are studies where NO relationship has been found between MORPHEA and BORRELIA BURGDORFERI, especially in the United States, and other studies predominantly in EUROPE where specific Borrelia DNA has been reported to have been found in MORPHEA lesions, a major topic of discussion today.
TREATMENTS:
The treatment of this pathology is a dermatological challenge, and what is sought with them is to improve the elasticity of the skin, reduce skin hardening, and improve aesthetics fundamentally, using for this:
A.--Topical treatments: Moisturizers, calcipotriene, tacrolimus, master formulas based on urea, salicylic acid and Dimethyl sulfoxide (DMSO), laser and phototherapy.
B.-Systemic treatments: Corticosteroids and methotrexate in inflammatory cases, Mycophenolate Mofetil, Cyclosporine, and antibiotics where a relationship with Borrelia burgdorferi has been demonstrated. Among the new treatments are biological drugs such as abatacept, a fusion antibody against CTLA-4, or tocilizumab, a fully humanized antibody against IL-6R, in the treatment of this pathology.
S.-Complementary treatments: In cases of facial disfigurement or atrophy, hyaluronic acid fillers and autologous fat injections have been used to improve aesthetic results.
D.-The classic vitamin E: hardly mentioned in studies, has proven to be VERY USEFUL in both MORPHEA and SCLERODERMA.
Here you will find the link where LOCALIZED SCLERODERMA is related to BORRELIA BURGDORFERI THE LYME DISEASE CLASSIFICATIONS AND CODES 2019 (CLICK)
Greetings...
Dr. José Lapenta R.
EDITORIAL ESPANOL:
====================
Hola amigos DERMAGICOS, de nuevo con ustedes. La MORFEA enfermedad del colágeno y tema interesante, pues los científicos discuten actualmente su relación con la Borrelia, ademas de ello de tratamiento controversial y difícil. Para algunas escuelas considerada como una variante de esclerodermia, denominada esclerodermia localizada, para otras no. Estas 47 referencias nos ilustran bien el tema.
Próxima edición: IMPLANTES MAMARIOS, LA PIEL Y LA SALUD.
...Existe una verdadera relación entre los implantes mamarios, las colagenosis y otras enfermedades ????
Saludos,,,
Dr. José Lapenta R.,,,
EDITORIAL ENGLISH:
===================
Hello DERMAGICS friends, again with you. The MORFEA a connective tissue disease, and interesting topic, because today the scientists discusses their relationship with the Borrelia, besides it of controversial and difficult treatment. For some schools considered as a variant of scleroderma called also localized scleroderma, for other not. These 47 references illustrate us well the topic.
Next edition: BREAST IMPLANTS, THE SKIN AND THE HEALTH.
..Is there a true relationship among the breast implants, the connective tissues diseases and other illnesses ????
Greetings,,,
Dr. José Lapenta R.
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DERMAGIC/EXPRESS(46)
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LA MORFEA / THE MORPHEA
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1.) Clinical characteristics associated with antihistone antibodies in patients with localized scleroderma.
2.) Extracorporeal photochemotherapy in systemic sclerosis and severe morphea.
3.) Bullous morphea: clinical, pathologic, and immunopathologic evaluation of thirteen cases.
4.) Classification of morphea (localized scleroderma) [see comments]
5.) No evidence for Borrelia burgdorferi infection in lesions of morphea and lichen sclerosus et atrophicus in Spain. A prospective study and literature review.
6.) Histological comparison of morphea and lichen sclerosus et atrophicus.
7.) The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993.
8.) Detection of Borrelia burgdorferi DNA (B garinii or B afzelii) in morphea and lichen sclerosus et atrophicus tissues of German and Japanese but not of US patients.
9.) Bullous morphea: a distinct entity?
10.) Role of Borrelia burgdorferi in the pathogenesis of morphea/scleroderma and lichen sclerosus et atrophicus: a PCR study of thirty-five cases.
11.) Antigen specificity of antihistone antibodies in localized scleroderma.
12.) Oral calcitriol as a new therapeutic modality for generalized morphea [see comments]
13.) Decreased expression of the human progenitor cell antigen (CD34) in morphea.
14.) Localized scleroderma.
15.) Soluble CD4 and CD8 in serum from patients with localized scleroderma.
16.) Immunocytochemical localization and serologic detection of transforming growth factor beta 1. Association with type I procollagen and inflammatory cell markers in diffuse and limited systemic sclerosis, morphea, and Raynaud's phenomenon.
17.) Postradiation morphea.
18.) Generalized morphea with vascular involvement. A case report and disaccharide analysis of the skin glycosaminoglycans.
19.) Topical calcipotriene for morphea/linear scleroderma.
20.) [Exposure to solvents in scleroderma: disseminated circumscribed scleroderma (morphea) in a painter exposed to perchloroethylene]
21.) Antinuclear antibodies in children with localized scleroderma.
22.) Self-involuting atrophoderma of the lateral-upper arm. A new benign variant of morphea?
23.) Localized forms of scleroderma, including morphea, linear scleroderma, and eosinophilic fasciitis.
24.) Low-dose methotrexate in the treatment of widespread morphea.
25.) Coexistence of generalized morphea with hisotological changes in lichen sclerosus et atrophicus and lichen planus.
26.) Antihistone antibodies in scleroderma.
27.) 'Swiss cheese' lymphangiectasia in a case of solitary morphea profunda.
28.) Neurologic abnormalities in two patients with facial hemiatrophy and sclerosis coexisting with morphea.
29.) [Deep morphea-type lesions, first manifestations of lymphocytic lymphoma]
30.) Response to methotrexate in a patient with idiopathic eosinophilic fasciitis, morphea, IgM hypergammaglobulinemia, and renal involvement.
31.) Morphea after silicone gel breast implantation for cosmetic reasons in an HLA-B8, DR3-positive woman.
32.) Autoantibodies to mitochondrial 2-oxo-acid dehydrogenase complexes in localized scleroderma.
33.) Association of eosinophilic fasciitis, multiple morphea and antiphospholipid antibody.
34.) Internal involvement in localized scleroderma.
35.) Mucin deposits in morphea and systemic scleroderma.
36.) Classification and epidemiology of scleroderma.
39.) A Systematic Review of Morphea Treatments and Therapeutic Algorithm.
40.) Morphea: The 2023 Update.
41.) Update on Morphea: Part II. Outcome Measures and Treatment.
42.) Diagnosis and Management of Morphoea in Children: An Overview.
47.) Morphea and Eosinophilic Fasciitis: An Update.
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1.) Clinical characteristics associated with antihistone antibodies in patients with localized scleroderma.
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Author
Sato S; Fujimoto M; Ihn H; Kikuchi K; Takehara K
Address
Department of Dermatology, Faculty of Medicine, University of Tokyo, Japan.
Source
J Am Acad Dermatol, 31(4):567-71 1994 Oct
Abstract
BACKGROUND: Recently we demonstrated the presence of antihistone antibodies (AHA) in localized scleroderma. OBJECTIVE: Our purpose was to determine clinical characteristics associated with AHA in patients with localized scleroderma. METHODS: We examined 57 serum samples by an enzyme-linked immunosorbent assay in the following three subgroups: 15 patients with generalized morphea, 27 with linear scleroderma, and 15 with morphea. We classified the patients as having generalized morphea when they had four or more lesions on at least two areas of the body, irrespective of whether the lesions were of morphea or linear type. RESULTS: AHA were detected in 42% of patients with localized scleroderma (24 of 57), and in 87% of patients with generalized morphea (13 of 15). The presence of AHA strongly correlated with the number of morphea lesions, the total number of lesions, and the number of involved areas of the body. However, AHA did not correlate with the presence or number of linear lesions. The presence of AHA showed a 87% sensitivity (13 of 15 patients) and a 74% specificity (31 of 42 patients) for generalized morphea. CONCLUSION: Our data suggest that AHA are a serologic marker for generalized morphea and that the validity of our new classification for generalized morphea is supported by the high frequency of AHA detection.
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2.) Extracorporeal photochemotherapy in systemic sclerosis and severe morphea.
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Author
Cribier B; Faradji T; Le Coz C; Oberling F; Grosshans E
Address
Clinique Dermatologique, H^opitaux Universitaires, Strasbourg, France.
Source
Dermatology, 191(1):25-31 1995
Abstract
BACKGROUND: Extracorporeal photochemotherapy (EP) is a new immune-modulating therapy which combines cytapheresis and extracorporeal irradiation of leukocytes by UVA. Recently, patients with systemic sclerosis (SS) have been treated by this method, but the efficacy of EP is still controversial. OBJECTIVE: Our purpose was to evaluate the efficacy and safety of EP in SS and in severe morphea. METHODS: In an open study, 7 patients with SS and 2 patients with severe morphea were treated by EP and were evaluated after 6 months of treatment. No other treatments were allowed during this period. RESULTS: Except one vasovagal reaction, EP was well tolerated in all patients. The surface of the cutaneous sclerosis was unchanged in 3 of the 7 patients with SS, whereas it was aggravated in the remaining 4 patients. There was no improvement of the visceral involvement of SS in these patients. Although the duration of SS was less than 4 years in 6 out of 7 patients, we did not observe any significant benefit from EP in SS patients. One patient with generalized morphea had stopped EP after 3 months because of loss of vascular access and could not be evaluated. In the last patient with linear morphea of the upper limbs, a reduction in the number of morphea plaques was observed, and the remaining lesions were less visible after 16 months of EP. CONCLUSIONS: The present results do not corroborate those previously published with regard to the efficiency of EP in SS of recent onset. The efficacy of ECP in morphea needs further confirmation.
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3.) Bullous morphea: clinical, pathologic, and immunopathologic evaluation of thirteen cases.
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Author
Daoud MS; Su WP; Leiferman KM; Perniciaro C
Address
Department of Dermatology, Mayo Clinic, Jacksonville, FL 32224.
Source
J Am Acad Dermatol, 30(6):937-43 1994 Jun
Abstract
BACKGROUND: Bullous morphea is a rare disease. Its pathogenesis is unknown. OBJECTIVE: We evaluated bullous morphea clinically, pathologically, and immunopathologically and investigated the role of spirochetes and eosinophils in its pathogenesis. METHODS: The clinical and pathologic findings from 13 patients with bullous morphea were reviewed. Tissue sections were studied with the Elias-Bosma stain for spirochetes and indirect immunofluorescence for eosinophil granule major basic protein. RESULTS: Bullae were found in all forms of morphea; the lower extremities were the most common sites of involvement. Lymphatic dilatation was found in 77% of the patients. Deposition of major basic protein was found in 60% of cases studied. There was no evidence of spirochetes in any of the specimens examined with the Elias-Bosma stain. CONCLUSION: Our results suggest that the pathogenesis of bullous morphea is related to lymphatic dilatation as well as release of major basic protein from eosinophils in some patients. We found no association between spirochetes and bullous morphea.
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4.) Classification of morphea (localized scleroderma) [see comments]
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Author
Peterson LS; Nelson AM; Su WP
Address
Division of Rheumatology and Internal Medicine, Mayo Clinic Rochester, MN 55905, USA.
Source
Mayo Clin Proc, 70(11):1068-76 1995 Nov
Abstract
OBJECTIVE: To classify and describe morphea (localized scleroderma). DESIGN: A review of morphea and its subtypes is presented. RESULTS: The current classification of morphea is incomplete and confusing. As knowledge of the spectrum of disease continues to evolve, the controversy and confusing nature of its multiple subtypes present a challenge for the physician who encounters a patient with this condition. Thus, we propose that morphea be classified into the following five groups: plaque, generalized, bullous, linear, and deep. This classification, based on clinical morphologic findings, will simplify the diagnostic and therapeutic approach. CONCLUSION: Morphea represents a wide variety of clinical entities that seen to be on the opposite end of the scleroderma spectrum from systemic sclerosis. The cutaneous lesions eventually evolve from a sclerotic stage to a nonindurated stage, and residual hypopigmentation or hyperpigmentation follows. The histologic pattern in patients with morphea is similar to that in patients with progressive systemic sclerosis. Although treatment is nonstandardized, hydroxychloroquine sulfate may be beneficial.
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5.) No evidence for Borrelia burgdorferi infection in lesions of morphea and lichen sclerosus et atrophicus in Spain. A prospective study and literature review.
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Author
Alonso-Llamazares J; Persing DH; Anda P; Gibson LE; Rutledge BJ; Iglesias L
Address
Department of Dermatology, Mayo Clinic and Mayo Foundation, Rochester, MN 55905, USA.
Source
Acta Derm Venereol, 77(4):299-304 1997 Jul
Abstract
The possible association of Borrelia burgdorferi with morphea and lichen sclerosus et atrophicus has been the focus of research and discussion in dermatology during the last 10 years. To investigate the etiopathogenic role of B. burgdorferi in morphea and lichen sclerosus et atrophicus lesions in Spain, we studied 14 cases: 8 patients with lichen sclerosus et atrophicus and 6 with morphea. For the whole group, a prospective study was performed, including serologic studies by indirect immunofluorescence, histologic evaluation of skin biopsy specimens, culture studies, and polymerase chain reaction with different primers sensitive for detecting virtually all B. burgdorferi strains tested to date. Although one patient with morphea had positive serologic findings at low titer, we were not able to culture or detect borrelial DNA in any of the specimens. These findings do not confirm an association between B. burgdorferi and morphea and lichen sclerosus et atrophicus.
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6.) Histological comparison of morphea and lichen sclerosus et atrophicus.
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Author
Nishioka S
Address
Department of Dermatology, Kurume University School of Medicine, Japan.
Source
Kurume Med J, 44(2):83-90 1997
Abstract
Although lichen sclerosus et atrophicus at an early stage and morphea can be differentiated clinically and histologically, both diseases at a late stage present diagnostic difficulties. In this study, collagen and acid glycosaminoglycans in the skin of both morphea and lichen sclerosus et atrophicus were examined by polarization microscopy. Picrosirius red and Toluidine blue (pH 5.8) were used as stains. Under polarized light, the picrosirius red-stained collagen fibers appeared green in the papillary and reticular dermis of morphea, except the fibers immediately below the epidermis, which appeared orange yellow. In lichen sclerosus et atrophicus, the collagen fibers appeared green in the reticular dermis at both early and late stage. In the papillary dermis the fibers appeared orange at an early stage and greenish orange at a late stage. Toluidine blue-stained birefringence in morphea diminished in the presence of MgCl2 at 0.2 M, in lichen sclerosus et atrophicus in the presence of MgCl2 at 0.3 M. Histologically, there were some differences in epidermal changes between the two diseases; the epidermis was thick in morphea and thin in lichen sclerosus et atrophicus. This difference seems to relate to that in the fibers beneath the
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7.) The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993.
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Author
Peterson LS; Nelson AM; Su WP; Mason T; O'Fallon WM; Gabriel SE
Address
Department of Health Sciences Research, Mayo Clinic, Rochester, MN 55905, USA.
Source
J Rheumatol, 24(1):73-80 1997 Jan
Abstract
OBJECTIVE: To determine the incidence, prevalence, survival rates, clinical manifestations, and longterm outcome of patients with morphea (localized scleroderma) and its subtypes over a 33 year period in Olmsted County, Minnesota. METHODS: We used the unique data resources of the Rochester Epidemiology Project to review all Olmsted County medical records with any potential diagnosis consistent with morphea (including plaque, generalized, bullous, linear, and deep entities) from 1960 through 1993. RESULTS: We screened 1030 medical records and identified 82 (59 female; 23 male) cases of morphea first diagnosed between 1960 and 1993. All cases were followed until death or migration from Olmsted County, a total of 754 person-years of observation. The annual age and sex adjusted incidence rate per 100,000 population was 2.7 (95% confidence interval 2.1, 3.3). The incidence rate increased significantly over the 33 years (p = 0.0037) on an average of 3.6% per year. The prevalence (estimated using cumulative incidence) at 80 years of age was about 2/1000. 50% of the patients had a cutaneous softening or evidence of disease resolution by 3.8 years' duration. The shortest active disease duration was found in the plaque group (50% resolution or skin softening by 2.7 years) compared to 5.5 years in the deep group. Arthralgias, synovitis, uveitis, and joint contractures were more frequent in the linear and deep categories. Although 9 patients (11%) developed some disease related disability over the followup period, this was common (44%) in the deep group. No case of morphea developed severe internal organ involvement and none progressed to systemic sclerosis. The survival rate was not significantly different from the general population (p = 0.409). CONCLUSION: Morphea, and its subtypes, are more common than previously recognized, and can lead to important disability.
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8.) Detection of Borrelia burgdorferi DNA (B garinii or B afzelii) in morphea and lichen sclerosus et atrophicus tissues of German and Japanese but not of US patients.
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Author
Fujiwara H; Fujiwara K; Hashimoto K; Mehregan AH; Schaumburg-Lever G; Lange R; Schempp C; Gollnick H
Address
Department of Dermatology, Wayne State University, Detroit, Mich, USA.
Source
Arch Dermatol, 133(1):41-4 1997 Jan
Abstract
OBJECTIVE: To elucidate the geographic and genospecific association of Borrelia with morphea and lichen sclerosus et atrophicus (LSA). DESIGN: The association of Borrelia burgdorferi with morphea and LSA has been reported, but is still controversial. We conducted a retrospective survey of Borrelia DNA in skin biopsy specimens. SETTINGS: The samples were collected from the outpatient clinic of university hospitals and a dermatopathology laboratory. PATIENTS: Skin biopsy specimens (19 morphea and 34 LSA) were obtained from patients in the United States, Japan, and Germany. DNA samples were subjected to amplification with polymerase chain reaction for B burgdorferi flagellin gene, and for the genotype-specific detection of B burgdorferi sensu stricto, Borrelia garinii, and Borrelia afzelii. RESULTS: Five cases of morphea and 2 cases of LSA in Germany and Japan yielded positive signals for B garinii or B afzelii, the European species. None of the American samples were positive for Borrelia polymerase chain reaction. Borrelia burgdorferi sensu stricto was not detected in any of the specimens. CONCLUSION: Morphea and LSA in Germany and Japan can be related with European genotypes of Borrelia.
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9.) Bullous morphea: a distinct entity?
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Author
Trattner A; David M; Sandbank M
Address
Department of Dermatology, Beilinson Medical Center, Petah Tiqva, Israel.
Source
Am J Dermatopathol, 16(4):414-7 1994 Aug
Abstract
A 57-year-old woman who had undergone lumpectomy for infiltrating duct-cell carcinoma of the right breast was found to have morphea after receiving radiation therapy. Three years later she developed a vesicular eruption on the area of the morphea, which was diagnosed as bullous morphea. The histologic findings of the vesicular component were characteristic of lichen sclerosus et atrophicus. It is suggested that in some cases bullous morphea represents a secondary appearance of bullous lichen sclerosus et atrophicus on a lesion of morphea.
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10.) Role of Borrelia burgdorferi in the pathogenesis of morphea/scleroderma and lichen sclerosus et atrophicus: a PCR study of thirty-five cases.
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Author
De Vito JR; Merogi AJ; Vo T; Boh EE; Fung HK; Freeman SM; Cockerell C; Stewart K; Marrogi AJ
Address
Department of Dermatology, Talane University School of Medicine, New Orleans, Louisiana 70112, USA.
Source
J Cutan Pathol, 23(4):350-8 1996 Aug
Abstract
Morphea (localized scleroderma), and lichen sclerosus et atrophicus (LSA) share common features with acrodermatitis chronica atrophicans (ACA), a known chronic form of borreliosis. These include similar histologic findings such as diffuse dermal fibrosis. These observations have led several investigators to consider the possibility of Borrelia burgdorferi (Bb) as a common etiologic factor among all of these diseases. The aim of this study is to investigate the role of Bb in the pathogenesis of morphea and LSA, by assaying for its presence in lesional skin biopsies from patients with these diseases. We utilized the nested polymerase chain reaction (PCR) technique to selectively amplify a longer segment of a Bb-specific somatic gene, on DNA from paraffin-embedded, formalin-fixed tissues. The results revealed no Bb-specific DNA sequence in 28 specimens of morphea/scleroderma and 7 of LSA with varying stages of disease. Furthermore, confirmatory Southern blot of the PCR product, resulted in similar findings. These data seriously question the role played by this spirochete in the pathogenesis of morphea and LSA, at least in the southeastern part of the USA.
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11.) Antigen specificity of antihistone antibodies in localized scleroderma.
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Author
Sato S; Fujimoto M; Ihn H; Kikuchi K; Takehara K
Address
Department of Dermatology, Faculty of Medicine, University of Tokyo, Japan.
Source
Arch Dermatol, 130(10):1273-7 1994 Oct
Abstract
BACKGROUND AND DESIGN: Recently, we detected antihistone antibodies (AHAs) in patients with localized scleroderma. However, the exact antigen specificity of AHAs in this disease is still unknown. Therefore, we determined the reactivity of AHAs with five individual histones and the correlation of AHAs with rheumatoid factor in localized scleroderma by means of enzyme-linked immunosorbent assay. Twenty patients with localized scleroderma who had IgG and/or IgM AHAs, as determined by enzyme-linked immunosorbent assay, were examined. These patients were classified into the following three subgroups: patients with generalized morphea (n = 11), patients with linear scleroderma (n = 6), and patients with morphea (n = 3). RESULTS: In generalized morphea, IgG AHAs strongly reacted with histones H1, H2A, and H2B; and IgM AHAs strongly reacted with H1 and H2B, as determined by means of enzyme-linked immunosorbent assay. The pattern of reactivity in linear scleroderma and morphea was similar to that in generalized morphea. A homogeneous immunofluorescent pattern on HEp-2 cells, which was produced by localized scleroderma sera, was completely abolished by absorption with total histones. By employing a latex agglutination test, IgM rheumatoid factor was detected in 60% of the 20 patients with localized scleroderma and at a frequency of 82% in those with generalized morphea. However, an absorption test of rheumatoid factor activity with human IgG revealed no cross-reactivity of AHAs with rheumatoid factor. CONCLUSIONS: Our data suggest that AHAs in localized scleroderma are directed against native chromatin, since H1, H2A, and H2B occupy a relatively exposed portion of chromatin.
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12.) Oral calcitriol as a new therapeutic modality for generalized morphea [see comments]
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Author
Hulshof MM; Pavel S; Breedveld FC; Dijkmans BA; Vermeer BJ
Address
Department of Dermatology, University Hospital Leiden, The Netherlands.
Source
Arch Dermatol, 130(10):1290-3 1994 Oct
Abstract
BACKGROUND: None of the commonly used drugs for the treatment of scleroderma appears to significantly influence the fibrotic stage of this disorder. Recently, a beneficial effect of the treatment with oral calcitriol (1,25 dihydroxyvitamin D3) in 10 patients with systemic sclerosis and four patients with morphea was described. This fact could be ascribed to the immunoregulatory effects of calcitriol observed in vitro and to inhibition of fibroblast growth. We treated three patients with extensive morphea with remarkable results. OBSERVATION: Three patients with generalized morphea were treated with calcitriol in an oral daily dose of 0.50 to 0.75 microgram. After 3 to 7 months of treatment, the mobility of the joints improved and the skin extensibility increased. No adverse effects were observed. The improvement persisted after discontinuation of therapy during a follow-up period of 1 to 2 years. CONCLUSION: Calcitriol showed a beneficial effect in generalized morphea during an open study. Double-blind, placebo-controlled trials are needed to assess its therapeutic value.
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13.) Decreased expression of the human progenitor cell antigen (CD34) in morphea.
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Author
Skobieranda K; Helm KF
Address
Department of Medicine, Pennsylvania State University College of Medicine, Milton S. Hershey Medical Center, Hershey 17033, USA.
Source
Am J Dermatopathol, 17(5):471-5 1995 Oct
Abstract
Morphea is an idiopathic disorder the hallmark of which is fibrosis of the skin. The regulatory factors resulting in the increased collagen production have not been determined. Within the dermis there are dendritic cells with possible immunological functions that express either the human progenitor cell antigen (CD34) or factor XIIIa. Immunohistochemical stains for factor XIIIa, CD34, S100, proliferating nuclear cell antigen, and vimentin were performed on 26 skin biopsies from patients with morphea and 11 biopsies from normal skin. A decreased number of CD34-expressing cells was seen in the affected skin from morphea patients, while there was an increased number of cells expressing factor XIIIa and vimentin. We hypothesize that CD34-positive dendritic cells may have a regulatory role in collagen synthesis and that lack of CD34 expression can be used as a marker for morphea.
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14.) Localized scleroderma.
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Author
Tuffanelli DL
Address
University of California, San Francisco 94108, USA.
Source
Semin Cutan Med Surg, 17(1):27-33 1998 Mar
Abstract
Localized scleroderma can be divided into three main subtypes: morphea, linear scleroderma, and generalized morphea. Plaque morphea usually has a good prognosis. Variants of morphea, including guttate morphea and atrophoderma of Pasini and Pierini, are seen. Linear scleroderma, whether involving an extremity or the face, is often associated with serological abnormalities. Cosmetic and functional prognosis may be poor. Therapy is usually ineffective. Generalized morphea may be difficult to differentiate from systemic scleroderma. However, progression to systemic scleroderma
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15.) Soluble CD4 and CD8 in serum from patients with localized scleroderma.
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Author
Sato S; Fujimoto M; Kikuchi K; Ihn H; Tamaki K; Takehara K
Address
Department of Dermatology, Faculty of Medicine, University of Tokyo, Japan.
Source
Arch Dermatol Res, 288(7):358-62 1996 Jun
Abstract
Localized scleroderma has been shown to be accompanied by various immunologic abnormalities. To obtain functional information on activated CD4+ or CD8+ T cells, we studied the levels of soluble CD4 (sCD4) and soluble CD8 (sCD8) in serum from patients with localized scleroderma. Serum samples were examined by enzyme-linked immunosorbent assay. The samples were obtained from 49 patients in the following three subgroups: 15 patients with generalized morphea, 22 with linear scleroderma, and 12 with morphea. The levels of sCD4 and sCD8 were significantly elevated in patients with generalized morphea. Furthermore, these patients showed significantly higher levels of sCD4 than those with systemic sclerosis (SSc). The frequency of positivity for IgG anti-single-stranded DNA (ssDNA) antibody was significantly higher in localized scleroderma patients with elevated sCD4 levels than in patients with normal sCD4 levels. The frequency of positivity for antinuclear antibodies, IgM antihistone antibodies, IgG anti-ssDNA antibody and rheumatoid factor, and elevated sCD23 levels were significantly higher in localized scleroderma patients with elevated sCD8 levels than in patients with normal sCD8 levels. Our findings suggest that both CD4+ and CD8+ T cells are activated in vivo in generalized morphea and that the immunologic events in generalized morphea are different from those in SSc.
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16.) Immunocytochemical localization and serologic detection of transforming growth factor beta 1. Association with type I procollagen and inflammatory cell markers in diffuse and limited systemic sclerosis, morphea, and Raynaud's phenomenon.
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Author
Higley H; Persichitte K; Chu S; Waegell W; Vancheeswaran R; Black C
Address
Celtrix Pharmaceuticals, Santa Clara, California.
Source
Arthritis Rheum, 37(2):278-88 1994 Feb
Abstract
OBJECTIVE. To determine the presence of transforming growth factor beta 1 (TGF beta 1) and inflammatory cell markers (HLA-DR and Factor XIIIa) and to compare these with the presence of type I procollagen, in clinically uninvolved and involved skin from patients with different subsets of systemic sclerosis (SSc), and to analyze circulating levels of TGF beta 1 in SSc patients. METHODS. TGF beta 1, HLA-DR, Factor XIIIa, and type I procollagen were detected in skin biopsy sections using a biotin-streptavidin-peroxidase system. Levels of circulating TGF beta 1 were measured using a capture enzyme-linked immunosorbent assay technique. RESULTS. Patients with active diffuse cutaneous SSc (dcSSc) showed minimal TGF beta 1 but significant type I procollagen staining in involved skin, while the clinically uninvolved skin of these patients showed moderate extracellular and intra-epidermal TGF beta 1 immunoreactivity. Patients with limited cutaneous SSc (lcSSc) showed elevated TGF beta 1 staining in both involved and uninvolved skin, as well as procollagen staining. Significant TGF beta 1 reactivity, HLA-DR and Factor XIIIa immunoreactivity, numerous inflammatory cells, and procollagen staining were seen in specimens from patients with morphea. Sequential biopsies suggested the presence of cytokine activity at the earliest stages of disease, which was not maintained with progression of sclerosis. Among the disease groups studied, elevated levels of circulating TGF beta 1 were seen only in patients with morphea. CONCLUSION. The pattern of TGF beta 1 staining in dermal sections from patients with dcSSc, lcSSc, and morphea suggests that this cytokine is important in the pathogenesis of scleroderma. Furthermore, the presence of TGF beta 1 prior to the onset of fibrosis indicates an early involvement of this growth factor, possibly in the inflammatory stage of the disease.
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17.) Postradiation morphea.
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Author
Gollob MH; Dekoven JG; Bell MJ; Assaad D; Rao J
Address
Department of Medicine, Sunnybrook Health Science Centre, Toronto, Ontario, Canada.
Source
J Rheumatol, 25(11):2267-9 1998 Nov
Abstract
We describe a 54-year-old woman who developed right breast morphea after radiotherapy following a lumpectomy for infiltrating lobular carcinoma. Skin biopsy confirmed the histological features of morphea. Treatment was initiated with both topical and intralesional steroid, resulting in marked improvement. Morphea following radiotherapy is an infrequently recognized phenomenon. However, when diagnosed early it may be effectively managed with local steroid treatment.
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18.) Generalized morphea with vascular involvement. A case report and disaccharide analysis of the skin glycosaminoglycans.
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Author
Akimoto S; Ishikawa O; Yokoyama Y; Amano H; Miyachi Y
Address
Department of Dermatology, Gunma University, School of Medicine, Maebashi, Japan.
Source
Acta Derm Venereol, 76(2):141-3 1996 Mar
Abstract
We report a 69-year-old man with severe generalized morphea, who showed over 80% of skin involvement, while the internal organs were not affected. We performed histological examinations and analysis of skin disaccharides constituting chondroitinase-digestible glycosaminoglycans in the center and periphery of the sclerotic lesions and the clinically uninvolved skin. In both the central and peripheral parts of the sclerotic lesions, sclerotic fibrosis and a dense perivascular cell infiltration, consistent with morphea, were seen in the entire dermis and subcutis. Furthermore, various vascular changes were observed, such as endothelial cell swell, thickened basement membrane and obstruction of vascular lumen in the fat lobules. In the clinically uninvolved skin, interstitial edema was prominent along with a slight perivascular cell infiltration. On disaccharide analysis, the increase in the amount of delta Di-4S(DS), the main disaccharide unit of dermatan sulphate, delta Di-6S and delta Di-6S, the main disaccharide units of chondroitin sulphate, and the decrease in delta Di-HA, which is derived from hyaluronate, were found not only in the sclerotic lesions but also in the clinically uninvolved skin, though less prominent. These alterations were consistent with systemic sclerosis, suggesting a close relationship between severe forms of generalized morphea and systemic sclerosis.
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19.) Topical calcipotriene for morphea/linear scleroderma.
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Author
Cunningham BB; Landells ID; Langman C; Sailer DE; Paller AS
Address
Department of Pediatrics, Northwestern University Medical School, Chicago, Illinois, USA.
Source
J Am Acad Dermatol, 39(2 Pt 1):211-5 1998 Aug
Abstract
BACKGROUND: Morphea and linear scleroderma are characterized by erythema, induration, telangiectasia, and dyspigmentation. There is no universally effective treatment. Oral calcitriol has been beneficial in the treatment of localized and extensive morphea/scleroderma, but the use of topical calcipotriene has not been reported. OBJECTIVE: The purpose of this study was to evaluate the efficacy and safety of topical calcipotriene 0.005% ointment in the treatment of localized scleroderma. METHODS: In a 3-month open-label study, 12 patients aged 12 to 38 years with biopsy-documented active morphea or linear scleroderma applied calcipotriene ointment under occlusion twice daily to plaques for 3 months. The condition of each patient had previously failed to respond to potent topical corticosteroids and, for some patients, systemic medications. Efficacy was assessed at baseline, 1 month, and 3 months. Levels of serum ionized calcium, intact parathyroid hormone, and 1,25-dihydroxyvitamin D and of random urinary calcium excretion were measured. RESULTS: During the 3-month trial, the condition of all 12 patients showed statistically significant improvement in all studied features. No adverse effects were reported or detected through laboratory monitoring of mineral metabolism. CONCLUSION: Topical calcipotriene 0.005% ointment may be an effective treatment for localized scleroderma, but double-blind placebo controlled studies are needed for confirmation.
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20.) [Exposure to solvents in scleroderma: disseminated circumscribed scleroderma (morphea) in a painter exposed to perchloroethylene]
=======================================================================
Author
Hinnen U; Schmid-Grendelmeier P; M¨uller E; Elsner P
Address
Dermatologische Klinik des Universit¨atsspitals Z¨urich.
Source
Schweiz Med Wochenschr, 125(50):2433-7 1995 Dec 16
Abstract
We report on a 45-year-old painter who developed morphea-like scleroderma. He had been exposed to perchlorethylene for one year while operating a metal degreaser; the perchlorethylene concentration in the workplace ranged between 10 and 25 ppm. Whereas systemic scleroderma has frequently been attributed to solvent exposure in the past, this is only the third description of a morphea-like scleroderma suspected to be related to occupational contact with organic solvents.
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21.) Antinuclear antibodies in children with localized scleroderma.
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Author
Rosenberg AM; Uziel Y; Krafchik BR; Hauta SA; Prokopchuk PA; Silverman ED; Laxer RM
Address
Department of Pediatrics, University of Saskatchewan, Saskatoon, Canada.
Source
J Rheumatol, 22(12):2337-43 1995 Dec
Abstract
OBJECTIVE: To determine the prevalence and antigenic specificities of antinuclear antibodies (ANA) in children with localized scleroderma. METHODS: The ANA profiles of 27 children with localized scleroderma were determined. The study group comprised 21 children with linear scleroderma, 5 with morphea, and 1 with combined linear scleroderma and morphea. Sera were evaluated for the presence of ANA by indirect immunofluorescence and for reactivity with specific nuclear antigens by ELISA and immunoblotting. RESULTS: Seventeen patients (63%) had positive tests for ANA. Of these sera 10 displayed a finely speckled pattern, 5 a combined nucleolar and finely speckled nuclear pattern, and 2 a nucleolar pattern only. Fourteen of 21 (67%) with linear scleroderma were ANA positive. Three of 5 patients with morphea (60%) had ANA. The 1 patient with both linear scleroderma and morphea was ANA negative. Fifteen sera (56%) contained antibodies to denatured DNA (dDNA). Eleven sera (41%) had antibodies to one or more high mobility group (HMG) proteins, 4 (15%) reacted with one or more histones and 1 serum (4%) reacted with topoisomerase I (Sc1-70). CONCLUSION: ANA are present in most children with localized scleroderma and frequently have specificity for dDNA and HMG proteins. Children with localized scleroderma, like patients with systemic sclerosis (SSc), commonly have ANA and antibodies to dDNA. Unlike patients with SSc, however, childhood localized scleroderma is uncommonly associated with antibodies to certain specific nuclear and nucleolar constituents that typically occur in association with SSc.
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22.) Self-involuting atrophoderma of the lateral-upper arm. A new benign variant of morphea?
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Author
Inazumi T; Kawashima J; Tajima S; Nishikawa T
Address
Department of Dermatology, Keio University School of Medicine, Shinjuku, Tokyo, Japan.
Source
Dermatology, 194(2):147-50 1997
Abstract
BACKGROUND: Atrophoderma of Pasini-Pierini and morphea are considered to be distinct clinical entities. However, some authors say that they are closely related diseases. We encountered 5 unique cases that did not fit the criteria for both diseases. OBJECTIVE AND METHODS: We report 5 cases with solitary atrophic lesions on the lateral-upper arm. They appeared as white to erythematous, non-indurated and slightly depressed lesions with a smooth surface, and the patients had no history of trauma or injection. RESULTS: Histological examinations showed slight to moderate lower dermal fibrosis without specific changes in adipose tissue. The lesions disappeared spontaneously within a year. CONCLUSION: Our cases may be a benign variant form of morphea. We propose the term 'self-involuting atrophoderma of the lateral-upper arm (SALA)' for these clinical features.
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23.) Localized forms of scleroderma, including morphea, linear scleroderma, and eosinophilic fasciitis.
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Author
Nelson AM
Address
Mayo Clinic, Department of Internal Medicine, Rochester, MN 55905, USA.
Source
Curr Opin Rheumatol, 8(5):473-6 1996 Sep
Abstract
Under the term localized scleroderma a spectrum of conditions is classified, ranging from localized plaques of morphea of cosmetic importance only, to deep lesions of linear scleroderma and eosinophilic fasciitis, which can result in considerable morbidity. The etiology is unknown; environmental, infectious, and autoimmune causes have been proposed. In the past year, a revised classification of morphea has been presented. Additional information relating to pathogenesis, laboratory studies, and associated manifestations is reviewed.
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24.) Low-dose methotrexate in the treatment of widespread morphea.
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Author
Seyger MM; van den Hoogen FH; de Boo T; de Jong EM
Address
Department of Dermatology, University Hospital Nijmegen, The Netherlands.
Source
J Am Acad Dermatol, 39(2 Pt 1):220-5 1998 Aug
Abstract
BACKGROUND: Low-dose methotrexate (MTX) has been shown to be effective in the treatment of systemic sclerosis. OBJECTIVE: We evaluated the effect of low-dose MTX on widespread morphea in a 24-week trial. METHODS: Oral MTX, 15 mg/week, was administered to nine patients. Clinical records (modified skin score [MSS], durometer score, and the scores on a visual analogue scale (VAS) of feelings of tightness and itching), as well as laboratory data were examined. Serum aminoterminal propeptide of type III procollagen (PIIINP) was determined at weeks 0, 12, and 24. RESULTS: At the end of the 24-week treatment period, significant improvement was observed in MSS (P=.01) and the VAS score for tightness (P < .01), whereas the durometer score (P=.07) and the VAS for itching (P=.07) showed a tendency toward improvement. PIIINP level did not alter. No serious adverse events were noted. CONCLUSION: These results suggest a beneficial effect of MTX on widespread morphea. Because spontaneous improvements are not uncommon, prospective double-blind, placebo-controlled studies are necessary to determine the usefulness of MTX in this disease.
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25.) Coexistence of generalized morphea with hisotological changes in lichen sclerosus et atrophicus and lichen planus.
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Author
Sawamura D; Yaguchi T; Hashimoto I; Nomura K; Konta R; Umeki K
Address
Department of Dermatology, Hirosaki University School of Medicine, Japan.
Source
J Dermatol, 25(6):409-11 1998 Jun
Abstract
We reported a 44-year-old Japanese woman with generalized multiple sclerotic plaques, which showed histological findings of morphea. This patient also had an erosive lesion on her mouth; its histological findings were consistent with lichen planus. A sclerotic lesion on her thigh showed the histological findings of lichen sclerosus et atrophicus (LSA). These data suggest that similar etiologic events or closely related pathologic processes are involved in morphea, lichen planus, and LSA.
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26.) Antihistone antibodies in scleroderma.
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Author
Parodi A; Drosera M; Barbieri L; Rebora A
Address
Department of Dermatology, University of Genoa, Italy.
Source
Dermatology, 191(1):16-8 1995
Abstract
BACKGROUND: Antihistone antibodies (AHA) are usually considered the serological marker of drug-induced lupus erythematosus, but recently they have been found in patients with systemic scleroderma (SSc) and morphea. OBJECTIVE AND METHODS: We determined AHA in 43 patients with SSc, 4 patients with overlap syndrome and 11 with morphea. RESULTS AND CONCLUSIONS: AHA were demonstrated in 41.8% of the SSc patients and in 36.3% of the morphea patients. Only 1 patient with overlap syndrome had AHA. Our SSc patients with AHA had frequent cardiac and renal involvements suggesting a prognostic value of AHA. In our morphea patients AHA did not correlate with clinical features.
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27.) 'Swiss cheese' lymphangiectasia in a case of solitary morphea profunda.
=======================================================================
Author
Ahn SK; Won JH; Lee SH; Lee IW; Lee WS; Choi EH
Address
Department of Dermatology, Yonsei University Wonju College of Medicine, Wonju, Korea.
Source
J Dermatol, 24(10):666-9 1997 Oct
Abstract
Reports of lymphangiectases that occur in lesions of morphea are rare. We describe a 24-year-old woman with a solitary morphea profunda associated with lymphangiectasia. Unlike previously reported cases, our case showed lymphatic dilatation resembling Swiss cheese and developed around a milium.
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28.) Neurologic abnormalities in two patients with facial hemiatrophy and sclerosis coexisting with morphea.
=======================================================================
Author
Menni S; Marzano AV; Passoni E
Address
Institute of Dermatological Sciences, University of Milan, IRCCS Ospedale Maggiore, Italy.
Source
Pediatr Dermatol, 14(2):113-6 1997 Mar-Apr
Abstract
Progressive facial hemiatrophy or Parry-Romberg syndrome is a rare entity characterized by unilateral atrophy of the skin, subcutaneous tissue, and the underlying bony structures. This syndrome has many features of linear scleroderma en coup de sabre but is distinguished by more extensive involvement of the lower face and by only slight cutaneous sclerosis. We describe two unusual children with both atrophic and sclerotic changes of half of the face coexisting with multiple plaques of typical morphea. Both children developed neurologic disturbances with cranial magnetic resonance imaging (MRI) abnormalities 2 years and 15 years, respectively, after the onset of cutaneous lesions. Thus considering that it may not be possible to correlate impairment in neurologic function and cutaneous disease, as illustrated by our patients, we emphasize the importance of an accurate follow-up.
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29.) [Deep morphea-type lesions, first manifestations of lymphocytic lymphoma]
=======================================================================
Author
Plantin P; Le Leannec N; Delmas A; Le Berre A; Leroy JP
Address
Service de Dermatologie, H^opital Laennec, Quimper.
Source
Ann Dermatol Venereol, 123(8):468-70 1996
Abstract
INTRODUCTION: The association between scleroderma and lymphoma is uncommon and can sometimes query an fortuitous association. More particular are observations where chronological and histological features show an evident link between a tumorous process and sclerosis. OBSERVATION: This case concerns a man 69 years old who was treated for one year for partial subcutaneous sclerosis present on his legs, thighs, fore-arms, and on the upper part of the trunk. No signs were present of visceral involvement evoking a systemic scleroderma and histology showed an intrication of a deep sclerosis, fasciitis and a tumoral lymphoma process. Diagnosis of lymphocytic lymphoma was confirmed. Initiating a chemotherapy (CHOP) allowed for a reduction of the sclerosis which didn't respond to the only corticotherapy. DISCUSSION: This observation can be linked to the association of fasciitis and lymphoma identified by Naschitz et al, but is different by: 1) the clinical aspect which correspond more to deep morphea lesions; 2) the histological link between sclerosis and lymphoma. This last point suggest that it is the tumoral population which have induced the sclerosis process linked to the secretion of pro-inflammatory factors. In adverse cases with longer delay between lymphoma and scleroderma this association may be fortuitous.
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30.) Response to methotrexate in a patient with idiopathic eosinophilic fasciitis, morphea, IgM hypergammaglobulinemia, and renal involvement.
=======================================================================
Author
Janzen L; Jeffery JR; Gough J; Chalmers IM
Address
Department of Internal Medicine, Health Sciences Centre, Winnipeg, Canada.
Source
J Rheumatol, 22(10):1967-70 1995 Oct
Abstract
A 35-year-old man with idiopathic eosinophilic fasciitis (EF) and morphea developed renal disease characterized by microscopic hematuria, nephrotic range proteinuria, and rapidly progressing hypertension, an association that has not previously been reported in EF. Initial clinical symptoms of EF began in July 1989; peripheral eosinophilia peaked at 30% in August 1990; an abnormal urinalysis was first observed in March 1992 and subsequently a renal biopsy was performed. Renal biopsy demonstrated focal segmental glomerulosclerosis and a subepithelial immune-type deposit. Partial fasciectomy and a course of methotrexate resulted in overall functional improvement of his extremities. Proteinuria and hematuria was reduced during methotrexate therapy.
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31.) Morphea after silicone gel breast implantation for cosmetic reasons in an HLA-B8, DR3-positive woman.
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Author
Di Lorenzo G; Mansueto P; Melluso M; Sangiorgi GB; Cigna D; Candore G; Caruso C
Address
Cattedra di Medicina Interna II, Istituto di Medicina Interna e Geriatria Universit`a di Palermo, Italia.
Source
Int Arch Allergy Immunol, 112(1):93-5 1997 Jan
Abstract
We describe an HLA-B8, DR3-positive patient with localized morphea after silicone gel breast implantation for cosmetic reasons. We believe that this case suggests that a genetic background, i.e. HLA-B8, DR3 haplotype, is involved in the autoimmune response to silicone.
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32.) Autoantibodies to mitochondrial 2-oxo-acid dehydrogenase complexes in localized scleroderma.
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Author
Fujimoto M; Sato S; Ihn H; Tamaki T; Kikuchi K; Soma Y; Tamaki K
Address
Department of Dermatology, Faculty of Medicine, University of Tokyo, Japan.
Source
Clin Exp Immunol, 105(2):297-301 1996 Aug
Abstract
Sera from patients with localized scleroderma frequently produce cytoplasmic staining by indirect immunofluorescence, although the antigen remains to be determined. We studied the prevalence, antigen specificity and associated clinical characteristics of anti-cytoplasmic antibodies in localized scleroderma. Serum samples from 60 patients with localized scleroderma were examined by indirect immunofluorescence analysis and immunoblotting. By immunofluorescence analysis on HEp-2 cell substrate, seven of 60 (12%) patients were shown to be positive for anti-cytoplasmic antibodies. Among these, six patients with generalized morphea had anti-mitochondrial antibodies as shown by immunoblotting: they showed reactivity with the E2 component of pyruvate dehydrogenase complex (PDC), with protein X, and with the E2 component of alpha-oxo-glutarate dehydrogenase complex, while two of them showed reactivity with PDC-E1 alpha. One of these patients who was positive for anti-PDC-E1 alpha antibody showed laboratory abnormalities, suggesting the presence of primary biliary cirrhosis. The age of disease onset was significantly higher in these six patients than in those without anti-mitochondrial antibodies. Furthermore, five of them were classified into generalized morphea with multiple plaque lesions but without linear lesions (multiple plaque type). These observations suggest that major antigens for anti-cytoplasmic antibodies in patients with localized scleroderma are mitochondrial enzymes, 2-oxo-acid dehydrogenase complexes. Patients with anti-mitochondrial antibodies may comprise a unique subset of localized scleroderma designated multiple plaque type of generalized morphea of older onset.
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33.) Association of eosinophilic fasciitis, multiple morphea and antiphospholipid antibody.
=======================================================================
Author
Castanet J; Lacour JP; Perrin C; Taillan B; Dubois D; Ortonne JP
Address
Department of Dermatology, University of Nice, France.
Source
Dermatology, 189(3):304-7 1994
Abstract
The occurrence of morphea-like changes during the course of eosinophilic fasciitis is considered to be rare. We observed such a case with simultaneous occurrence of both types of lesions. Histologically, fibrosis and inflammatory infiltrate were seen in the entire dermis, the subcutis and the fascia, suggesting that the same process might account for all skin changes. In addition, our patient had an antiphospholipid antibody, neurologic symptoms and livedo-like cutaneous lesions. However, whether the antiphospholipid antibody played a pathogenic role or not remains unclear. Corticosteroid treatment was successful.
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34.) Internal involvement in localized scleroderma.
=======================================================================
Author
Dehen L; Roujeau JC; Cosnes A; Revuz J
Address
Department of Dermatology, H^opital Henri Mondor, Universit´e Paris XII, Cr´eteil, France.
Source
Medicine (Baltimore), 73(5):241-5 1994 Sep
Abstract
We studied 76 consecutive patients with localized scleroderma (morphea with or without linear scleroderma) and analyzed the frequency, prognosis, and predictors of internal involvement in a subset of 53 patients systematically investigated for the presence of such involvement. Internal involvement was found by systematic examination in 16 patients. Only 2 of them, including 1 patient who developed systemic scleroderma, had symptomatic and severe visceral disease. The other 14 patients had asymptomatic and minor abnormalities consisting of abnormal lower sphincter pressure, and/or peristaltic failure in the esophagus and slightly impaired carbon monoxide diffusion in the lung. Frequent seroimmunologic abnormalities had no predictive value. Three parameters were found to be associated with internal involvement: male gender (p < 0.05), increasing number of plaque-like lesions (p = 0.02) and hypergamma-globulinemia at 1st examination (p < 0.005). Mild esophageal and pulmonary involvement are not rare in morphea but are usually silent. In our series, after a median follow-up of 48 months, such involvement did not impair the prognosis. The mildness of these visceral abnormalities suggests that they do not justify routine detection in asymptomatic patients. Morphea and systemic scleroderma behave as 2 different diseases.
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35.) Mucin deposits in morphea and systemic scleroderma.
=======================================================================
Author
Rongioletti F; Gambini C; Micalizzi C; Pastorino A; Rebora A
Address
Department of Dermatology, University of Genoa, Italy.
Source
Dermatology, 189(2):157-8 1994
Abstract
BACKGROUND: Though rarely reported, mucin deposition may be observed in scleroderma. OBJECTIVE: To verify the frequency of significant amounts of mucin in the biopsy specimens. METHODS: Biopsies from 20 patients with scleroderma were reviewed and stained to verify the presence of mucin. RESULTS: Mucin deposits were found in all of the 20 specimens. CONCLUSION: Mucin deposition is probably a constant feature in both morphea and systemic scleroderma. Its relevance in differential diagnosis between scleredema and scleroderma is debatable.
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36.) Classification and epidemiology of scleroderma.
=======================================================================
Author
Mayes MD
Address
Division of Rheumatology, Wayne State University, Detroit, MI 48201, USA.
Source
Semin Cutan Med Surg, 17(1):22-6 1998 Mar
Abstract
Scleroderma is classified as two separate but related entities, a localized form and a systemic form. The classification scheme for morphea presented here is that of Peterson et al, which divides morphea into five categories: plaque, generalized, bullous, linear, and deep. Using this system, these authors estimated the incidence rate of localized scleroderma to be 27 new cases per million population per year. Overall survival was similar to that of the general population. There was a preponderance of female cases (approximately 3:1) for all forms of morphea except for linear scleroderma, which had an even sex distribution. Systemic scleroderma is divided into limited and diffuse disease based on the extent of skin involvement. Recent estimates have placed the incidence rate of systemic sclerosis in the United States at 19 new cases per million adults per year, with an overall prevalence of 240/million adults. The female-to-male ratio is approximately 5:1. The prevalence of scleroderma varies by geographic region and ethnic background and is higher in the United States than in Europe or Japan. Although systemic sclerosis survival has improved over the past two decades, with 5-year survival over 80%, long-term survival is significantly lower than expected, and morbidity is considerable.
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DATA-MÉDICOS/DERMAGIC-EXPRESS No (46) 24/03/99 DR. JOSE LAPENTA R.
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Produced by Dr. José Lapenta R. Dermatologist
Venezuela 1.998-2.024
Producido por Dr. José Lapenta R. Dermatólogo
Venezuela
1.998-2.024
Tlf: 0414-2976087 - 04127766810
