REFERENCIAS BIBLIOGRÁFICAS / BIBLIOGRAPHICAL REFERENCES

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1.) Atypical manifestations of pityriasis lichenoides chronica: development into paraneoplasia and non-Hodgkin lymphomas of the skin. 

2.) Comparative clinicopathological study on pityriasis lichenoides chronica and small plaque parapsoriasis. 

3.) The transformation of pityriasis lichenoides chronica into parakeratosis variegata in an 11-year-old girl. 

4.)Immunopathologic studies in pityriasis lichenoides. 

5.)Psoralens and ultraviolet A therapy of pityriasis lichenoides. 

6.) Phototherapy of pityriasis lichenoides. 

7.) Long-term follow-up of photochemotherapy in pityriasis lichenoides. 

8.) [Ulcers of the tongue, pityriasis lichenoides and primary parvovirus B19 infection] 

9.) The relation between toxoplasmosis and pityriasis lichenoides chronica. 

10.) Pityriasis lichenoides in children: clinicopathologic review of 22 patients. 

11.) Pityriasis lichenoides in children: a long-term follow-up of eighty-nine cases. 

12.) Pityriasis lichenoides in children: therapeutic response to erythromycin. 

13.) [Pityriasis lichenoides in a sibling pair] 

14.) Cutaneous T-cell lymphoma (parapsoriasis en plaque). An association with pityriasis lichenoides et varioliformis acuta in young children. 

15.) Pityriasis lichenoides et varioliformis acuta in HIV-1+ patients: a marker of early stage disease. The Military Medical Consortium for the Advancement of Retroviral Research (MMCARR). 

16.) [Lichenoid pityriasis. Clinical study of 13 cases]. 

17.) Immunopathology of pityriasis lichenoides acuta. 

18.) Pityriasis lichenoides et varioliformis acuta in pregnancy: a case report. 

19.) Mucha-Habermann disease and its febrile ulceronecrotic variant.

20.) Febrile ulceronecrotic Mucha-Habermann disease: a case report and review of the literature. 

21.) Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta. 

22.) Mucha-Habermann disease-like eruptions due to Tegafur. 

23.) [Parakeratosis variegata after pityriasis lichenoides et varioliformis acuta]. 

24.) Methotrexate treatment of pityriasis lichenoides and lymphomatoid papulosis. 

25.) Pityriasis lichenoides and lymphomatoid papulosis. 

26.) Clinical and histologic differentiation between lymphomatoid papulosis and pityriasis lichenoides. 

27.) Lymphomatoid papulosis/pityriasis lichenoides in two children. 

28.) Immunohistology of pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica. Evidence for their interrelationship with lymphomatoid papulosis. 

29.) Lymphomatoid papulosis: clinicopathological comparative study with pityriasis lichenoides et varioliformis acuta. 

30.) Examination of cutaneous T-cell lymphoma for human herpesviruses by using the polymerase chain reaction 

31.) Molecular diagnosis of lymphocytic infiltrates of the skin. 

32.) Gene rearrangements and T-cell lymphomas. 

33.) Lymphomatoid papulosis associated with pityriasis lichenoides et varioliformis acuta with transition in a large-cell anaplastic cutaneous Ki-1 lymphoma. Treatment with alpha-interferon 

34.) Pityriasis lichenoides chronica with acral distribution mimicking palmoplantar syphilid. 

35.) Pityriasis lichenoides and acquired toxoplasmosis. 

36.) Paraneoplastic pityriasis lichenoides chronica. 

37.) Cutaneous T-cell lymphoma presenting with 'segmental pityriasis lichenoides chronica'. 

38.) Successful long-term use of cyclosporin A in HIV-induced pityriasis lichenoides chronica [letter] 

39.) Pityriasis lichenoides chronica resolving after tonsillectomy. 

40.) Segmental pityriasis lichenoides chronica. 

41.) Pityriasis lichenoides et varioliformis acuta and group-A beta hemolytic streptococcal infection. 

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1.) Atypical manifestations of pityriasis lichenoides chronica: development  into paraneoplasia and non-Hodgkin lymphomas of the skin. 

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AU: Panizzon-RG; Speich-R; Dazzi-H 

AD: Department of Dermatology, University Hospital Zurich, Switzerland. 

SO: Dermatology. 184(1):65-9 1992 

PY: 1992 

PT: JOURNAL-ARTICLE 

AB-A: Three patients with atypical courses and manifestations of pityriasis  lichenoides chronica (PLC) are presented. The first patient is a  21-year-old white woman who showed a good response of her PLC lesions as  well as her reactive oligoarthritis to repeated PUVA treatments combined  with oral prednisone during 1 year.

The effect of the treatment then  decreased. The patient developed a low-grade malignant lymphoma of the  lung. When the lymphoma of the lung improved after chemotherapy, the PLC  eruptions improved, too. The second patient is a 41-year-old man, whose  Hodgkin's disease stage IVa was successfully treated by chemotherapy and  radiotherapy in 1984. In 1987 he showed PLC lesions which responded well to  PUVA therapy, later also in combination with etretinate. Until 1988  repeated skin biopsies revealed a non-specific eczematous pattern. In 1989  the recalcitrant PLC eruptions finally revealed a pleomorphic non-Hodgkin  lymphoma of the skin with medium-sized cells.

The third patient had a PLC  for about 9 years when Hodgkin's disease stage Ia was diagnosed. At the  beginning the skin biopsy showed an eczematous pattern, but 2 years later,  in 1990, skin infiltrations of a large-cell, anaplastic non-Hodgkin  lymphoma were seen. These cases show that PLC in rare cases may either  represent a paraneoplastic skin disease or may itself develop into  cutaneous lymphomas. (Abstract from CANCERLIT) 

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2.) Comparative clinicopathological study on pityriasis lichenoides  chronica and small plaque parapsoriasis. 

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SO - Am J Dermatopathol 1988 Jun;10(3):189-96 

AU - Benmaman O; Sanchez JL 

PT - JOURNAL ARTICLE 

AB - The term parapsoriasis refers to a group of chronic asymptomatic  scaly dermatoses of unknown etiology about which there is still controversy  over the nosology and nomenclature of the different conditions that  comprise the group, particularly pityriasis lichenoides chronica (PLC) and  small plaque parapsoriasis (SPP).

In an attempt to establish the  distinctive clinicopathologic features of these two dermatosis, we  prospectively studied 44 patients who presented with the typical clinical  and histologic picture of either of these two diseases. SPP was clinically  characterized by scaly oval plaques on the trunk and proximal aspect of  extremities. Spongiosis was the salient histopathologic feature, with  absence of fibrosis or melanophages.

PLC presented with a scaly papular  eruption over the trunk and extremities and histologically was  characterized by an interface dermatitis. We conclude that sufficient  clinical and histologic features differentiate these two entities and we  propose that the term parapsoriasis be used only to designate SPP and large  plaque parapsoriasis. 

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3.) The transformation of pityriasis lichenoides chronica into parakeratosis variegata in an  11-year-old girl. 

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Author 

Niemczyk UM; Zollner TM; Wolter M; Staib G; Kaufmann R 

Address 

Department of Dermatology, University of Frankfurt Medical School, Germany. 

Source 

Br J Dermatol, 137(6):983-7 1997 Dec 

Abstract 

Parakeratosis variegata is a rare disorder with unknown aetiology. In a few  cases it arises from benign skin diseases such as pityriasis lichenoides et  varioliformis acuta (Mucha Habermann disease) or pityriasis lichenoides  chronica.

However, transformation into malignant diseases such as cutaneous  T-cell lymphoma has been observed. We report an 11-year-old girl with a  10-year history of pityriasis lichenoides chronica now presenting with  parakeratosis variegata.

Analysis of skin infiltrating T cells showed  clonally rearranged T-cell receptor gamma chains occurring with a frequency  of more than 2%. This finding is compatible with the clinical observation  of parakeratosis variegata transforming into a malignant T-cell disorder.  We therefore suggest that patients suffering from parakeratosis variegata  and other diseases such as pityriasis lichenoides et varioliformis acuta or  pityriasis lichenoides chronica should be continuously monitored. 

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4.) Immunopathologic studies in pityriasis lichenoides. 

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SO - Arch Dermatol Res 1988;280 Suppl:S61-5 

AU - Giannetti A; Girolomoni G; Pincelli C; Benassi L 

PT - JOURNAL ARTICLE 

AB - Skin biopsy specimens from five patients with pityriasis lichenoides  et varioliformis acuta and from six patients with pityriasis lichenoides  chronica were studied by direct immunofluorescence and by an  immunoperoxidase technique using a panel of monoclonal antibodies. The  dermal inflammatory infiltrate was composed of T cells, macrophages, and a  small proportion of CD1a+ cells, mostly perivascular.

CD8+ cells  (cytotoxic/suppressor phenotype) predominated in the epidermis according to  the degree of epidermal necroses, whereas CD4+ cells (helper/inducer  phenotype) were superior in number among dermal T cells.

A few B cells and  Leu7+ cells were detected in only a small proportion of lesions. The  results obtained confirm that the two conditions are variants of a single  disease process and suggest that cell-mediated immune mechanisms may be  important in the pathogenesis of the epidermal and vascular damage.  Endothelial cells (HLA-DR+ and HLA-DQ+) and CD1a+ cells (epidermal and  possibly dermal) could be primarily involved, acting as antigen-presenting  cells. 

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5.) Psoralens and ultraviolet A therapy of pityriasis lichenoides. 

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SO - J Am Acad Dermatol 1984 Jan;10(1):59-64 

AU - Powell FC; Muller SA 

PT - JOURNAL ARTICLE 

AB - Three patients with long-standing pityriasis lichenoides, which was  resistant to other forms of therapy, were successfully treated with PUVA  (psoralens and ultraviolet light of wavelength A). One patient had complete  clearing of all lesions, and the other two had marked improvement. PUVA is  being used to treat increasing numbers of patients with pityriasis  lichenoides, and the results have been very good. 

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6.) Phototherapy of pityriasis lichenoides. 

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Arch Dermatol 1983 May;119(5):378-80 

LeVine MJ 

Eleven patients with chronic pityriasis lichenoides chronica were treated  with topically applied bland emollient cream and minimally erthemogenic  doses of UV radiation from fluorescent sunlamps. The conditions of all  patients cleared completely in an average of 29 treatments, requiring an  average UV dose of 388 millijoules/sq cm at clearance. Phototherapy  provides a convenient effective outpatient therapy for pityriasis  lichenoides chronica. 

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7.) Long-term follow-up of photochemotherapy in pityriasis lichenoides. 

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Acta Derm Venereol 1982;62(5):442-4 

Boelen RE, Faber WR, Lambers JC, Cormane RH 

Five patients with a histopathologically confirmed diagnosis of pityriasis  lichenoides were treated with PUVA or irradiated with a light source  emitting UVB and UVA, without prior intake of psoralens. All patients  showed a good response to treatment.

Long-term follow up showed that  patients remained free of lesions during a period of 20 to 36 months; 3  patients had a recurrence of the disease, though less extensive than  before, after 25, 23, and 23 months, respectively. 

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8.) [Ulcers of the tongue, pityriasis lichenoides and primary parvovirus B19 infection] 

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Author 

Labarthe MP; Salomon D; Saurat JH 

Address 

Service de Dermatologie, Hospital Cantonal Universitaire de Gen`eve, Suisse. 

Source 

Ann Dermatol Venereol, 123(11):735-8 1996 

Abstract 

INTRODUCTION: We report a case of parapsoriasis en gouttes (or pityriasis  lichenoides) which presents two peculiarities. First, the patient had  lingual ulcerations and second, the eruption appeared during a  seroconversion for Parvovirus B19.

OBSERVATION: A 25 old woman presented a  first episode of characteristic parapsoriasis en gouttes associated with  purpuric palmoplantar lesions and lingual ulcerations, reaching deep  muscular in histology.

DISCUSSION: This observation of parapsoriasis en  gouttes, peculiar because of lingual ulcerations, is mostly interesting  because of its association with a primo-infection to Parvovirus B19. The  receptor of the virus is localised on endothelial cells and that could  explain purpuric lesions and ulcerations observed. 

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9.) The relation between toxoplasmosis and pityriasis lichenoides chronica. 

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Author 

Nassef NE; Hammam MA 

Address 

Department of Parasitology, Faculty of Medicine, Menoufia University, Egypt. 

Source 

J Egypt Soc Parasitol, 27(1):93-9 1997 Apr 

Abstract 

Pityriasis lichenoides chronica (PLC) is a rare skin disease of uncertain  aetiology. Many infectious agents have been incriminated as the cause of  the disease. One of these agents is toxoplasmosis.

The aim of this work was  to find out if there is a relationship between toxoplasmosis and PLC.  Twenty two patients (17 males and 5 females) diagnosed clinically and  histopathologically as PLC were chosen for this study. Also twenty  apparently healthy individuals free from skin lesions were included as a  control group.

Patients and controls were examined clinically for signs of  toxoplasmosis and submitted for indirect haemagglutination (IHA) and  indirect immunofluorescent antibody (IFA) tests in our Parasitology  laboratory for serodiagnosis of toxoplasmosis. Toxoplasmosis was diagnosed  in 8 (36.36%) and 3 (15%) in PLC patients and controls respectively by both  tests. Using pyrimethamine and trisulfapyrimidines in treating PLC patients,  showed subsidence of skin lesions in five patients with toxoplasmosis  within two months from the beginning of therapy.

The remaining patients  showed no response to treatment. On conclusion, toxoplasmosis appears to  play a role in the aetiology of PLC and serological tests for diagnosing  toxoplasmosis should be performed in all PLC patients. 

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10.) Pityriasis lichenoides in children: clinicopathologic review of 22 patients. 

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Author 

Roman´i J; Puig L; Fernández-Figueras MT; de Moragas JM 

Address 

Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Barcelona,  Spain. 

Source 

Pediatr Dermatol, 15(1):1-6 1998 Jan-Feb 

Abstract 

Pityriasis lichenoides (PL) is a cutaneous disease of unknown origin, with  an autoinvolutive course, that can occur in pediatric patients.  Traditionally, acute and chronic variants have been described, but other  special forms of presentation have been reported.

We reviewed the clinical  records and histopathologic specimens of all pediatric patients diagnosed  with PL in our hospital from 1980 to 1995 to assess the clinicopathologic  features of this disorder in our environment. Twenty-two of the 118 cases  reviewed were pediatric patients less than 15 years old (12 males and 10  females, 18.6% of all patients). Their ages ranged from 3 to 15 years, with  a mean of 9.3 years.

Most of the patients (72%) had the chronic variant of  the disease, while the remainder had an acute course. One patient suffered  from acute ulceronecrotic PL. Systemic treatments prescribed were  erythromycin in eight patients, PUVA in five patients, and methotrexate in  one patient. Three patients had a prolonged course with more than two  episodes. Acute and chronic PL are polar extremes, but individual cases  cannot be classified only on the basis of histopathologic data, since  coexistence of lesions in different stages of evolution can lead to  sampling bias.

Acute ulceronecrotic forms and the presence of a variable  degree of cellular atypia in the infiltrate are liable to cause  differential diagnostic problems with lymphomatoid papulosis (LP), which  cannot be completely resolved on the basis of T-cell receptor clonal  rearrangement detection. 

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11.) Pityriasis lichenoides in children: a long-term follow-up of eighty-nine cases. 

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J Am Acad Dermatol 1990 Sep;23(3 Pt 1):473-8 

Gelmetti C, Rigoni C, Alessi E, Ermacora E, Berti E, Caputo R 

Department of Dermatology and Pediatric Dermatology I, University of Milan,  Italy. 

Pityriasis lichenoides is usually classified into an acute and a chronic  form. From a review of 89 cases of the disease seen since 1974 it seems  that a more realistic classification into three main groups, according to  the distribution of pityriasis lichenoides lesions, could be made, namely,  a diffuse, a central, and a peripheral form, each characterized by a  different clinical course.

Conversely, no correlations were detected in our  series between the severity of skin lesions and their distribution or the  overall course of the disease.

None of our cases suggests the possible  evolution of pityriasis lichenoides into lymphomatoid papulosis. Although  no infectious causative agent has been identified, a viral origin seems  likely in some cases. Most patients responded favorably to UVB irradiation. 

Our conclusions are (1) that pityriasis lichenoides is probably a clinical  disorder with a diverse etiology and (2) that its classification by  distribution seems more useful than its subdivision into an acute and a  chronic form. 

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12.) Pityriasis lichenoides in children: therapeutic response to erythromycin. 

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J Am Acad Dermatol 1986 Jul;15(1):66-70 

Truhan AP, Hebert AA, Esterly NB 

Fifteen of twenty-two children with pityriasis lichenoides were treated  with oral erythromycin. Eleven (73%) had a remission, usually within 2  months. Two others showed partial improvement, and two were unimproved.  Seven of the children who experienced a remission were off erythromycin and  free of lesions after 2 to 5 months of therapy. A trial of erythromycin as  described herein should be considered in children with pityriasis  lichenoides before other, possibly more toxic, measures are instituted. 

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13.) [Pityriasis lichenoides in a sibling pair] 

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ARTICLE SOURCE: Hautarzt (Germany, West), Nov 1981, 32(11) p592-4 

AUTHOR(S): Deuchert C 

PUBLICATION TYPE: JOURNAL ARTICLE 

ABSTRACT: Two brothers are reported, who had pityriasis lichenoides within  an interval of eighteen months. The hitherto unknown etiology of this  dermatosis is discussed. 

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14.) Cutaneous T-cell lymphoma (parapsoriasis en plaque). An association with pityriasis  lichenoides et varioliformis acuta in young children. 

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Authors 

Fortson JS. Schroeter AL. Esterly NB. 

Institution  Department of Dermatology, Wright State University School of Medicine,  Dayton, Ohio 45401-0927. 

Source 

Archives of Dermatology. 126(11):1449-53, 1990 November. 

Abstract 

Pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis  lichenoides chronica (PLC) are related benign disorders without recognized  association with cutaneous T-cell lymphoma (CTCL).

We report the cases of  two children with documented PLEVA evolving into CTCL over several years.  One child had the clinical lesions of PLC but the dermatopathologic  findings of PLEVA at age 2 years. At age 12 years, he had skin changes of  poikiloderma atrophicans vasculare and dermatopathologic findings  consistent with parapsoriasis en plaque.

The second child presented at age  7 years with scaling dermatitis and dermatopathologic findings of PLEVA. At  age 12 years, the histologic diagnosis was parapsoriasis.

Monoclonal  antibody studies performed on biopsy specimens from both patients revealed  70% to 100% cells staining with CD5, 80% to 90% staining with CD4, 30% to  50% staining with CD8, and an increase in CD1-staining cells in the  papillary dermis, indicating a predominantly helper T-cell infiltrate. We  believe that PLC and PLEVA may be part of the spectrum of CTCL.  Furthermore, CTCL may be more common in young children than once thought. 

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15.) Pityriasis lichenoides et varioliformis acuta in HIV-1+ patients: a marker of early  stage disease. The Military Medical Consortium for the Advancement  of Retroviral Research (MMCARR). 

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Author 

Smith KJ; Nelson A; Skelton H; Yeager J; Wagner KF 

Address 

Medical Research Institute of Chemical Defense, Aberdeen, Maryland, USA. 

Source 

Int J Dermatol, 36(2):104-9 1997 Feb 

Abstract 

BACKGROUND: The high incidence of cutaneous disease in HIV-1+ patients may  be a marker of the chronic state of immune activation. In addition,  specific cutaneous diseases may be related to the pattern and degree of  immune dysregulation present in the patients at the time of the eruption.  We have observed that HIV-1+ patients with pityriasis lichenoides et  varioliformis acuta (PLEVA) were in the early to mid stage of HIV-1 disease. 

MATERIALS AND METHODS: To determine if there was a correlation between the  phenotype of the lymphoid infiltrate and surface markers of the epidermis  and the known changes in early or late-stage HIV-1 disease, we studied five  HIV-1+ patients with PLEVA.

Cutaneous biopsy specimens were obtained and  immunohistochemical stains were used to determine the expression of ELAM-1,  ICAM-1, and HLA-DR and the phenotype of the lymphoid infiltrate.

RESULTS:  The HIV-1+ patients showed increased expression of HLA-DR on keratinocytes  as well as on the mononuclear and dendritic cell populations in the  epidermis and dermis. The majority of T cells were activated CD8+ cells. 

CONCLUSIONS: Immunophenotyping of the inflammatory infiltrate in these  patients is consistent with a pattern of immune dysregulation seen only in  earlier stages of HIV-1 disease. Thus, PLEVA may be useful as a marker of  early to mid stages of HIV-1 disease. 

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16.) [Lichenoid pityriasis. Clinical study of 13 cases]. 

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Med Cutan Ibero Lat Am 1977;5(3):189-96 

[Article in Spanish] 

Bravo Piris J 

13 patients with Pityriasis Lichenoides are studied clinical and  histologically, showing a clinical polymorphism of the lesions, mainly in  the papulous, vesiculous, and necrotic ones. The data about age, sex,  evolution and response to the treatment in the present study are similar to  those found by other authors.

Constantly, we found, a variable degree of  vasculitis. In almost all the cases there was a damage of the epithelium  --exoserosis and exocytosis--, as well as presence in some cases, of red  cells extravasated within the epidermis. In upper dermis we found in all  biopsies, divers degrees of perivascular cell infiltration mainly composed  of lymphocytes and histiocytes with predominance of the last ones, in five  cases.

In the majority of our cases, there was a strong relationship  between the clinical and the histological aspects, but in some cases, mild  lesions showed an acute microscopical picture. We are of the opinion that  Pityriasis Lichenoides must be considered as a different entity from  Parapsoriasis.

In addition, we think that PL, is a clinical picture that  manifests itself as a chronic or an acute form, and both types can be seen  in the disease evolution. Finally, we could not find an evident influence  and a positive response to the treatment in our patients with the classical 

therapeutics. 

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17.) Immunopathology of pityriasis lichenoides acuta. 

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ARTICLE SOURCE: J Am Acad Dermatol (United States), May 1984, 10(5 Pt 1)  p783-95 

AUTHOR(S): Muhlbauer JE; Bhan AK; Harrist TJ; Moscicki RA; Rand R;  Caughman W; Loss B; Mihm MC Jr 

PUBLICATION TYPE: JOURNAL ARTICLE 

ABSTRACT: Eleven biopsy specimens (five papules and six dusky or crusted  lesions) from four patients with pityriasis lichenoides et varioliformis  acuta ( PLEVA ) were studied by direct immunofluorescence and  immunoperoxidase technics. Slight vascular deposits of IgM and C3 were  present in most lesions.

Slight perivascular deposits of fibrin were  observed in early lesions; more extensive perivascular and interstitial  deposits of fibrin were detected in advanced lesions. Most of the  infiltrating cells were T lymphocytes; cells with cytotoxic/suppressor  phenotype (T8-positive) were generally more numerous than cells with  helper/inducer phenotype (Leu-3a-positive, T4-positive). A marked increase  in epidermal T8-positive cells over epidermal Leu-3a/T4-positive cells was  found in late lesions.

Moreover, a reduction of the ratio of circulating  T4-positive to T8-positive cells was observed in most cases. The number of  epidermal T6-positive (Langerhans/indeterminate) cells was decreased in the  lower as compared with the upper stratum spinosum.

About 5% of perivascular  infiltrating cells were T6-positive. These results suggest that  cell-mediated immune mechanisms are probably important in the pathogenesis  of PLEVA 

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18.) Pityriasis lichenoides et varioliformis acuta in pregnancy: a case report. 

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Author 

Fukada Y; Okuda Y; Yasumizu T; Hoshi K 

Address 

Department of Obstetrics and Gynecology, Yamanashi Medical University, Japan. 

Source 

J Obstet Gynaecol Res, 24(5):363-6 1998 Oct 

Abstract 

If pityriasis lichenoides et varioliformis acuta (PLAVA) exists in the  vagina or cervical os of the uterus, it may cause premature labor and  premature rupture of the membranes. 

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19.) Mucha-Habermann disease and its febrile ulceronecrotic variant.  Author 

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Tsuji T; Kasamatsu M; Yokota M; Morita A; Schwartz RA 

Address 

Department of Dermatology, Nagoya City University Medical School, Nagoya,  Japan. 

Source 

Cutis, 58(2):123-31 1996 Aug 

Abstract 

In 1916 Mucha and in 1925 Habermann reported an acute form of pityriasis  lichenoides characterized by the abrupt onset of papulovesicular eruptions  and gave the name, pityriasis lichenoides et varioliformis acuta (PLEVA) or  Mucha-Habermann disease (MH).

In 1966, Degos reported a rare febrile  ulceronecrotic variant of MH. MH occurs mainly in young adults, while  febrile ulceronecrotic Mucha-Habermann's disease (FUMHD) occurs more  frequently in children.

The etiology of MH remains obscure, but it may be  the result of a hypersensitivity reaction to an infectious agent.

Although  clinical and histologic features of the disease in children are similar to  those of adults, more diseases need to be differentiated in pediatric  patients. In addition, a number of effective therapeutic options in adults  with MH are unsuitable for use in pediatric patients, to whom beginning  with oral antibiotics, usually erythromycin, is recommended.

A summary of  previously reported fifteen cases with FUMHD, including our case, is listed. 

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20.) Febrile ulceronecrotic Mucha-Habermann disease: a case report and review of the  literature. 

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Author 

Su´arez J; L´opez B; Villalba R; Perera A 

Address 

Section of Dermatology, Hospital La Candelaria, Santa Cruz de Tenerife,  Spain. 

Source 

Dermatology, 192(3):277-9 1996 

Abstract 

A 32-year-old male with febrile ulceronecrotic Mucha-Habermann disease  (FUMHD) responsive to methotrexate is reported. This is a severe variant of  pityriasis lichenoides et varioliformis acuta characterized by the acute  onset of a widespread ulceronecrotic cutaneous eruption together with high  fever and systemic involvement. To our knowledge, only 13 patients with  FUMHD have been reported to date. 

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21.) Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta. 

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Dermatology 1994;189 Suppl 2:50-3 

De Cuyper C, Hindryckx P, Deroo N 

Algemeen Ziekenhuis Sint-Jan, Ruddershove, Brugge, Belgium. 

An unusually severe form of pityriasis lichenoides et varioliformis acuta  (PLEVA) with a fatal outcome in an 82-year-old woman is reported. After a  period of a mild eruption, extensive polymorphous, papular and  ulcerohemorrhagic skin lesions developed, associated with intermittent high  temperature and constitutional symptoms.

Skin biopsies showed the typical  histopathological changes of PLEVA. Early recognition of this severe  variant of PLEVA is important, since the fulminating course can lead to  death. 

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22.) Mucha-Habermann disease-like eruptions due to Tegafur. 

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Author 

Kawamura K; Tsuji T; Kuwabara Y 

Address 

Department of Dermatology, Nagoya City University Medical School, Japan. 

Source 

J Dermatol, 26(3):164-7 1999 Mar 

Abstract 

The first case of Mucha-Habermann disease-like drug eruptions due to  Tegafur is reported. A 59-year-old man noticed various skin lesions after  he had taken 300 mg of Tegafur daily for about 200 days.

The patient had  papulonecrotic eruptions on his trunk and extremities. The histology from a  papular lesion revealed epidermal necrosis surrounded by spongiosis,  perivascular inflammatory infiltrations composed of lymphocytes and  erythrocytes, and endothelial swelling.

The etiology of Mucha-Habermann  disease is not known, but an immune mechanism may be supported by our case. 

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23.) [Parakeratosis variegata after pityriasis lichenoides et varioliformis acuta]. 

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Hautarzt 1995 Jul;46(7):498-501 

Kiene P, Folster-Holst R, Mielke V 

Universitats-Hautklinik, Kiel. 

We report on a 34-year-old male patient who developed generalized  parakeratosis variegata lesions 4 years after suffering from pityriasis  lichenoides et varioliformis acuta. For further investigation of a possible  interrelationship between these two diseases of the parapsoriasis group and  their relationship to the T-cell type of cutaneous non-Hodgkin-lymphoma,  histological, immunohistological and molecular-biological techniques were  applied. We were able to demonstrate typical morphological features common  to both diseases, and a polyclonal T-cell infiltrate in both.

It is  concluded that pityriasis lichenoides et varioliformis acuta and  parakeratosis variegata are separate entities without monoclonal  rearrangement or signs of malignancy. 

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24.) Methotrexate treatment of pityriasis lichenoides and lymphomatoid papulosis. 

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Cutis 1979 May;23(5):634-6 

Lynch PJ, Saied NK 

Pityriasis lichenoides is a notoriously difficult disease to treat. Three  patients with this condition and a fourth with lymphomatoid papulosis have  been successfully treated with doses of methotrexate once a week. Toxicity  noted during the treatment periods has been minimal. 

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25.) Pityriasis lichenoides and lymphomatoid papulosis. 

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Semin Dermatol 1992 Mar;11(1):73-9 

Rogers M 

Department of Dermatology, Children's Hospital, Camperdown, Australia. 

The clinical features, histopathology, immunopathology, and management of  pityriasis lichenoides and lymphomatoid papulosis are discussed, with  particular emphasis on the pediatric aspects of these conditions.

The  difficulties in logically separating pityriasis lichenoides into an acute  (pityriasis lichenoides et varioliformis acuta) and a chronic (pityriasis  lichenoides chronical) form are addressed. The development of  lymphoreticular malignancy in patients with lymphomatoid papulosis has been  well documented, but pityriasis lichenoides has characteristically been  regarded as a benign condition.

However, recent reports of the development  of large plaque parapsoriasis in patients with pityriasis lichenoides have  led to a reconsideration. Some of these patients were in the pediatric age  group.

Although there are significant clinical, histopathological, and  immunopathological differences between pityriasis lichenoides and  lymphomatoid papulosis, the demonstration of similar clonal T cell receptor  gene rearrangements and the confirmation of the potentially premalignant  nature of both suggests that there may indeed be an interrelationship  between these two controversial entities.

Close follow-up of patients with  both of these conditions is recommended, with observation being  discontinued only when the patient has been free of lesions for several  years. 

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26.) Clinical and histologic differentiation between lymphomatoid papulosis and pityriasis  lichenoides. 

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J Am Acad Dermatol 1985 Sep;13(3):418-28 

Willemze R, Scheffer E 

The relationship between lymphomatoid papulosis and pityriasis lichenoides  is a matter of considerable debate. Differentiation between these two  conditions is, however, important because patients with lymphomatoid  papulosis, unlike those with pityriasis lichenoides, may develop systemic  lymphoma and thus require long-term follow-up. In our study the clinical  and histologic features of eighty-two patients with pityriasis lichenoides  and twenty-six patients with lymphomatoid papulosis were reviewed and  compared.

Clinical and histologic differences were recognized, not only  allowing differentiation between the two conditions, but also suggesting  that they are pathogenetically distinct diseases. Finally, evidence is  presented to suggest that the different views on the relationship between  these diseases mainly result from differences in patient selection. 

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27.) Lymphomatoid papulosis/pityriasis lichenoides in two children. 

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Pediatr Dermatol 1987 Nov;4(3):238-41 

Ashworth J, Paterson WD, MacKie RM 

Department of Dermatology, University of Glasgow, United Kingdom. 

Two children developed lymphomatoid papulosis/pityriasis lichenoides at  ages 3 and 6 years. Follow-up continued for 13 years in the former patient  and for 6 years in the latter. Both children now have continuing low-grade  disease activity requiring in the one case topical corticosteroid therapy  and in the other low-dose systemic steroid therapy.

These children are  reported to emphasize to pediatricians, pediatric pathologists, and  hematologists that pseudolymphomatous conditions can exist in young  children and do not require potent cytotoxic therapy. In both of our  patients, the initial diagnosis was thought to be an aggressive lymphoma. 

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28.) Immunohistology of pityriasis lichenoides et varioliformis acuta and  pityriasis lichenoides chronica. Evidence for their interrelationship with  lymphomatoid papulosis. 

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J Am Acad Dermatol 1987 Mar;16(3 Pt 1):559-70 

Wood GS, Strickler JG, Abel EA, Deneau DG, Warnke RA 

Pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides  chronica are idiopathic, papular eruptions that exhibit certain  clinicopathologic similarities to each other and to lymphomatoid papulosis.  In order to determine if these disorders are also similar immunologically,  we studied the immunopathology of five biopsy specimens from three cases of  pityriasis lichenoides et varioliformis acuta and three biopsy specimens  from three cases of pityriasis lichenoides chronica.

We then compared them  to our prior immunohistologic study of nine cases of lymphomatoid  papulosis. Pityriasis lichenoides et varioliformis acuta and pityriasis  lichenoides chronica both exhibited a dermal and epidermal infiltrate of  CD4+ and CD8+ T cells expressing activation antigens. These were admixed  with numerous macrophages.

The lesional epidermis was diffusely human  lymphocyte antigen (HLA)-DR+ and contained decreased CD1+ dendritic cells.  Endothelial cells were also HLA-DR+. Cells bearing the phenotypes of B  cells, follicular dendritic cells, or natural killer/killer cells were  essentially absent. Except for the lack of large atypical cells, the  results resembled those described previously for lymphomatoid papulosis. 

These findings indicate that pityriasis lichenoides chronica, pityriasis  lichenoides et varioliformis acuta, and lymphomatoid papulosis share  several immunohistologic features. Together with certain clinicopathologic  similarities, they are consistent with the hypothesis that these three 

disorders are interrelated. 

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29.) Lymphomatoid papulosis: clinicopathological comparative study with  pityriasis lichenoides et varioliformis acuta. 

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J Dermatol 1991 Oct;18(10):580-5 

Erpaiboon P, Mihara I, Niimura M 

Department of Dermatology, Jikei University School of Medicine, Tokyo, Japan. 

We have compared the clinical and histopathological features of 6 patients  with lymphomatoid papulosis (LP) and 14 patients with pityriasis  lichenoides et varioliformis acuta (PLEVA). There were some differences  between the clinical features in the two diseases, including the size and  appearance of skin lesions and the duration of the course of disease.

Ki-1  Ag positive, large, atypical, lymphoid cells were always seen in  lymphomatoid papulosis; none of lymphoid cells of pityriasis lichenoides et  varioliformis acuta demonstrated this antigen. We conclude that  lymphomatoid papulosis and PLEVA, although sharing some common features,  should be considered to be different clinical and immunopathological  entities. 

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30.) Examination of cutaneous T-cell lymphoma for human herpesviruses by  using the polymerase chain reaction 

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AU: Brice-SL; Jester-JD; Friednash-M; Golitz-LE; Leahy-MA; Stockert-SS; 

Weston-WL 

AD: B-153, Department of Dermatology, University of Colorado, Health  Sciences Center, 4200 East 9th Avenue, Denver, CO 80262, United States 

SO: J-Cutan-Pathol. 20(4):304-7 1993 

CP: Denmark 

PT: JOURNAL-ARTICLE 

AB-A: The etiology of cutaneous T-cell lymphoma remains unknown, although  an association with viral infection, in particular certain retroviruses and  human herpesviruses, has been suggested.

The purpose of this study was to  examine skin biopsies of cutaneous T-cell lymphoma for the presence of  Epstein-Barr virus, herpes simplex virus type 1 and type 2, and human  herpesvirus-6 by using the polymerase chain reaction. Lesional skin  biopsies from 30 patients with cutaneous T-cell lymphoma were studied. 

Control specimens included biopsies from 9 patients with lymphomatoid  papulosis and 10 patients with pityriasis lichenoides et varioliformis  acuta. DNA extracted from each specimen, as well as from a known positive  control for each virus, was examined by using the polymerase chain reaction  with viral-specific primers.

Each DNA specimen was also amplified with  control primers for human beta globin. The specificity of the amplified  products was confirmed by Southern analysis. Neither Epstein-Barr virus nor  herpes simplex virus was detected in any of the patient specimens examined.  Human herpesvirus-6 was detected in one specimen of cutaneous T-cell  lymphoma and one specimen of lymphomatoid papulosis.

These results do not  support a role for any of these herpesviruses in the pathogenesis of  cutaneous T-cell lymphoma. (Abstract from CANCERLIT AND EMBASE) 

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31.) Molecular diagnosis of lymphocytic infiltrates of the skin. 

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Authors 

Weinberg JM. Rook AH. Lessin SR. 

Institution  Department of Dermatology, University of Pennsylvania, Philadelphia. 

Source 

Archives of Dermatology. 129(11):1491-500, 1993 November. 

Abstract 

BACKGROUND: Advances in our understanding of the molecular genetics of  lymphocyte antigen receptors (B-cell immunoglobulin and T-cell antigen  receptor), have led to the application of molecular biologic techniques to  molecularly characterize lymphocytic infiltrates of the skin. Molecular  diagnosis refers to the application of these techniques as a diagnostic aid  in the clinicopathologic evaluation of cutaneous lymphocytic infiltrates. 

OBSERVATION: Molecular studies have clinical application in the  determination of lineage and detection of retroviruses in cutaneous  lymphoid neoplasms, distinguishing between lymphoproliferative and reactive  infiltrates, and staging and monitoring response to therapy in cutaneous  T-cell lymphoma.

Southern blot analysis of immunoglobulin and T-cell  antigen receptor gene rearrangements may fail to aid the clinician in  establishing a diagnosis of a cutaneous malignancy due to the limits of  detection sensitivity in minimally infiltrated lesions (eg, parapsoriasis  and patch-stage mycosis fungoides) or the still uncertain prognostic  significance of clonality in benign cutaneous diseases (eg, follicular  mucinosis, pityriasis lichenoides et varioliformis acuta, lymphomatoid  papulosis, and cutaneous lymphoid hyperplasia).

CONCLUSIONS: Molecular  studies have enormous research value, providing new means to explore the  pathogenesis and clonal evolution of lymphoproliferative skin diseases.  Presently, however, they have limited applications as an independent  diagnostic tool.

As our understanding of the clinical and biologic  significance of the molecular detection of clonal lymphocyte populations in  the skin expands and as the application of polymerase chain reaction  amplification provides us with greater detection sensitivity and  specificity, the clinical utility of molecular diagnosis of lymphocytic  infiltrates of the skin will be enhanced. 

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32.) Gene rearrangements and T-cell lymphomas. 

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Authors 

Terhune MH. Cooper KD. 

Institution  Department of Dermatology, University of Michigan School of Medicine, Ann 

Arbor. 

Source 

Archives of Dermatology. 129(11):1484-90, 1993 November. 

Abstract 

BACKGROUND: Cutaneous T-cell lymphomas comprise a broad spectrum of  neoplasia ranging from indolent to highly aggressive types. To determine  subset lineage and malignant vs benign nature, morphologic analysis,  immunophenotyping, and flow cytometry have been used. However, given the  shortcomings of these methods, molecular genetic techniques, which take  particular advantage of the clonal nature of malignancy, are now being  applied to better characterize and diagnose these lymphomas.

RESULTS: Each  antigen-specific T cell and its clonal progeny has a unique rearrangement  of its T-cell receptor gene such that it can recognize very specific  antigenic epitopes.

By visualizing these particular T-cell receptor gene  rearrangements, Southern hybridization techniques and polymerase chain  reaction amplification can detect clonal populations of T cells in the  skin, blood, and lymph nodes of patients with T-cell leukemias and  lymphomas.

Clonal T-cell populations have also been found in cases of  benign disorders such as lymphomatoid papulosis and pityriasis lichenoides  et varioliformis acuta. Although these disorders usually have a benign  outcome, they may represent dysplastic clonal lymphoid expansions with a  high incidence of spontaneous regression.

CONCLUSIONS: Molecular genetic  techniques have added to our ability to diagnose, characterize, and monitor  the course of T-cell lymphomas and leukemias. In addition, they may provide  insight into the pathogenesis of certain benign disorders. 

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33.)Lymphomatoid papulosis associated with pityriasis lichenoides et  varioliformis acuta with transition in a large-cell anaplastic cutaneous  Ki-1 lymphoma. Treatment with alpha-interferon 

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AU: Hilbert-E; Detmar-M; Gollnick-H; Bruchhauser-U; Orfanos-CE 

SO: Z-HAUTKR. 67/9 (832) 1992 

CO: ZHKRA 

PY: 1992 

LA: German 

CP: Federal-Republic-of-Germany 

PT: Journal-Article 

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34.) Pityriasis lichenoides chronica with acral distribution mimicking  palmoplantar syphilid. 

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Acta Derm Venereol 1999 May;79(3):239 

Chung HG, Kim SC 

Publication Types: 

Letter 

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35.) Pityriasis lichenoides and acquired toxoplasmosis. 

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Int J Dermatol 1999 May;38(5):372-4 

Rongioletti F, Delmonte S, Rebora A 

Department of Dermatology, University of Genoa, Italy. 

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36.) Paraneoplastic pityriasis lichenoides chronica. 

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J Eur Acad Dermatol Venereol 1999 Mar;12(2):189-90 

Lazarov A, Lalkin A, Cordoba M, Lishner M 

Letter 

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37.) Cutaneous T-cell lymphoma presenting with 'segmental pityriasis lichenoides  chronica'. 

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Clin Exp Dermatol 1998 Sep;23(5):232 

Child FJ, Fraser-Andrews EA, Russell-Jones R 

Publication Types: 

Letter 

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38.) Successful long-term use of cyclosporin A in HIV-induced pityriasis  lichenoides chronica [letter] 

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Author 

Griffiths JK 

Source 

J Acquir Immune Defic Syndr Hum Retrovirol, 18(4):396-7 1998 Aug 1 

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39.) Pityriasis lichenoides chronica resolving after tonsillectomy. 

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Br J Dermatol 1993 Sep;129(3):353-4 

Takahashi K, Atsumi M 

Publication Types: 

Letter 

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40.) Segmental pityriasis lichenoides chronica. 

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Clin Exp Dermatol 1996 Nov;21(6):464-5 

Cliff S, Cook MG, Ostlere LS, Mortimer PS 

Publication Types: 

Letter 

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41.) Pityriasis lichenoides et varioliformis acuta and group-A beta  hemolytic streptococcal infection. 

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AU: English-JC-3rd; Collins-M; Bryant-Bruce-C 

AD: Department of Primary Care and Community Medicine, USA MEDDAC, Ft.  Campbell, Kentucky 42223-5349, USA. 

SO: Int-J-Dermatol. 1995 Sep; 34(9): 642-4 

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DATA-MÉDICOS/DERMAGIC-EXPRESS No 2-(82) 11/12/99 DR. JOSÉ LAPENTA R. 

UPDATED 20 JUNE 2025

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Produced by Dr. José Lapenta R. Dermatologist

Venezuela 1.998-2.025

Producido por Dr. José Lapenta R. Dermatólogo Venezuela 1.998-2.025

Tlf: 0414-2976087 - 04127766810