EDITORIAL ESPANOL:

====================

Hola amigos de la red, en esta oportunidad DERMAGIC les presenta una pequeña revisión de la enfermedad: ESCLEREDEMA DE BUSCHKE, asociada a diabetes mellitus. La semana pasada fue requerida en la la lista DERMLIST de Brasil. Espero que estas 39 referencias nos ilustren bien el tema, incluidas las de 2024.

En el attach dos láminas ilustrativa: escleredema de Buschke, cuello, hombros y espalda.

Saludos,,,

Dr. José Lapenta R.,,,

 EDITORIAL ENGLISH:

===================

Hello friends of the net, in this opportunity DERMAGIC presents a small revision of the illness: SCLEREDEMA OF BUSCHKE, associated to Diabetes mellitus. Last week it was required in the the list DERMLIST of Brazil. I hope these 39 references illustrate us well the topic. including those of 2024.

In the attach two illustrative sheets: scleredema of Buschke, neck, shoulders and back.

Greetings,,,

Dr. José Lapenta R. 

=====================================================================

DERMAGIC/EXPRESS(63)

=====================================================================

ESCLEREDEMA DE BUSCHKE / SCLEREDEMA OF BUSCHKE

=====================================================================

REFERENCIAS BIBLIOGRÁFICAS / BIBLIOGRAPHICAL REFERENCES 

=====================================================================

A.- Scleredema Adultorum of Buschke: An Under Recognized Skin Complication of Diabetes.

B.- Screening for the Presence of Scleroedema Adultorum of Buschke in Patients With Diabetes Mellitus: Newly Diagnosed Patients Had a High Prevalence of Dyslipidaemia.

C.- Scleredema Adultorum of Buschke: A Case Report and Review of the Literature.

D.- Treatment of Scleroedema Adultorum Buschke: A Systematic Review.

E.- Systemic Involvement in Scleredema of Buschke Associated With IgG-kappa Paraproteinemia.

F.- Ultrasonographic Findings of Scleredema Adultorum of Buschke Involving the Posterior Neck.

G.- Radiotherapy of Benign Diseases-Scleredema Adultorum Buschke.

1.) Scleredema of Buschke: a report of seven cases. 

2.) In vivo study of skin mechanical properties in scleredema of Buschke. 

3.) Systemic involvement in scleredema of Buschke associated with IgG-kappa paraproteinaemia. 

4.) Scleredema of Buschke successfully treated with electron beam therapy. 

5.) Scleroderma-like disorders. 

6.) [Scleredema adultorum Buschke. Case report and review of the literature] 

7.) Scleredema diabeticorum of Buschke confined to the thighs. 

8.) [Scleredema, acanthosis nigricans and IgA/Kappa multiple myeloma] 

9.) Bath-PUVA therapy in three patients with scleredema adultorum. 

10.) Diabetic scleredema: a case report and biochemical analysis for

glycosaminoglycans. 

11.) Severe diabetic scleredema with extension to the extremities and

effective treatment using prostaglandin E1. 

12.) [Systemic Buschke's scleredema with cardiomyopathy, monoclonal IgG

kappa gammopathy and amyloidosis. Case report with autopsy].

13.) Demonstration of increased levels of type I collagen mRNA using

quantitative polymerase chain reaction in fibrotic and granulomatous skin

diseases.

14.) Increased collagen propeptides in the skin of a scleredema patient but no change in re epithelialisation rate.

15.) Scleroedema in a child.

16.) Biochemical characterization and tissue distribution of the scleredema in a case of Buschke's disease.

17.) Scleredema adultorum: case report and demonstration of abnormal expression of extracellular matrix genes in skin fibroblasts in vivo and in vitro.

18.) Scleredema revisited. A poststreptococcal complication.

19.) A fatal case of scleredema of Buschke.

20.) Electron-beam therapy in scleredema adultorum with associated monoclonal hypergammaglobulinaemia.

21.) Diabetic scleredema and scleroderma-like changes in a patient with maturity onset type diabetes of young people.

22.) Scleredema and smoldering myeloma.

23.) Acute scleredema with fatal outcome.

24.) Acid mucopolysaccharide staining in scleredema.

25.) Scleredema of Buschke associated with rheumatoid arthritis and

Sjogren's syndrome.

26.) [Post-traumatic scleroedema adultorum].

27.) Scleroedema diabeticorum: a minor but often unrecognized complication of diabetes mellitus.

28.) Cardiomyopathy and multiple myeloma. Complications of scleredema adultorum.

29.) Scleredema adultorum associated with a monoclonal gammopathy and generalized hyperpigmentation.

30.) Scleredema and paraproteinemia. Enhanced collagen production and elevated type I procollagen messenger RNA level in fibroblasts grown from cultures from the fibrotic skin of a patient.

31.) Paraproteinemia in patients with scleredema. Clinical findings and serum effects on skin fibroblasts in vitro.

32.) Biochemical characterization and tissue distribution of the scleredema in a case of Buschke's disease.

=====================================================================

=====================================================================

1.) Scleredema of Buschke: a report of seven cases. 

=====================================================================

Author 

Tate BJ; Kelly JW; Rotstein H 

Address 

Dermatology Unit, Alfred Hospital, Prahran, Vic, Australia. 

Source 

Australas J Dermatol, 37(3):139-42 1996 Aug 

Abstract 

Scleredema of Buschke is an uncommon dermatosis characterized by thickened,

indurated skin, sometimes with erythema. Histopathology shows thickened

dermal collagen with a mild infiltrate of mucin in the deeper dermis. Seven

adults with scleredema are presented, four females and three males, and

their mean age at diagnosis was 54 years. All had diabetes mellitus for an

average of 13 years prior to the onset of scleredema. Complications of

diabetes, especially retinopathy (n = 5), neuropathy (n = 4) and peripheral

vascular disease (n = 3), were present in five patients. One patient died

(cause not established), and another has life-threatening cardiomyopathy.

We have no evidence that the scleredema was a significant aetiologic factor

in either case, despite published reports of fatalities related to

scleredema. Three patients were followed up for more than 1 year and,

irrespective of therapy, the scleredema did not resolve in any patient. 

=====================================================================

2.) In vivo study of skin mechanical properties in scleredema of Buschke. 

=====================================================================

Author Dobrev H 

Address 

Department of Dermatology, Medical University, Plovdiv, Bulgaria. 

Source 

Acta Derm Venereol, 78(2):103-6 1998 Mar 

Abstract 

A non-invasive, in vivo suction device was used to investigate the

mechanical properties of the skin in a patient with scleredema of Buschke.

Clinical scoring of skin induration and measurements of skin elasticity

were performed over 9 anatomic regions on admission and after 3 (on

discharge), 17 and 28 months. Immediate distension, final distension and

immediate retraction were significantly decreased, while the viscoelastic

to elastic ratio was significantly increased in the patient as compared to

the healthy controls. Delayed distension and biological elasticity were

preserved. Low values of skin distensibility correlated with a severe skin

induration (p < 0.001). The changes were more expressive with the 8

mm-diameter measuring probe than the 2 mm-diameter probe. The method

applied can be used for objective and quantitative assessment of skin

involvement in scleredema of Buschke. 

=====================================================================

3.) Systemic involvement in scleredema of Buschke associated with IgG-kappa

paraproteinaemia. 

=====================================================================

Author 

Basarab T; Burrows NP; Munn SE; Russell Jones R 

Address 

Department of Dermatology, Ealing Hospital, Southall, Middlesex, UK. 

Source 

Br J Dermatol, 136(6):939-42 1997 Jun 

Abstract 

Scleredema is a rare primary cutaneous mucinosis. Systemic involvement is

uncommon and histological confirmation is often lacking. We report a case

of a 60-year-old man with scleredema and evidence of mucin deposition on

biopsies from multiple extracutaneous sites. The bone marrow, nerve,

hepatic and salivary gland involvement seen on histology in our patient has

not, to our knowledge, been previously reported in this condition. 

=====================================================================

4.) Scleredema of Buschke successfully treated with electron beam therapy. 

=====================================================================

Author 

Tamburin LM; Pena JR; Meredith R; Soong VY 

Address 

Department of Dermatology, University of Alabama at Birmingham, USA. 

Source 

Arch Dermatol, 134(4):419-22 1998 Apr 

=====================================================================

=====================================================================

5.) Scleroderma-like disorders. 

=====================================================================

Author 

Jablonska S; Blaszczyk M 

Address 

Department of Dermatology, Warsaw School of Medicine, Poland. 

Source 

Semin Cutan Med Surg, 17(1):65-76 1998 Mar 

Abstract 

Scleroderma-like disorders are widely disparate conditions mimicking either

systemic sclerosis or cutaneous localized scleroderma, not infrequently

displaying features of both. Some are exclusively sclerotic, some

scleroatrophic with prevailing sclerosis or atrophies. The recognition of

scleroderma-like disorders is of practical importance because by

establishing the cause of the disease, it is possible to introduce an

effective therapy, as in scleredema Buschke or scleredema diabeticorum,

sclerodermiform porphyria, Borrelia burgdorferi-induced sclerodermiform

acrodermatitis atrophicans, sclerodermiform phenylketonuria, drug-induced

conditions, and so on. Scleroderma-like disorders strongly suggest that the

pathogenesis of skin sclerosis and internal involvement may be divergent,

and of various causes. Some of them, such as atrophoderma Pasini-Pierini or

progressive facial hemiatrophy, frequently overlapping with scleroderma,

make the differentiation very difficult, if at all possible, and the

diagnosis is often arbitrary. Some, as sclerodermiform graft-versus-host

reaction, point to the autoimmune origin of scleroderma. The amply-covered

congenital sclerodermiform conditions present a large spectrum of still not

widely known and extremely heterogeneous syndromes, associated with

numerous anomalies and/or malignancies. 

=====================================================================

6.) [Scleredema adultorum Buschke. Case report and review of the literature] 

=====================================================================

Author 

Ulmer A; Schaumburg-Lever G; Bauer J; K¨otter I; Fierlbeck G 

Address 

Universit¨ats-Hautklinik T¨ubingen. 

Source 

Hautarzt, 49(1):48-54 1998 Jan 

Abstract 

Scleredema adultorum of Buschke is a rare disorder of unknown aetiology. It

is characterized by diffuse, non-pitting swelling and induration of the

skin. Skin biopsies reveal marked thickening of the dermis due to

collagenous replacement of the subcutis and deposition of hyaluronic acid

between the collagen fibers. The disease classically only affects the skin.

In 24 cases an associated monoclonal gammopathy has been detected. A

75-year-old patient had a 19-year history of scleredema adultorum. In

addition to a monoclonal gammopathy the patient suffered from involvement

of the tongue, pharynx and upper esophagus. Furthermore a polyneuropathy,

ocular involvement with restricted eye movements and a sicca syndrome were

present. The simultaneous occurrence of cutaneous scleredema with any one

of the above mentioned symptoms has been reported before. The wide variety

of extracutaneous manifestations of scleredema as found in our patient is

amazing and has not been previously described. 

=====================================================================

7.) Scleredema diabeticorum of Buschke confined to the thighs. 

=====================================================================

Author 

Farrell AM; Branfoot AC; Moss J; Papadaki L; Woodrow DF; Bunker CB 

Address 

Department of Dermatology, Charing Cross and Westminster Medical School,

London, U.K. 

Source 

Br J Dermatol, 134(6):1113-5 1996 Jun 

Abstract 

We describe a patient with insulin-dependent diabetes without vascular

complications in whom scleredema was confined to the thighs. Electron

microscopy demonstrated heterogeneity in both the size and density of the

collagen fibrils and the presence of filamentous material between them. 

=====================================================================

8.) [Scleredema, acanthosis nigricans and IgA/Kappa multiple myeloma] 

=====================================================================

Author 

Valente L; Velho GC; Farinha F; Bernardo A; Ribeiro P; Massa A 

Address 

Service de M´edecine, hospital Geral de Santo Ant´onio, Porto, Portugal. 

Source 

Ann Dermatol Venereol, 124(8):537-9 1997 

Abstract 

BACKGROUND: Scleredema is an uncommon disease of unknown origin.

Characteristic thick skin with symmetrical diffuse induration develops. The

infiltration begins on the face and neck then extends to the root of the

upper limbs and trunk. There are three clinical types of scleredema. The

first is preceded by an upper airway infection and progresses rapidly

before regressing spontaneously within a few months. The second type is

associated with chronic diabetes. The third type is associated with

monoclonal gammapathy, rarely of myelomatous type, and develops

insidiously. Acanthosis nigricans can be a paraneoplastic syndrome, often

associated with a gastrointestinal cancer. Few cases associating scleredema

and acanathosis nigricans have been reported. CASE REPORT: A 56-year old

woman had developed scleredema over the last 6 years when acanthosis

nigricans appeared together with IgA kappa multiple myeloma. Treatment with

melphalan and prednisolone was effective against the myeloma as well as the

scleredema and acanthosis nigricans. Discussion: Only five cases of

associated scleredema and multiple myeloma have been reported, four with

kappa IgG myeloma and one with IgA myeloma. An association between

acanthosis nigricans and sclerederma could be coincidental although the

fact that the different manifestations regressed together after the myeloma

treatment would suggest some relationship between these three diseases. 

=====================================================================

9.) Bath-PUVA therapy in three patients with scleredema adultorum. 

=====================================================================

Author 

Hager CM; Sobhi HA; Hunzelmann N; Wickenhauser C; Scharenberg R; Krieg T;

Scharffetter-Kochanek K 

Address 

Department of Dermatology, University of Cologne, Germany. 

Source 

J Am Acad Dermatol, 38(2 Pt 1):240-2 1998 Feb 

Abstract 

BACKGROUND: Scleredema adultorum (SA) is a rare connective tissue disorder

for which no treatment has proven to be effective. OBJECTIVE: Our purpose

was to determine the effect of bath-PUVA therapy on SA. METHODS: Three

patients were treated. Clinical evaluation of skin induration and thickness

as well as ultrasonography were performed at baseline and after treatment.

RESULTS: All three patients showed substantial clinical improvement with

bath-PUVA therapy (median of 59 treatments and a cumulative UVA dose of

245.7 J/cm2). Ultrasonography showed significant reduction in both skin

thickness and density. CONCLUSION: Bath-PUVA therapy appears to be

effective in the treatment of SA. 

=====================================================================

10.) Diabetic scleredema: a case report and biochemical analysis for

glycosaminoglycans. 

=====================================================================

Author 

Kobayashi T; Yamasaki Y; Watanabe T 

Address 

Division of Dermatology, Tokyo Daini National Hospital, Higashigaoka,

Meguro, Japan. 

Source 

J Dermatol, 24(2):100-3 1997 Feb 

Abstract 

We report a patient with the typical lesions of diabetic scleredema.

Histological findings of the involved skin were thickening of the dermis,

depositions of mucins, and fibrosis. Biochemical analysis revealed an

increase in glycosaminoglycans in the involved skin as well as in the

cutaneous lupus mucinosis. Mucinous materials were composed of hyaluronic

acid. 

=====================================================================

11.) Severe diabetic scleredema with extension to the extremities and

effective treatment using prostaglandin E1. 

=====================================================================

Author 

Ikeda Y; Suehiro T; Abe T; Yoshida T; Shinoki T; Tahara K; Nishiyama M;

Okabayashi T; Nakamura T; Itoh H; Hashimoto K 

Address 

Second Department of Internal Medicine, Kochi Medical School, Nankoku. 

Source 

Intern Med, 37(10):861-4 1998 Oct 

Abstract 

We report a 49-year-old woman with severe diabetic scleredema (DS). The

patient had non-insulin-dependent diabetes mellitus (NIDDM) for 9 years and

noticed thickened skin on her back 3 years previously. Her DS rapidly

extended to her back and extremities with pain and immobility. Her symptoms

of DS improved dramatically after establishing strict glycemic control and

intravenous administration of prostaglandin E1 (PGE1). However, the

histological findings of her skin biopsy did not change even after the

treatment for 12 weeks, and her symptoms worsened again after

discontinuation of glycemic control and PGE1 treatment. The causes of DS

have been considered to be metabolic abnormalities associated with

hyperglycemia and hypoxia in the skin due to diabetic microangiopathy. PGE1

was an effective treatment for DS in our patient. Strict control of

hyperglycemia and PGE1 treatment may be sufficient to manage DS, although a

very long treatment period is necessary. 

======================================================================

12.) [Systemic Buschke's scleredema with cardiomyopathy, monoclonal IgG

kappa gammopathy and amyloidosis. Case report with autopsy].

======================================================================

Medicina (B Aires) 1998;58(5 Pt 1):501-3 

Paz RA, Badra RE, Marti HM, Maxit MJ

Hospital Privado de Comunidad, Mar del Plata, Argentina.

hpc-idd@argenet.com.ar 

A 73 year old retired truck driver and blacksmith was studied in June 1996

for thoracic pain and was diagnosed as acute pericarditis which responded

well to steroid treatment. In January 1997, he noted swelling of the

abdominal skin, genitalia and limbs, sparing the feet. He was euthyroid,

did not have evidence of diabetes or a Raynaud's phenomenon. His

proteinogram showed an IgG-Kappa monoclonal paraprotein M component, 1.31

g/oo. TSH and tetraiodotironine were normal; ESR 16 mm in the first hour.

As he did not respond to treatment he was referred to our hospital in March

1997. On physical examination the most relevant findings were a non-pitting

edema of the abdomen and lower limbs, sparing the feet. An echocardiogram

was consistent with an infiltrative cardiomyopathy. Soon after his

hospitalization his condition worsened suddenly with severe bradicardia

(28/minute) due to a junctional rhythm and righ bundle branch block. He

suffered a cardiac arrest and died. The autopsy findings favoured the

diagnosis of systemic scleredema adultorum of Buschke. Amyloid deposits

were also found although not abundant, with a similar distribution except

in the skin. In this article the clinical and autopsy findings are

presented in a patient showing coexistence of systemic Buschke's scleredema

with an infiltrative cardiomyopathy, IgG Kappa gammopathy and amyloidosis. 

======================================================================

13.) Demonstration of increased levels of type I collagen mRNA using

quantitative polymerase chain reaction in fibrotic and granulomatous skin

diseases.

======================================================================

Br J Dermatol 1998 Jul;139(1):23-6 

Tasanen K, Palatsi R, Oikarinen A

Department of Dermatology, Oulu University Hospital, Finland. 

Collagen changes occur in localized scleroderma, scleredema and

sarcoidosis. Previous biochemical, immunohistochemical and in situ

hybridization studies have revealed increased collagen synthesis in these

diseases. In the present study, we measured by pro alpha 1 (I) collagen and

beta-actin mRNA levels in skin punch biopsy specimens from lesional and

healthy skin using a quantitative polymerase chain reaction (PCR). In this

method, the targeted mRNA and a synthetic RNA as a internal standard are

co-amplified together with the same primers. The amount of pro alpha 1 (I)

collagen mRNA in cutaneous sarcoidosis lesions was found to be increased

about two- to threefold compared with the values obtained for the healthy

skin of the same two patients. In lesional skin of three patients with

localized scleroderma the number of pro alpha 1 (I) collagen molecules was

increased about two-fold. The beta-actin mRNA values were at the same level

in the affected and unaffected skin of all the patients studied. In

conclusion, a marked increase in type I collagen gene expression was seen

in localized scleroderma and scleredema, leading to fibrosis of the skin,

and in a granulomatous skin disease, cutaneous sarcoidosis. 

======================================================================

14.) Increased collagen propeptides in the skin of a scleredema patient but

no change in re-epithelialisation rate.

======================================================================

Acta Derm Venereol 1996 Jul;76(4):305-9 

Haapasaari KM, Kallioinen M, Tasanen K, Risteli J, Palatsi R, Oikarinen A

Department of Dermatology, University of Oulu, Finland. 

Scleredema is a rare disease, affecting the skin connective tissue with

increased amounts of collagen and glycosaminoglycans. In the present study,

the collagen synthesis and re-epithelialisation rate were measured from a

64-year-old male patient, who rapidly developed extensive tightening of the

skin, without any underlying disease. The skin was thickened at several

sites when measured with ultrasound, and the histology revealed

accumulation of glycosaminoglycans and collagen bundles. The collagen

synthesis rate was measured from suction blisters induced on two different

sites of the skin before the treatment and three times later up to 6 months

after the treatment with a systemic steroid was started. The aminoterminal

propeptide of type I collagen (PINP) was increased manifold in the affected

skin when compared with the controls, indicating active collagen deposition

in vivo. Systemic steroid medication with high doses (over 20 mg/d)

decreased both the type I and the type III collagen propeptide levels. The

time schedule of the decreases in the propeptides in the thickened,

affected skin and in the clinically normal-looking skin varied, and

especially in the thickened skin in the abdomen the decrease in PINP was

noted only after 3 months of prednisolone therapy. When the prednisolone

dose was only 10 mg daily, the propeptides were again up-regulated, perhaps

reflecting the natural course of the disease. The re-epithelialisation

rates at two different sites of the patient were similar to those in the

controls, suggesting that even massive fibrosis with active deposition of

collagen does not alter the basal rate of re-epithelialisation in the skin.

In conclusion, collagen synthesis is markedly elevated in scleredema,

leading to fibrosis of the skin. A recently developed method utilizing

assays of collagen propeptides from suction blister fluid allows monitoring

of the collagen synthesis and detection of changes in the collagen

synthesis during the treatment of fibrotic disorders. 

======================================================================

15.) Scleroedema in a child.

======================================================================

J Dermatol 1996 Jul;23(7):495-8 

Mitsuhashi Y, Kondo S, Shimizu Y

Department of Dermatology, Yamagata University School of Medicine, Japan. 

A 3-year-old Japanese girl with scleroedema was reported. She had had no

signs of diabetes but did have a preceding bacterial infection in the

tonsils three weeks before the skin lesion appeared. The skin on the face,

shoulders, extensor aspect of the upper arms, and proximal half of the

forearms was indurated. The skin lesions expanded from the middle part of

the forearms to the wrists during the observation period. Thereafter, the

induration gradually disappeared. A literature review revealed there were

only six reports of scleroedema in children under 15 years old before 1996

in Japan; a total of 166 cases of the disease was reported in the same

period. Five out of these six cases were not diabetes-associated. All but

one of these six patients were female. Juvenile scleroedema seems to be

rare in Japan. 

======================================================================

16.) Biochemical characterization and tissue distribution of the scleredema

in a case of Buschke's disease.

======================================================================

Acta Derm Venereol 1987;67(3):193-8 

Roupe G, Laurent TC, Malmstrom A, Suurkula M, Sarnstrand B

Biopsies from a patient with a longstanding form of scleredema adultorum

Buschke were analysed for morphological and biochemical changes in the

dermal connective tissue. By light microscopy the tissue changes were

located to the deep part of the reticular dermis. Therefore dermal tissue

was separated into a superficial and a deep part and analysed

biochemically. By this procedure it was possible to show that the

concentration of hyaluronan in the deep part of the dermis was increased.

The urinary excretion of methylimidazole acetic acid, an indicator of the

mast cell mass in the body, was also elevated. 

======================================================================

17.) Scleredema adultorum: case report and demonstration of abnormal

expression of extracellular matrix genes in skin fibroblasts in vivo and in

vitro.

======================================================================

Br J Dermatol 1995 Jun;132(6):992-9 

Varga J, Gotta S, Li L, Sollberg S, Di Leonardo M

Department of Medicine, Jefferson Medical College, Thomas Jefferson

University, Philadelphia, PA 19107, USA. 

To elucidate the mechanisms involved in the development of cutaneous

fibrosis in scleredema adultorum, we studied a patient with long-standing

scleredema who had no history of diabetes mellitus or preceding febrile

illness. Histological examination of a biopsy specimen from involved

forearm skin demonstrated marked thickening of the dermis and accumulation

of mucin between collagen bundles. Increased levels of type I collagen

mRNA, as evidenced by positive in situ hybridization signals with an alpha

1(I) procollagen cDNA were found in numerous fibroblasts throughout the

dermis. The expression of several genes coding for proteins involved in the

maintenance of connective tissue was examined by determining in vitro

protein production and mRNA levels in fibroblasts from the affected skin.

Total protein production, glucosamine incorporation and collagen synthesis,

were elevated by 44-97% in scleredema fibroblasts, compared with

fibroblasts from two healthy individuals. Levels of mRNAs for alpha 1(I)

and alpha 1(III) procollagens and fibronectin were elevated in scleredema

fibroblasts, whereas mRNA levels for the tissue inhibitor of

metalloproteinase were unaltered compared with control cultures. The

results suggest that fibroblasts from the involved skin in non-diabetic

patients with scleredema may exhibit a biosynthetically activated

phenotype, which persists for several years. These alterations are likely

to be involved in the development of the cutaneous induration and

thickening which is characteristic of this disease. 

======================================================================

18.) Scleredema revisited. A poststreptococcal complication.

======================================================================

Clin Pediatr (Phila) 1994 Oct;33(10):606-10 

Cron RQ, Swetter SM

Department of Pediatrics, Lucille Salter Packard Children's Hospital at

Stanford, California. 

Scleredema is a rare connective disease which must be differentiated from

scleroderma in childhood. Scleredema is characterized by thickening of the

dermis of the neck, head, and upper trunk. We report a case of scleredema

in an 8-year-old boy with coincident streptococcal colonization. The

patient report demonstrates many of the common features of scleredema,

including an associated streptococcal infection, a relatively benign

presentation of illness, and the characteristic mucopolysaccharide

intradermal staining on skin biopsy. The literature on scleredema is

reviewed, focusing on the disease course, differential diagnosis, and an

overview of the proposed three subgroups of scleredema. The association of

scleredema to a prior streptococcal infection is explored, and a proposed

autoimmune pathophysiology of the disease, as it relates to streptococcal

infection, is presented. 

======================================================================

19.) A fatal case of scleredema of Buschke.

======================================================================

Br J Dermatol 1994 May;130(5):669-70 

Sansom JE, Sheehan AL, Kennedy CT, Delaney TJ

Department of Dermatology, Bristol Royal Infirmary, U.K. 

Scleredema of Buschke is a rare disorder characterized by the development

of areas of skin induration which usually resolve spontaneously. It is

occasionally associated with a benign gammopathy, and rarely with

myelomatosis. We describe a 60-year-old woman with extensive skin changes,

who developed IgA myeloma. Unusually, her skin disease did not respond to

conventional myeloma therapy. Death occurred as a consequence of the

progressive skin disease. 

======================================================================

20.) Electron-beam therapy in scleredema adultorum with associated

monoclonal hypergammaglobulinaemia.

======================================================================

Br J Dermatol 1994 Mar;130(3):394-7 

Angeli-Besson C, Koeppel MC, Jacquet P, Andrac L, Sayag J

Department of Dermatology, CHU la Timone, Marseille, France. 

We report a case of scleredema adultorum (Buschke's disease) associated

with an IgA kappa monoclonal hypergammaglobulinaemia. A significant

improvement in the skin was obtained with electron-beam therapy. Scleredema

would appear to be linked to monoclonal hypergammaglobulinaemia, but the

relationship between the skin disorder and the immunoglobulin abnormality

remains to be elucidated. 

======================================================================

21.) Diabetic scleredema and scleroderma-like changes in a patient with

maturity onset type diabetes of young people.

======================================================================

Dermatology 1994;188(3):228-31 

Iwasaki T, Kohama T, Houjou S, Iriuchijima T, Ohtsuka T, Ishikawa T,

Yamakage A, Mori M

Department of Internal Medicine, University School of Medicine, Maebashi,

Japan. 

A 21-year-old housewife with maturity onset type diabetes of young people

developed scleredema diabeticorum, scleroderma-like skin thickness on

forearms and dorsum of hands, digital sclerosis and cheiroarthropathy. She

had diabetes mellitus since the age of 11 years. Her grandfather on the

mother's side, her mother and 3 of 5 her mother's brothers and sisters have

diabetes mellitus. Blood glucose was 295 mg/dl. Urinary glucose was 5.3

g/day. Nail fold capillary microscopy revealed a progressive systemic

sclerosis pattern. Histologically, hematoxylin and eosin sections from back

and forearm skin demonstrated broad collagen bundles separated by widened

clear spaces throughout the thickened dermis. 

======================================================================

22.) Scleredema and smoldering myeloma.

======================================================================

J Am Acad Dermatol 1992 Feb;26(2 Pt 2):319-21 

Schmidt KT, Gattuso P, Messmore H, Shrit MA, Massa M, Welykyj S

Department of Dermatology, Loyola University Medical Center, Maywood, IL

60153. 

A 46-year-old white man had classic findings of scleredema; subsequently, a

monoclonal immunoglobulin G lambda light chain was detected in his serum.

Findings of a bone marrow biopsy specimen revealed that he had an increased

percentage of plasma cells, some of which were atypical. A diagnosis of

scleredema and smoldering myeloma was made. 

======================================================================

23.) Acute scleredema with fatal outcome.

======================================================================

J Assoc Physicians India 1990 Oct;38(10):807-8 

Shah PK, Lakhotia M, Jain SK, Meena RC

Department of Internal Medicine, Dr SN Medical College, Hospitals, Jodhpur. 

Death due to scleredema is rare. We report a patient with scleredema, which

had an acute onset and rapid progression, leading to death within a month.

Such an acute course terminating in fatality has not been described earlier. 

======================================================================

24.) Acid mucopolysaccharide staining in scleredema.

======================================================================

J Cutan Pathol 1990 Aug;17(4):211-3 

Cole HG, Winkelmann RK

Department of Dermatology, Mayo Clinic, Rochester, MN 55905. 

We studied 26 formalin-fixed biopsy specimens taken from 22 patients with

scleredema and found positive staining in 67% of the tissues when both

colloidal iron and alcian blue methods were used. Single stains were

positive in only half the tissues. Positive findings were noted in both

patients with diabetes (11 patients) and in those without it (11 patients).

Our experience indicates that multiple biopsies may be necessary to

demonstrate mucin in the dermis. Specimens obtained by incisional biopsy

are preferred. 

======================================================================

25.) Scleredema of Buschke associated with rheumatoid arthritis and

Sjogren's syndrome.

======================================================================

Br J Dermatol 1989 Oct;121(4):517-20 

Miyagawa S, Dohi K, Tsuruta S, Shirai T

Department of Dermatology, Nara Medical University, Japan. 

A case of scleredema of Buschke associated with rheumatoid arthritis and

Sjogren's syndrome is described. The onset of the skin changes and

rheumatoid arthritis was almost simultaneous and the sicca syndrome

developed 4 years later. 

======================================================================

26.) [Post-traumatic scleroedema adultorum].

======================================================================

Derm Beruf Umwelt 1989 Sep-Oct;37(5):176-8 

Bergner T, Przybilla B

Dermatologischen Klinik und Poliklinik, Ludwig-Maximilians-Universitat

Munchen. 

A 48-year-old male developed an induration of the skin of the neck a few

days after a blunt trauma to this region had occurred. A diagnosis of

scleredema adultorum (Buschke) could be established 3 years later. In view

of the history and the lack of other diseases thought to be related to the

elicitation of scleredema, a traumatic origin of the skin condition is

probable. 

======================================================================

27.) Scleroedema diabeticorum: a minor but often unrecognized complication

of diabetes mellitus.

======================================================================

Diabet Med 1988 Jul-Aug;5(5):465-8 

Sattar MA, Diab S, Sugathan TN, Sivanandasingham P, Fenech FF

Department of Medicine, Faculty of Medicine, Kuwait University, Safat. 

The association of specific dermatological complications with diabetes

mellitus is well recognized. Of 100 hospital-based patients with diabetes

mellitus (age 48 years +/- 2SE), 14% had scleroedema diabeticorum. The

affected subjects had a higher prevalence of retinopathy (p less than

0.001) and albuminuria (p less than 0.025). The duration of scleroedema

correlated with the duration of diabetes (p less than 0.005). These

findings highlight the relatively common occurrence of this skin condition

which often goes unrecognized in people with diabetes. 

======================================================================

28.) Cardiomyopathy and multiple myeloma. Complications of scleredema

adultorum.

======================================================================

Arch Intern Med 1988 Mar;148(3):551-3 

Rimon D, Lurie M, Storch S, Halon D, Eisenkraft S, Laor A, Cohen L

Department of Medicine B, Carmel Hospital, Haifa, Israel. 

Multiple myeloma and congestive heart failure developed in a patient with

long-standing scleredema adultorum. Staining of the myocardium, performed

after her death, was positive for acid mucopolysaccharide and negative for

amyloid. To the best of our knowledge, this is the first case in which acid

mucopolysaccharide has been demonstrated in the myocardium, thus explaining

the cardiomyopathy of scleredema adultorum. Review of the world literature

enabled us to identify a statistically significant increased prevalence of

plasma cell dyscrasia among patients with protracted scleredema. In all

patients, plasma cell dyscrasia appeared years after the onset of

scleredema. Immunofluorescent studies were negative for immunoglobulin

deposition. We assume, therefore, that the plasma cell dyscrasia was

secondary to scleredema. 

======================================================================

29.) Scleredema adultorum associated with a monoclonal gammopathy and

generalized hyperpigmentation.

======================================================================

Arch Dermatol 1987 May;123(5):629-32 

McFadden N, Ree K, Soyland E, Larsen TE

Scleredema associated with a monoclonal gammopathy and generalized skin

pigmentation is described in a 56-year-old man with hyperlipoproteinemia

and cardiovascular disease. The patient had IgG-lambda paraproteinemia,

without any evidence of multiple myeloma or immunoglobulin deposition in

affected skin. Ultrastructural studies of pigmented lesional skin showed

increased transfer of melanosomes to basal keratinocytes and dermal

melanophages containing complex melanosomes. In addition, cytoplasmic,

electron-opaque lipid droplets were seen in approximately every third

keratinocyte or melanocyte, while only an occasional dermal cell contained

lipid droplets. The hyperpigmentation appeared to be directly related to

the scleredema, while the lipid deposition in skin was a likely consequence

of the hyperlipoproteinemia. The findings further support the contention

that paraproteinemia and hyperpigmentation may, in some patients, be

associated features of scleredema adultorum. 

======================================================================

30.) Scleredema and paraproteinemia. Enhanced collagen production and

elevated type I procollagen messenger RNA level in fibroblasts grown from

cultures from the fibrotic skin of a patient.

======================================================================

Arch Dermatol 1987 Feb;123(2):226-9 

Oikarinen A, Ala-Kokko L, Palatsi R, Peltonen L, Uitto J

An edematous rash developed on the abdominal skin of a 76-year-old woman

who had had diabetes mellitus for ten years. Some months later, the

affected skin became thickened and indurated. Histopathologic examination

revealed marked dermal fibrosis with excessive deposition of collagen. The

patient also had IgA (k-type) paraproteinemia. Fibroblast cultures from the

affected and unaffected skin were studied for collagen metabolism.

Procollagen synthesis was elevated about sixfold on fibroblasts derived

from the affected skin. A similar increase was detected in messenger RNA

(mRNA) levels using a complementary DNA clone specific for human pro alpha

1(l) collagen mRNA. The elevated mRNA level could be the result of

increased transcriptional activity of collagen genes or decreased

degradation of collagen mRNAs. Our findings suggest that increased collagen

deposition may account for the marked dermal fibrosis that we observed in

this patient. 

======================================================================

31.) Paraproteinemia in patients with scleredema. Clinical findings and

serum effects on skin fibroblasts in vitro.

======================================================================

J Am Acad Dermatol 1987 Jan;16(1 Pt 1):96-107 

Ohta A, Uitto J, Oikarinen AI, Palatsi R, Mitrane M, Bancila EA, Seibold

JR, Kim HC

Four patients with paraproteinemia and scleredema were studied. Histologic

features included marked thickening and fibrosis of the dermis and

subcutis. Variable amounts of mucin deposits were detected in the

interfibrillar spaces. Serum from one patient significantly stimulated

collagen production in normal skin fibroblast cultures, whereas serum from

another patient stimulated collagen production in autologous cell cultures.

Moreover, serum from one patient stimulated the [35S]sulfate incorporation

into the fibroblasts. Circulating serum factors, possibly related to the

paraprotein, may enhance the synthesis of extracellular macromolecules by

dermal fibroblasts in these patients, thus providing a mechanism for dermal

fibrosis. 

======================================================================

32.) Biochemical characterization and tissue distribution of the scleredema

in a case of Buschke's disease.

======================================================================

Acta Derm Venereol 1987;67(3):193-8 

Roupe G, Laurent TC, Malmstrom A, Suurkula M, Sarnstrand B

Biopsies from a patient with a longstanding form of scleredema adultorum

Buschke were analysed for morphological and biochemical changes in the

dermal connective tissue. By light microscopy the tissue changes were

located to the deep part of the reticular dermis. Therefore dermal tissue

was separated into a superficial and a deep part and analysed

biochemically. By this procedure it was possible to show that the

concentration of hyaluronan in the deep part of the dermis was increased.

The urinary excretion of methylimidazole acetic acid, an indicator of the

mast cell mass in the body, was also elevated. 

====================================================================

DATA-MÉDICOS/DERMAGIC-EXPRESS No (63) 30/06/99 DR. JOSE LAPENTA R. 

====================================================================

Produced by Dr. José Lapenta R. Dermatologist
Venezuela 1.998-2.024

Producido por Dr. José Lapenta R. Dermatólogo Venezuela 1.998-2.0024

Tlf: 0414-2976087 - 04127766810