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At least 19 recordsLinked to original sources
PubMedDate not supplied

Scleredema adultorum.

Scleredema adultorum is a rare connective tissue disorder reported usually following streptococcal infection, influenza, measles, and mumps. It has been reported occasionally following trauma and tuberculous lymphadenitis. This is a report of scleredema adultorum developing after chicken pox in an eight-year-old male child. The diagnosis was established by characteristic picture on skin biopsy using special stain. The patient had a benign course and a spontaneous recovery in two weeks. The case has been reported as the first case of scleredema adultorum developing after chicken pox.

Chickenpox↗
PubMed2005

Successful UVA1 phototherapy in a patient with scleredema adultorum.

Scleredema adultorum is a rare connective tissue disorder of unknown cause. Both bath-PUVA and cream-PUVA therapy were reported to be effective. We describe a patient with scleredema adultorum who showed a striking clinical improvement with a medium-dose UVA1 phototherapy (single dose, 50 J/cm(2); 35 treatments).

Humans↗
PubMed1980

Histologic observations of pleomorphic corynebacterium-like microorganisms in diabetic scleredema adultorum.

Diabetic scleredema adultorum is a rare connective tissue disorder characterized by scleroderma-like changes, usually affecting the skin of the neck, shoulders, and upper back in diabetics. Presented herein are two cases in which pleomorphic, but predominantly coccoid forms, were observed in acid-fast, Giemsa, and Gram-stained tissue, sections of the affected skin. Culture of the skin was positive for a microaerophilic, corynebacterium-like organism in one case and an anaerobic Propionibacterium (Corynebacterium) sp in the other case. The morphologic appearance of the bacterial isolates greatly resembled the morphologic forms observed in the tissue sections, suggesting that bacteria, possibly in a cell-wall-deficient phase, may play a role in the pathogenesis of this disorder. Findings of similar bacteria in previously reported cases of other connective tissue disorders such as sclerodermiformis, and rheumatoid arthritis, might support our idea that bacteria could supply the antigenic stimulus for the production of scleredema.

Adult↗
PubMed1998

Bath-PUVA therapy in three patients with scleredema adultorum.

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.

Humans↗
PubMed2004

Radiotherapy of benign diseases-scleredema adultorum Buschke.

BACKGROUND: Scleredema adultorum Buschke is a rare disorder characterized by thickening of the dermis of the neck, head and upper trunk. Its etiology is unknown, but there may be a preceding history of infection and there is a known association with diabetes mellitus. Women are more frequently affected. Usually, the disease is self-limiting but some patients show progressive disease. In these cases therapeutic options are poor, with only case reports and small series supporting their use. CASE REPORT: A 58-year-old patient with a scleredema of the neck and upper trunk is described, who was treated twice within 6 months by electron-beam radiation therapy. After the second course his symptoms improved significantly. A review of the literature of radiation treatment of this disease is given. CONCLUSION: . Regardless of the possible mechanisms in pathogenesis and treatment of scleredema adultorum Buschke, the application of ionizing radiation is an important, effective and well-tolerated therapy option in the treatment of severe cases and may candidate as the first-line treatment of this disease.

Humans↗
PubMed2002

[Scleredema adultorum in secondary hyperparathyroidism].

Scleredema adultorum, or Buschke's scleredema, belongs to the group of mucinoses. It is characterised by thickened and indurated skin. Histopathology shows thickened dermis with an infiltration of mucin between swollen collagen bundles. There are reports about many associations with scleredema adultorum, e.g., with diabetes mellitus and multiple myeloma. One case is known with associated primary hyperparathyroidism. For the first time we report a case of scleredema adultorum and secondary hyperparathyroidism, in a 46-year-old patient. Both forms of hyperparathyroidism have increased levels of parathormone. Therefore, these increased levels could have an influence on collagen metabolism.

Biopsy↗
PubMedDate not supplied

Scleredema adultorum associated with hyperkeratosis.

Scleredema adultorum is a rare skin disorder reported to occur usually following streptococcal infections. It has characteristic clinical and histopathologic findings; however, associated hyperkeratosis is not known. We report a 6-year-old girl with scleredema adultorum and hyperkeratosis. The patient's disease had a benign course. To our knowledge, this patient is the first reported with scleredema adultorum associated with hyperkeratosis.

Anti-Bacterial Agents↗
PubMed2006

Scleredema adultorum of Buschke: a case report and review of the literature.

OBJECTIVES: To present a case of scleredema adultorum of Buschke associated with hypergammaglobulinemia and review the literature pertaining to this disease. METHODS: Search of MEDLINE (PubMed) was performed using the words "Scleredema Adultorum." Only cases in the English language, with pertinent clinical information for analysis, were included. RESULTS: We present a case of scleredema associated with hypergammaglobulinemia. The skin findings had been progressive for 2 years before he was diagnosed with scleredema. Our patient was found to have a B-cell lymphoma before being diagnosed with scleredema. The progression of skin thickening halted with no apparent correlation to immunosuppressive therapies or chemotherapeutic agents. A total of 165 cases of scleredema adultorum are described in the literature. There are 3 types of scleredema adultorum. Type 1 is usually preceded by a febrile episode and resolves spontaneously. Type 2 is associated with developing paraproteinemias including multiple myeloma. Type 3 is associated with diabetes mellitus. CONCLUSIONS: Scleredema adultorum of Buschke is a rare disorder which is sometimes associated with hypergammaglobulinemia; our patient had a B-cell lymphoma. Physicians should suspect scleredema in any patient with diffuse skin thickening where the hands and feet are spared, particularly if diabetes or a preceding febrile episode were present. Once the diagnosis of scleredema is made, evidence for the presence of hypergammaglobulinemia should be sought.

Adult↗
PubMed1998

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

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.

Aged↗
PubMed2005

Scleredema adultorum of Buschke presenting as periorbital edema: a diagnostic challenge.

Scleredema adultorum is a rare sclerotic disorder characterized by diffuse swelling and nonpitting induration of the skin. Its occurrence has been documented in association with infections, diabetes mellitus, paraproteinemia, multiple myeloma, and monoclonal gammopathy. We report an unusual case of a 48-year-old man with an asymptomatic bilateral eyelid edema of sudden onset. During a period of 6 months, the condition slowly progressed to extensive nonpitting edematous swelling restricted to the periorbital sites. The presumptive diagnosis of scleredema adultorum was confirmed by the presence of typical histologic findings. This case is unique in that the periorbital swelling remained as the sole clinical manifestation of scleredema during the 5-year follow-up and was complicated with partial vision blockage.

Collagen↗
PubMed2003

Scleredema adultorum of Buschke treated with radiation.

BACKGROUND: Scleredema adultorum of Buschke is an uncommon skin condition that may accompany diabetes mellitus. Current therapeutic options are poor, with only case reports and small series supporting their use. OBSERVATION: We describe 3 patients with severe restrictive scleredema associated with type 2 diabetes mellitus who responded to radiation therapy after other treatments had failed. Angle of shoulder abduction was used as an objective measurement of improvement, while the patients rated several subjective areas, including arm range of motion, ease of performing daily tasks, ease of breathing, and neck thickening. Objective improvement tended to plateau over time but remained well above baseline. The duration of subjective improvement was variable, lasting from a few months to the end of the observation time of 2 1/2 years. CONCLUSION: Though not consistently durable in its effect, radiation therapy appears to be a viable therapeutic option in extreme cases of this difficult-to-treat disease.

Follow-Up Studies↗
PubMed1988

Cardiomyopathy and multiple myeloma. Complications of scleredema adultorum.

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.

Biopsy↗
PubMed1982

Scleredema adultorum of Buschke with upper esophageal involvement.

A 55-yr-old white female with biopsy proven scleredema adultorum was evaluated for progressive dysphagia. Esophageal motility studies revealed persistently high upper esophageal pressures without response to deglutition, and minimal peristalsis in the upper body of the esophagus. A cine-esophagram revealed massive aspiration of barium into the nasopharynx and lungs, concommitant with stenosis in the upper esophagus. The patient died presumptively from aspiration. This patient exemplifies a potentially lethal complication of scleredema adultorum i.e., upper esophageal infiltration with resultant aspiration.

Deglutition Disorders↗
PubMed1987

Scleredema adultorum associated with a monoclonal gammopathy and generalized hyperpigmentation.

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.

Histocytochemistry↗
PubMed1994

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

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.

Adolescent↗
PubMed2000

Scleroderma-like syndrome in a child: eosinophilic fasciitis or scleredema adultorum?

UNLABELLED: A 6-year-old female patient presented with a rapidly progressive scleroderma-like syndrome involving almost the entire integument. Initially clinical patterns and histopathological data of both eosinophilic fasciitis and scleredema adultorum were present. The course of the disease remained unusual for both conditions but finally argued in favour of the diagnosis of eosinophilic fasciitis. CONCLUSION: Eosinophilic fasciitis and scleredema adultorum might be subtypes of one disease entity.

Child, Preschool↗