Autoimmune Diseases Skin

Scleredema Adultorum Buschke

Scleredema Adultorum Buschke is not a form of scleroderma; but the skin involvement is similar.

Overview

Scleredema Adultorum Buschke is not a form of scleroderma, but it causes skin hardening that appears similar to systemic scleroderma. It is usually caused by diabetes, but it can also be caused by HIV, hyperparathyroidism, multiple myeloma, rheumatoid arthritis, and Sjogren's. (Also see Diabetes, Rheumatoid Arthritis, Sjogren's, and Scleroderma)

Scleredema. The initial skin changes of scleredema occur on the face, the neck, or the upper part of the back. Patients may report difficulty in smiling, in opening their mouths, and in wrinkling their foreheads. They may have limited range of motion. Medscape.

Scleredema Scleredema presents with symmetrical hardening and thickening of the dermal or middle layer of the skin with mucin deposits between collagen bundles. The diagnosis is confirmed by skin biopsy. DermNet NZ.

Case Report: A case of concurrent systemic sclerosis and scleredema (SAB). This case highlights multiple disease associations of SAB and the importance of high clinical suspicion in atypical disease presentations. PMC, JAAD Case Rep, 2019 Nov; 5(11): 940–942.

Case Reports: Scleredema of Buschke associated with lichen sclerosus. Both lichen sclerosus et atrophicus with scleredema adultorum of Buschke could be considered part of the spectrum of sclerodermoid disorders with common underlying pathogenetic mechanisms. PubMed, Indian J Dermatol Venereol Leprol. (Also see Lichen Sclerosus)

Treatments

For UVA1 treatment, first contact your doctor to see if it they will recommend it for you. It is not used for lichen sclerosus that is on the genital area. If they recommend and prescribe it for you, try to find UVA1 facilities near you. Some dermatology offices (usually large offices or with major health centers) have UVA1 equipment (not UVB). It may also be possible to buy UVA1 phototherapy equipment for home use. It is manufactured by Daavlin and distributed worldwide. Home users must be monitored by a physician, who will prescribe the necessary dosage and monitor for possible side-effects, such as skin cancer.

Scleredema Treatment and Management. Types 2 and 3 scleredema associated with a monoclonal gammopathy or diabetes typically does not regress spontaneously and no therapy is consistently effective. Most therapeutic successes described are limited to single case reports or small case series; there are no comparative data and no approved algorithm for scleredema treatment. Medscape.

Scleredema Treatments. Many different types of medications have been tried as treatments for scleredema, but no one medication has been proven to be best. Individuals may benefit from corticosteroids, cyclosporine, methotrexate, UVA1 phototherapy, or psoralen with ultraviolet light A. If infection is present, antibiotics may be required. If diabetes is present, it should be controlled with diet, exercise, and medication. Physical therapy may help if movement of body parts is restricted. VeryWell.

(Case Report) Scleredema treated with broad-band ultraviolet A phototherapy plus colchicine. We report the first case of scleredema that improved markedly with low-dose broad-band UVA plus colchicine treatment. PubMed, Photodermatol Photoimmunol Photomed.

Case Report: Scleredema adultorum successfully treated with narrow–band ultraviolet B phototherapy. We describe a case of scleredema adultorum successfully treated with narrow–band ultraviolet B and discuss a potential mechanism explaining its efficacy for fibrotic skin diseases. PubMed, Mod Rheumatol.

Personal Stories

Sue Ann: Mother of Son with Scleredema Since Christmas of 2000 the left side of my son's face thickened even more. Feburary 9 he was told he has cataracts in both eyes…

Wade: Scleredema vs Scleroderma At first look, my doctor thought I might have scleroderma, but after doing blood work, they said my tests came back negative…

Research for Scleredema adultorum Buschke

Sclerema neonatorum (SN): a review of nomenclature, clinical presentation, histological features, differential diagnoses and management. The histological features of skin biopsy should be used to establish diagnosis of SN, SCFN and scleredema as disease-specific treatment is imperative in SN due to high fatality. PubMed, J Perinatol.

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