J Rheum Dis.  2011 Dec;18(4):324-326. 10.4078/jrd.2011.18.4.324.

A Case of Scleredema Associated with Dermatomyositis

• Park JH
• Lee SH
• Lee JH
• Hong SC
• Hong MJ
• Park E
• Han JS
• Lee CI
• Kim SM
• Kim HR

• KMID: 2223140
• DOI: http://doi.org/10.4078/jrd.2011.18.4.324

Abstract

Dermatomyositis (DM) is rare systemic inflammatory disease with typical skin manifestations and muscular involvement. Various skin lesions can accompany this disease, such as Gottron's sign, Heliotrope rash, mechanic's hands, V sign and shawl sign. Scleredema is a very rare skin manifestation in DM. We report a case of DM in a 63-year-old woman, who had scleredema on her thighs. A diagnosis of DM was established by clinical manifestation, elevated muscle enzyme levels, electromyogram measures, and muscle biopsy findings. She was successfully treated with the immunosuppressants methotrexate, cyclosporine, and steroids (low dose).

Keyword

Dermatomyositis; Scleredema; Immunosupressants

MeSH Terms

Biopsy
Cyclosporine
Dermatomyositis
Exanthema
Female
Hand
Humans
Immunosuppressive Agents
Methotrexate
Middle Aged
Muscles
Scleredema Adultorum
Skin
Skin Manifestations
Steroids
Thigh
Cyclosporine
Immunosuppressive Agents
Methotrexate
Steroids

Figure

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  Figure 1. Skin lesions shows (A) erythema and edema of eyelids (Heliotrope rash), (B) diffuse erythematous rash on the anterior chest, neck, upper back (V sign) and (C) diffuse erythematous swelling and scleroderma change on thigh.

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  Figure 2. Skin biopsy shows that thickened collagen with increased interstitial space throughout reticular dermis and normal epidermis and fat tissues. (A) H-E stain (×100) demonstrated copious amounts of interstitial mucin. (B) Alcian blue stain (×400).

Reference

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