Scleroderma Mimics

Abstract

Scleroderma is a rare systemic autoimmune disease with multiple organ manifestations, including skin fibrosis. The groups of disorders classified as scleroderma mimics share the common thread of skin thickening but are otherwise quite incongruous in terms of underlying disease process and other organ involvement. This article reviews the clinical presentation, etiology, and treatment options available for scleroderma mimics, including morphea, scleredema, diabetic cheiroarthropathy, scleromyxedema, nephrogenic systemic fibrosis, and eosinophilic fasciitis. Through greater understanding of these diseases and the associated extradermal implications, we hope to facilitate recognition of scleroderma and its mimics.

Therapy resistant idiopathic scleredema: an underlying pathology not always present

“…Biopsy is not always necessary for the diagnosis of scleredema (6). Biopsy of skin shows normal epidermis and no inflammation in dermis.…”

“…There is no consensual treatment for scleredema (6). Because the disease is self-limited in type 1, therapy is not necessary.…”

“…Some type 3 patients appear to improve with better glycemic control. Numerous other therapies including cyclosporin, methotrexate, high-dose penicillin, corticosteroids, prostaglandin E1, D-penicillamine, intralesional hyaluronidase, systemic photochemotherapy with PUVA, bath-PUVA, UVA-1 phototherapy, electron-beam radiotherapy, and extracorporeal photopheresis have been tried in case reports or small case series which ended with limited effect (1,2,(6)(7)(8)(9)(10). Prognosis is largely dependent on the underlying etiology.…”

A case of angioedema-like atypic scleromyxedema responding to treatment with steroid

“…Yüz bölgesinde deri kalınlaşması derin oluklar oluşturur ve tipik aslan yüz görünümüne neden olur. Deri kalınlaşmasına ikincil gelişen eklem kontraktürleri, miyopatik etkilenmeye bağlı gelişen özefagus hareket bozukluğu, yutkunma güçlüğü ve bazen eşlik eden ANA pozitifliği nedeniyle sistemik skleroz ile karışabilir 8 . Reynaud fenomeninin eşlik etmemesi, kalsinozis kutis yokluğu ve tırnak yatağı kapiller proliferasyonunun olmayışı skleromiksödem tanısını düşündürür 9 .…”

Eosinophilia in Rheumatologic/Vascular Disorders

“…63 Other entities including chronic graft-versus-host disease, nephrogenic systemic fibrosis, scleromyxedema, and scleredema can be differentiated from DFE based on clinical features and skin biopsy. 95 The differential diagnoses for skin thickening with eosinophilia include EMS and toxic oil syndrome. Pneumonitis or neuropathy can be seen in EMS.…”