Degos-Like Lesions Associated with Systemic Lupus Erythematosus

Abstract

Degos disease, also referred to as malignant atrophic papulosis, was first described in 1941 by Köhlmeier and was independently described by Degos in 1942. Degos disease is characterized by diffuse, papular skin eruptions with porcelain-white centers and slightly raised erythematous telangiectatic rims associated with bowel infarction. Although the etiology of Degos disease is unknown, autoimmune diseases, coagulation disorders, and vasculitis have all been considered as underlying pathogenic mechanisms. Approximately 15% of Degos disease have a benign course limited to the skin and no history of gastrointestinal or central nervous system (CNS) involvement. A 29-year-old female with history of systemic lupus erythematosus (SLE) presented with a 2-year history of asymptomatic lesions on the dorsum of all fingers and both knees. The patient had only skin lesions and no gastrointestinal or CNS vasculitis symptoms. Her skin lesions were umbilicated, atrophic porcelain-white lesions with a rim of erythema. On the basis of clinical, histologic, and laboratory findings, a diagnosis of Degos-like lesions associated with SLE was made. The patient had been treated for SLE for 7 years. Her treatment regimen was maintained over a 2 month follow-up period, and the skin lesions improved slightly with no development of new lesions.

<p>Degos-Like Lesions In Association With Connective Tissue Diseases: A Report Of Three Cases And Literature Review</p>

“…For the resolution phase, typical lesions marked by porcelain-white scars with surrounding erythema. Lastly, only white scars with irregular border remain 3,8. The character of Degos-like lesions is indistinguishable from classic Degos disease.…”

Long-Term Follow-Up of A Patient with Juvenile Dermatomyositis and Degos-Like Disease

“…5 Classic Degos' disease features typical skin lesions and multiple limited infarcts in other organs, such as bowel and central nervous system; approximately 15% of the cases are a benign form often limited to the skin. On the other hand, Degos-like lesions occur in patients with underlying diseases: SLE, antiphospholipid syndrome, SS, RA, [3][4][5] and DM/JDM. 4,7−13 In both types, skin lesions are distinctively characterized by diffuse papules with porcelain-white centers and slightly raised erythematous telangiectatic rims.…”

“…4,7−13 In both types, skin lesions are distinctively characterized by diffuse papules with porcelain-white centers and slightly raised erythematous telangiectatic rims. 1,5 On histopathology, the lesions demonstrate hyperkeratosis, epidermal atrophy, and an underlying wedge-shaped area of dermal ischemia and necrosis with a prominent lymphocytic in ltrate; vessels in the papillary dermis show brinoid necrosis and thrombosis. Mucin deposition is seen in all stages, and brin deposition may be also observed.…”

“…Several authors suggest Degos' disease actually represents a clinical and histological endpoint reaction pattern to vascular injury derived from different disease processes, instead of a distinct clinical entity; which could explain its association with autoimmune disorders. [3][4][5] There are only 10 previously reported cases of DM with Degos-like disease, 4,7−13 7 of them in adult patients (3 males and 4 females), with a median age at diagnosis of 37 years (20 to 50 years) and a fatal outcome in 4 (57%), as can be seen in Table 1. In this group of patients, cutaneous lesions typical of Degos were observed in 5 cases.…”

“…1 Degos-like disease has been associated with several rheumatologic conditions such as systemic lupus erythematosus (SLE), antiphospholipid syndrome, systemic sclerosis (SS) and rheumatoid arthritis (RA). [3][4][5] Herein we describe a patient with juvenile dermatomyositis (JDM) associated with Degos-like disease.…”