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

Abstract

Degos disease is characterized by atrophic porcelain-white papules with peripheral erythema. Degos-like lesions have been reported in association with several connective tissue diseases (CTDs), mostly systemic lupus erythematosus (SLE), and rarely in dermatomyositis and systemic sclerosis. Herein, we report three cases of Degos-like lesions in CTDs. Two cases had Degos-like lesions linked to dermatomyositis. Both cases presented with severe inflammatory myopathy without pathognomonic cutaneous signs for dermatomyositis and lacked myositis-specific autoantibodies. Our third case, to our knowledge, is the first case of Degos-like lesions in association with overlapping systemic sclerosis and SLE without major organ involvement. The clinical presentation and histopathology findings of our cases support that Degos-like lesions may not be a specific entity but can be considered as a unique clinical pattern expressed in patients with CTDs.

A Rare Presentation of Systemic Lupus Erythematosus with Leg Ulcers and Degos-Like Lesions

“…[3][4][5] Till date 12 cases of DD with SLE have been reported, with majority in females. 6 Degos-like lesions have preceded other features of SLE by 2 years and 8 years in two cases. 7 One female presented with DD developed lupus nephritis during pregnancy 1-year later.…”

“…3 Others presented with concomitant or late-onset of Degos-like lesions by 1-7 years. 6 Malar erythema, photosensitivity, chronic cutaneous LE, bullous SLE, and arthritis were the clinical features of SLE reported with Degos-like lesions. 6 Our patient had oral ulcers, palmoplantar and leg ulcers, malar rash, along with Degos-like lesions.…”

An Elderly Female with Progressive Inflammatory Scar-Like Papules

“…The etiology of KDD remains largely unknown. [ 4 ] KDD may occur de novo in patients with no prior medical history or the setting of other autoimmune conditions, most frequently connective tissue diseases such as systemic lupus erythematosus. [ 4 ] Other disease associations include some inherited/acquired coagulopathies and viral infections.…”

“…[ 4 ] KDD may occur de novo in patients with no prior medical history or the setting of other autoimmune conditions, most frequently connective tissue diseases such as systemic lupus erythematosus. [ 4 ] Other disease associations include some inherited/acquired coagulopathies and viral infections. This particular case is an example of KDD occurring in the setting of antiphospholipid syndrome as evidenced by the patient's history of venous thromboembolism and elevated cardiolipin antibodies.…”

Degos disease: a case report and review of the literature

“…Degos disease (DD), also called “malignant atrophic papulosis” or “lethal cutaneous and gastrointestinal arteriolar thrombosis,” is a rare occlusive arteriopathy having unknown pathogenesis and involves predominantly small-caliber vessels of the dermis, gastrointestinal tract, central nervous system, and occasionally other organs [ 1 – 3 ]. Not more than 200 cases are reported in the literature [ 1 , 4 ].…”