TOP DERMATOLOGIST TALKS ABOUT A RARE DISORDER OF DEGOS’ DISEASE

November 27, 2018drikram Case Studies - Best dermatologist in Islamabad Prof. Dr. Ikram Ullah Khan

Case Report Degos’ Disease: A Rare Disorder

Prof. Dr. Ikram Ullah-Khan, Saima Rafiq Department of Dermatology, Pakistan Institute of Medical Sciences, Islamabad : Degos’ Disease Case Report

Abstract

Degos’ disease, also known as malignant atrophic papulosis, is an uncommon multisystem lymphocytic vasculitis characterized by widespread thrombosis of small vessels affecting the skin, gastrointestinal, ocular, and central nervous systems. We present a case of Degos’ disease in a middle-aged woman. The diagnosis was established based on clinical presentation and histopathological findings.

Introduction

Degos’ disease, or malignant atrophic papulosis, is a progressive vasculopathy that leads to occlusion of small and medium-sized arteries. The disease manifests through skin and gastrointestinal lesions, with neurological features also being common. Skin lesions typically appear first and may remain the only symptom for several years. The etiology of Degos’ disease remains unclear, though it is characterized by vasculitis and thrombotic tendencies. Histopathologically, early lesions exhibit a chronic inflammatory cell infiltrate around vessels, while later stages show characteristic wedge-shaped sclerotic changes in the dermis.

Case Report

A 50-year-old woman presented with a one-year history of pink-colored papular lesions on her legs. The lesions began as small papules on her thighs and gradually spread to her lower legs and abdomen. Some lesions had central atrophy and white scars. Skin biopsy showed dermal necrosis in a wedge-shaped pattern and thickened vessel walls. Laboratory investigations were within normal limits. The patient did not exhibit any involvement of other systems.

Figure 1: Multiple papular lesions over legs. Few of them show central atrophy

Figure 2: A close-up showing central atrophy and porcelain-white scales

Discussion

Degos’ disease, first described by Kohlmeier in 1941 and further recognized by Degos in 1942, is a rare condition with around 150 reported cases in the literature. This vasculopathy results in occlusion of small and medium-sized arteries, causing tissue infarction. It can manifest as a benign cutaneous form or a lethal multiorgan systemic variant. The skin lesions are pathognomonic, presenting as pink or yellow papules that heal with a central porcelain-white zone of atrophy. Systemic involvement, particularly in the gastrointestinal tract, is common in the systemic variant. Neurologic and ocular manifestations can also occur.

The exact cause of Degos’ disease is not fully understood, but it is considered a vasculopathy or endovasculitis. Different mechanisms have been proposed, including disturbances in immunity, viral infections, and abnormalities in the blood clotting system. While some cases show antiphospholipid antibodies, evidence for antibodies against endothelial cell components is lacking. The disease primarily affects adults, with a male-to-female ratio of around 3:1.

Treatment options are limited, and no successful medical therapy exists. Antiplatelet drugs such as aspirin may reduce new lesion formation in cases with only skin involvement. Other treatments include topical corticosteroids, pheniormin, iodohydroxyquinoline, sulphonamides, heparin, azathioprine, methotrexate, cyclosporine, tacrolimus, and pentoxifylline.

References:

(Please note that references are included in the original text and can be referenced as needed.)

Address for Correspondence: Dr. Ikram Ullah Khan, Department of Dermatology, Pakistan Institute of Medical Sciences, Islamabad.