Abstract

A 38-year-old Caucasian female presented to dermatology for a two-year history of a rash on the trunk and extremities with associated joint pain. Two punch biopsies performed by an outside dermatologist were suggestive of tumid lupus. Physical exam revealed scattered discrete, umbilicated atrophic, porcelain-white papules with telangiectatic and erythematous borders involving her trunk, arms, and thighs. Histologic examination demonstrated abundant mucin within the superficial and deep dermis and subcutis with a superficial and deep perivascular and periadnexal lymphohistiocytic infiltrate. In light of the striking clinical findings, a diagnosis of Degos disease or Degos-like lesions in the setting of lupus erythematosus was made. Subsequent workup for lupus was negative. Careful clinical-pathologic correlation is important to the diagnosis of Degos disease as patients can have systemic manifestations with poor outcomes. Several case reports have noted Degos-like lesions in patients with lupus erythematosus and dermatomyositis and some authors postulate that Degos disease may represent a distinctive pattern of disease, rather than a distinct entity. While some cases are benign and exhibit only cutaneous lesions, patients must be followed for development of gastrointestinal and neurologic involvement which can be fatal. Treatment of Degos disease is challenging, as no effective therapy has been identified. Eculizumab which blocks complement component 5, has shown promise in severe disease. Antithrombotic agents, anticoagulants, pentoxifylline, ticlopidine, prostaglandin E1, interferon alpha-2a, and IVIG have been used with variable success. Given the possibility of life-threatening multisystem disease, interdisciplinary coordination is crucial. To date, our patient has had a benign course with skin limited disease.

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