Degos' disease mimicking vasculitis

Degos' disease mimicking vasculitis.

Introduction Degos’ disease is a rare disorder with multisystem involvement and unknown etiology. This entity was first described by Degos in 1942 (1,2). Other synonyms for this disease are malignant atrophic papulosis, atrophic papulosquamous dermatitis, fatal cutaneous-intestinal syndrome, and thromboangiitis cutaneointestinalis disseminata (3). It has been more commonly reported in whites, m...

Degos disease mimicking primary vasculitis of the CNS

A 4-year-old boy developed a headache. Initial evaluation revealed a normal neurologic examination and a right subdural hygroma on CT. Worsening headaches led to hospitalization at an outside institution. MRI showed leptomeningeal enhancement; magnetic resonance angiography (MRA) was normal. Infectious, rheumatologic, hematologic, and CSF studies were unrevealing. He then developed a left-sided...

[Dowling-Degos disease].

Dowling-Degos disease.

Dowling-Degos disease (DDD) is a rare autosomal dominant inherited pigmentary disorder of the flexures with a reticulate aspect and with presence of prominent comedone-like lesions and pitted scars. The diagnosis includes acanthosis nigricans as well as other reticulate pigmentary disorders classified into: dyschromatrosis symmetrica hereditaria (DSH), dyschromatosis universalis hereditaria (DU...

Benign cutaneous Degos disease

A 41-year-old woman presented with a 2-year history of multiple asymptomatic pea-sized papules with an erythematous, telangiectatic rim surrounding an atrophic, porcelain-white center on the trunk and extremities (Figure 1). She had experienced abdominal fullness and vomiting for 1 year. Histopathologic examination of a skin biopsy from the left forearm revealed hyperkeratosis, epidermal atroph...