Letter to the Editor
Two cases of Degos disease with different prognosis

Degos disease, also known as malignant atrophic papulosis, is a rare and often fatal multisystem microvascular disease, which is characterized by pauci-inflammatory thrombotic microangiopathy. The clinical symptoms consist of disseminated, red papules with central umbilication or atrophy, which may progress to lesions with central necrosis and a telangiectatic rim, leaving porcelain-like depressed scars. The gastrointestinal tract and central nervous system may also be involved in some patients with Degos disease [1]. In the current report, we describe two clinically and pathologically typical cases of Degos disease with completely different prognosis.
Case 1: A 73-year-old woman presented with an 18-month, full-body, recurrent eruption of macula lesions. The lesions were initially noticed on the dorsal surface of the hands. In over 2 weeks, these macules evolved into ulcers and healed, leaving central atrophic scars. Similar lesions also rapidly appeared over the trunk and extremities (Figures 1 A, B). This patient had no family history of similar symptoms. Blood differential results were normal. Liver and kidney values, and levels of C-reactive protein, immunoglobulin, C3, and C4 were also normal. Other laboratory examinations showed negative results for the presence of antinuclear, anti-extractable nuclear antigen, antinuclear cytoplasmic, and anti-2GP1 antibodies. We detected an index value of 45 RU/ml for anticardiolipin antibody in the serum (normal < 12 RU/ml). Abdominal ultrasonography and chest radiography were normal during the first visit. Pathological examination of a biopsy obtained from the skin on the back revealed wedge-shaped necrosis in the dermis (Figure 1 C). Lymphocyte infiltration in blood vessels surrounding necrotic areas and vascular thrombosis were observed (Figure 1 D). The lesions were partly controlled after 2 months of treatment with dipyridamole (75 mg/day) and aspirin (75 mg/day). The patient developed appendicitis and peritonitis during the next 6 months of follow up. Intravenous cyclophosphamide (600 mg/week for 2 months) was administered, but the patient died of a gastrointestinal tract perforation after 3 weeks.
Case 2: A 39-year-old Chinese woman was admitted to our hospital because of a 6-year history of recurrent maculopapules, which were distributed over the trunk and extremities. Physical examination of the skin revealed maculopapules (3–5 mm diameter) on the abdomen (Figure 2). Some lesions evolved...


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