Malignant form of atrophic papulosis with lethal abdominal involvement

Abstract

Atrophic papulosis (Kohlmeier–Degos disease or Degos disease) is a rare occlusive arteriopathy involving small-calibre vessels of the dermis, gastrointestinal tract, central nervous system (CNS) and occasionally other organs.\ud It would appear to be a vasculopathy or an endovasculitis, with a purely cutaneous benign variant and a systemic variant with cutaneous manifestations (malignant atrophic papulosis).\ud Gastrointestinal involvement is the most frequent and lethal systemic complication.\ud This is the most common cause of death, followed, less frequently, by CNS bleeding, and pleural or pericardial involvement

Disseminated Whitish Papules and Scars on a Patient With Bowel Perforation

“…1 It usually affects middle-aged persons, 2,3 although cases in children have been described. The disease has a male predominance (3:1), 1,4 with an earlier onset being reported in women. 2,5 The etiology of AP remains unknown.…”

“…1,3 Predisposing conditions associated with AP include parvovirus B19 infection, lupus erythematosus, dermatomyositis, scleroderma, and antiphospholipid syndrome. [4][5][6] The disease can affect the skin, gastrointestinal tract, and central nervous system, 2,3,6 with less common involvement of other organs. 3,5 Cutaneous lesions start as erythematous papules 7,8 that develop into porcelain-white scars surrounded by an erythematous rim with fine telangiectasias.…”

“…8 The latter description is considered pathognomonic for AP. [1][2][3][4][5] The lesions are usually asymptomatic 2,7 and are distributed predominantly across the trunk and extremities, sparing the scalp and palmoplantar region. 8 Systemic involvement can include bowel perforation, peritonitis, cerebral artery thrombosis, cerebral hemorrhage, among others.…”

A Case of Benign Atrophic Papulosis in a Young Male

“…Pediatric cases have also been reported 3 . The differences between MAP and BAP is shown in the Table 1 1 2 3 4 . MAP and BAP can be distinguished only by the presence of systemic disease in MAP, which has a median onset of 1 year (0.03~0.97 quantiles: 0~7 years) after cutaneous presentation and portends a poor prognosis 1 .…”

“…First line treatments include aspirin, pentoxifylline, dipyridamole, ticlopidine. Recently, treatment with eculizumab and treprostinil have demonstrated partial improvement 4 . When systemic involvements such as acute abdomen or cerebral artery thrombosis occurs, surgical operation and thrombolytic therapy are feasible.…”