Malignant atrophic papulosis

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports

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Atrophic papulosis, also known as Köhlmeier-Degos disease or Degos disease, is a rare occlusive vasculopathy for which treatment is challenging. Atrophic papulosis occurs in three distinct clinical contexts: malignant atrophic papulosis (MAP); benign atrophic papulosis (BAP); and atrophic papulosis in the context of autoimmune connective tissue disease.

MAP is associated with significant gastrointestinal (GI) and neurologic disease. GI involvement occurs in half of patients with MAP and can manifest as abdominal pain, indigestion, constipation, or diarrhea. Central nervous system involvement manifests as headaches, dizziness, paresthesias, and seizures. Ocular, hepatorenal, and cardiopulmonary systems may also be involved. BAP is a skin-limited disease without systemic symptoms and is a retrospective diagnosis that requires long-term follow-up, as the cutaneous findings usually precede systemic manifestations. The probability of only having BAP at onset is approximately 70% but increases to 97% after 7 years of skin-limited disease. Systemic disease in MAP has a median onset of 1 year after cutaneous findings. The 5-year survival rate of MAP is 55%, and the most common cause of death is peritonitis secondary to intestinal perforation. Lastly, atrophic papulosis may be observed in patients with systemic lupus erythematosus, antiphospholipid syndrome, dermatomyositis, or systemic sclerosis, without the potentially catastrophic neurologic or GI disease associated with MAP.

Early, fully developed, and late papules demonstrate a spectrum of overlapping phase-dependent changes. Early papules are skin colored with a lymphocytic infiltrate. Established papules demonstrate porcelain-white centers and erythematous peripheral rims. Dermoscopy demonstrates white stellate structureless areas surrounded by telangiectasia, hyperpigmentation, and follicular plugs. Fully developed lesions demonstrate vasculitis and superficial changes of lichen sclerosus. Late papules feature atrophy without erythema, corresponding to the classic histopathology: wedge-shaped dermal necrosis and thrombotic vasculopathy.

MAP has been reported in association with factor V Leiden, antiphospholipid and anticardiolipin antibodies, and parvovirus B19 infection. In the past decade, evidence of high expression of interferon-α and excessive deposition of late-stage complement components C5b-9 in diseased vessels provided deeper insight into the pathogenesis of this vasculopathy and identified the most effective therapy, eculizumab .