Malignant atrophic papulosis (Köhlmeier-Degos disease) - A review

Abstract

Definition of the diseaseMalignant atrophic papulosis (MAP), described independently by Köhlmeier and Degos et al., is a rare, chronic, thrombo-obliterative vasculopathy characterized by papular skin lesions with central porcelain-white atrophy and surrounding teleangiectatic rim.EpidemiologyLess than 200 cases have been described in the literature. The first manifestation of MAP usually occurs between the 20th and 50th year of life.Clinical descriptionThe cutaneous clinical picture is almost pathognomonic. The histology is not consistent but in most cases it shows a wedge-shaped connective tissue necrosis in the deep corium due to a thrombotic occlusion of the small arteries. In the systemic variant, manifestations mostly occur at the intestine and central nervous system.EtiologyThe etiopathogenesis of the disease remains unknown, a genetic predisposition may occur. Vasculitis, coagulopathy or primary dysfunction of the endothelial cells have been implicated.Diagnostic methodsDiagnosis is only based on the characteristic skin lesions.Differrential diagnosisIt depends on the clinical presentation of MAP, but systemic lupus erythematosus and other connective tissue diseases need to be considered.ManagementNo effective treatment exists for the systemic manifestations, while compounds that facilitate blood perfusion have achieved a partial regression of the skin lesions in single cases.PrognosisAn apparently idiopathic, monosymptomatic, cutaneous, benign variant and a progressive, visceral one with approx. 50% lethality within 2–3 years have been reported. Systemic manifestations can develop years after the occurrence of skin lesions leading to bowel perforation and peritonitis, thrombosis of the cerebral arteries or massive intracerebral hemorrhage, meningitis, encephalitis, radiculopathy, myelitis.

Degos-Like Lesions Associated with Systemic Lupus Erythematosus

“…Immunosuppression with corticosteroids or other agents may worsen skin lesions and complications of Degos disease. Other therapies without proven benefits include antibiotics, arsenic, chloroquine, azathioprine, methotrexate, phenylbutazone, and interferon514. Our patient was treated with hydroxychloroquine, prednisolone, and cyclosporine for seven years, and with beraprost and pentoxifylline for two years for SLE.…”

“…First, classic Degos disease has characteristic skin lesions and affects other organs with multiple limited infarcts. Skin lesions are usually the first manifestation of the disease, although its poor prognosis is the result of gastrointestinal and CNS involvement5. With systemic involvement, the reported mean survival time is approximately 2 years, but it varies from less than 1 year to more than 12 years6.…”

Challenging Mimickers of Primary Systemic Vasculitis

“…Described in 1941 by Kohlmeier 90 and a year later by Degos and colleagues, 91 it is a rare entity with fewer than 200 cases reported in the literature to date. [90][91][92] Although its cause remains unknown, the condition has been described as a familial autosomal dominant disorder as well as in association with autoimmune conditions such as dermatomyositis. 93,94 The hallmark of the disease are characteristic skin lesions that appear as papules (0.5-1 cm) with a white, atrophic center and an erythematous rim, most commonly on the trunk and extremities (Fig.…”

“…Mortality from ischemic complications is high: 50% of patients succumb to the disease within 2 to 3 years. 92 Because there are no diagnostic laboratory findings, the diagnosis is typically confirmed with skin biopsy. There are no diagnostic serologic or other laboratory findings.…”

“…98 Dysfunction of endothelial cells and fibrinolysis has also been implicated in MAP. 92 Several therapies have been attempted in MAP but none has been widely successful to date, except in anecdotal cases. Success with antithrombotic agents and anticoagulants such as aspirin, dipyridamole, and heparin has been described in case reports.…”