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Malignant Atrophic Papulosis at a Glance
  • A rare, primary vaso-occlusive disorder, affecting mainly the skin, the gastrointestinal tract, and the central nervous system.
  • Diagnosis relies on clinicopathologic evaluation.
  • Characterized by numerous typical porcelain-white, atrophic papules, with a rim of rosy erythema and/or telangiectases.
  • Pathology shows a wedge-shaped area of dermal necrosis with edema and mucin deposition.
  • Benign forms without extracutaneous involvement are possible.

In the early 1940s, Köhlmeier first described malignant atrophic papulosis, whereas Degos, Delort, and Tricot recognized it as a specific entity a year later.13 We now know that the clinically distinctive lesion of the so-called Degos disease is a marker of a cutaneous thrombo-obliterative vasculopathy rather than of a specific disease per se. Indeed, such lesions can be found in at least two distinctive clinical settings: (1) as an apparent idiopathic disease, either classic Degos disease or its benign variant, or (2) as a surrogate clinical finding in some connective tissue diseases such as the antiphospholipid syndrome, lupus erythematosus, dermatomyositis, and systemic sclerosis.

Classic Degos disease is rare, with about 200 reported cases. It almost always occurs in Caucasians, but cases have been observed in African-American patients and in Japan. The disease most commonly presents between the third and fourth decades, but can occur at any age. Men are more often affected than women (ratio, 3:1). The majority of cases are sporadic, but familial cases have been described, and most of these cases are consistent with an autosomal dominant pattern of inheritance.4

The etiology of Degos disease is unknown. The histopathologic findings in patients with malignant atrophic papulosis suggest a primary vaso-occlusive process. Thus, a vascular coagulopathy and/or endothelial cell damage should be considered as the major pathogenic mechanism. A combination of prothrombotic factors possibly plays a role in triggering the full-blown disease. Extensive studies of prothrombotic factors were performed in some patients with Degos disease, and no single abnormality was repeatedly identified. All patients should be screened for the presence of antiphospholipid antibodies/lupus anticoagulant and cryoglobulins, although Assier et al did not find the former in their series of 15 patients.5 Inhibition of fibrinolysis and platelet abnormalities, including increased platelet adhesiveness and spontaneous aggregation, were reported in some patients.58

The lesions of Degos disease should be viewed as a cutaneous marker indicative of intraluminal thrombosis. Several disease processes may converge to produce those clinical and histologic findings.

Figure 171-1

Approach to the patient with malignant atrophic papulosis.

History

Patients seek medical advice for the appearance of small cutaneous lesions that usually are neither pruritic nor painful. In some patients, history and/or review of systems will reveal signs indicative of extracutaneous involvement: abdominal pain, diarrhea, melena, nausea, blurred vision, hemiparesis, paresthesia, or ...

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