Excerpt from Malignant Atrophic Papulosis

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Background

Kohlmeier described a case of malignant atrophic papulosis (MAP) as a form of thromboangiitis obliterans in 1941. It was recognized as a distinct clinical entity by Degos in 1942, hence the name. Since that time, 2 distinct clinical patterns have been recognized. A malignant variant affects multiple organ systems and results in death (most commonly from intestinal perforation) within a 2-year period. A benign form that is limited to the skin has a prolonged survival and low morbidity. A rare familial form has also been described, which also has a benign prognosis.

Pathophysiology

MAP is a multisystem disorder involving small-caliber blood vessels. The disease is characterized by narrowing and occlusion of the lumen by intimal proliferation and thrombosis, which leads to ischemia and infarction of the involved organ systems. MAP is different from other vasculitides in that inflammation is not a prominent component of the disease. MAP may involve the gastrointestinal and genitourinary tracts, central and peripheral nervous systems, skin, heart, lungs, eyes, pancreas, adrenals, and kidneys. The disease involves the skin alone in 37% of cases. The gastrointestinal tract is involved in about 50% of cases, and neurologic involvement occurs in approximately 20% of patients.

Frequency

International

This is a rare disease, with approximately 200 cases reported to date.

Most of the cases are sporadic, although a benign familial variant has been described. There have been approximately 30 reports involving 10 families. Only 4 of the 30 cases had systemic involvement.

Mortality/Morbidity

• The morbidity and mortality of MAP depend upon the extent of disease involvement. The benign cutaneous variant occurs in approximately 4-15% of cases. Most patients with the cutaneous-limited variant, who were monitored for over a decade, have not suffered significant morbidity. With systemic disease, the reported mean survival is approximately 2 years, but there is a wide variation, from less than 1 year to more than 12 years. The main causes of morbidity and mortality are bowel infarction, bowel perforation, CNS infarction and hemorrhage, and pleuropericardial disease. The benign and malignant variants are clinically indistinguishable initially but become distinct once systemic complications arise. Lack of systemic involvement at 2 years after diagnosis portends a better prognosis.

Race

Most cases of MAP reported from Europe and North America have been in whites. The disease also has been reported from Japan, India, and Africa.

Sex

MAP affects both sexes. A slight male predominance has been reported but has not been substantiated.

Age

The disease predominantly affects young adults, but cases have been described in infants and children.

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