Malignant Atrophic Papulosis

Updated: Jun 17, 2016
  • Author: L Campbell Levy, MD; Chief Editor: BS Anand, MD  more...
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Overview

Background

Kohlmeier described a case of malignant atrophic papulosis (MAP) as a form of thromboangiitis obliterans in 1941. [1] It was recognized as a distinct clinical entity by Degos in 1942, hence the name. [2] 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 been described, which also has a benign prognosis.

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Pathophysiology

Malignant atrophic papulosis 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. Malignant atrophic papulosis is different from other vasculitides in that inflammation is not a prominent component of the disease. Malignant atrophic papulosis 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. [3]

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Etiology

The etiology of malignant atrophic papulosis is unknown. Autoimmune, hypersensitivity, viral, and genetic factors leading to endothelial dysfunction, small vessel vasculitis, or a coagulopathy have all been implicated. None has been confirmed or is supported by strong evidence. It has also been theorized that malignant atrophic papulosis may not be a distinct disease but rather several processes that converge to produce characteristic clinical and histologic findings.

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Epidemiology

International data

Malignant atrophic papulosis 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 (13%) had systemic involvement.

Race-, sex-, and age-related demographics

Most cases of malignant atrophic papulosis reported from Europe and North America have been in white individuals. The disease has also been reported from Japan, India, and Africa.

Malignant atrophic papulosis affects both sexes. A slight male predominance has been reported but has not been substantiated.

The disease predominantly affects young adults, but cases have been described in infants and children. Moss et al reported the case of a 6-month-old infant who was admitted to the emergency department with bilateral subdural fluid collections and skin ulcers that resembled cigarette burns. [4] Due to the infant's presentation, child abuse was suspected; however, during the child's admission, his neurologic condition continued to deteriorate, with progressive cerebral infarctions, and his skin ulcers revealed failure to heal. Histology confirmed the diagnosis of Degos disease. The child received palliative care and died 8 weeks after presentation. [4]

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Prognosis

Patients with multisystem involvement have a poor prognosis, with a mean survival of approximately 2 years. Patients with the benign, cutaneous-limited variant have a much better outcome with a prolonged survival.

Mortality/morbidity

The morbidity and mortality of malignant atrophic papulosis 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. [5, 6]  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.

Complications

Complications of malignant atrophic papulosis include the following:

  • Gastrointestinal bleeding
  • Intestinal perforation and peritonitis
  • Bowel ischemia
  • Cerebral infarcts
  • Spinal cord infarcts
  • Subdural/intracerebral hemorrhage
  • Neuropathy
  • Pericarditis
  • Pleuritis
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