Malignant and benign forms of atrophic papulosis (Köhlmeier-Degos disease): systemic involvement determines the prognosis.

BACKGROUND: Atrophic papulosis (Köhlmeier-Degos disease) is a rare disease of unknown aetiology. The cutaneous signs--papular skin lesions with central porcelain-white atrophy and surrounding telangiectatic rim--are almost pathognomonic. Extracutaneous, systemic involvement includes multiple limited infarcts of the gastrointestinal system, central nervous system and other organs.
OBJECTIVES: To assess prospectively the demographics, epidemiological data and prognosis of patients with atrophic papulosis evaluated in a single centre.
METHODS: A prospective, single-centre, cohort study at diagnosis was performed on a series of 39 patients with atrophic papulosis, first seen between 2000 and 2007 and evaluated up to 2012.
RESULTS: The occurrence of cutaneous lesions defined the onset of disease in all cases. The mean age of onset was 35.4 ± 12.3 years and the male-to-female ratio was 1 : 1.4. In total, 9% of patients reported familial occurrence. Extracutaneous (systemic) signs were recorded in 29% of the patients, whereas the median time for development of systemic manifestations was 1 year (0.03-0.97 quantiles: 0-7 years) after the occurrence of cutaneous lesions. The prognosis was determined mainly by the presence of systemic involvement. 73% of the patients with systemic manifestations (73% developed intestinal perforation) died, while none of the patients with only cutaneous disease had a lethal outcome. The cumulative 5-year survival rate in patients with systemic disease was 54.5%.
CONCLUSIONS: Atrophic papulosis, previously called malignant atrophic papulosis, should be classified into a malignant, systemic form and a benign, cutaneous one, the latter being more common. The probability of having a benign form of the disease at onset is approximately 70%, increasing to 97% after 7 years of monosymptomatic cutaneous course.