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

Background Atrophic papulosis (Kohlmeier-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+/-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.