Atrophic papulosis (Köhlmeier-Degos disease) revisited: a cross-sectional study on 105 patients.

BACKGROUND: Atrophic papulosis is a very rare vascular disease of unknown pathogenesis, mostly described by case reports. OBJECTIVE: To assess demographic data and prognosis in patients with atrophic papulosis. METHODS: A single-centre study was performed on a series of 105 patients with atrophic papulosis, diagnosed 2000-2021. Patients were referred and diagnosed at the evaluation centre and patients' clinical data were provided by the Degos Support Network and evaluated by the authors for confirming the diagnosis of skin lesions and fulfilling the diagnostic criteria for a malignant subset. A unique set of variables were collected from all patients. RESULTS: The mean age of disease onset was 33.3 ± 18.3 years and the male-to-female ratio was 1:1.6. The family history rate was 8.1%. The classification into a benign, merely cutaneous disease (benign atrophic papulosis), and malignant atrophic papulosis, associating cutaneous and visceral lesions was confirmed due to their striking prognostic difference. Benign atrophic papulosis was detected in 41% of the patients with no deaths occurring throughout the follow-up period (median 3.00 years; range 0.13-23). Malignant atrophic papulosis was reported in 59% of patients with 47.5% multisystemic involvement and a median skin lesion onset to systemic symptoms duration of 0.54 years (-6 to 20). The gastrointestinal tract and central nervous system were equally involved; however, the neurological involvement-caused death rate was slightly higher. The disease-specific mortality rate of malignant atrophic papulosis was 22.6%. CONCLUSIONS: Atrophic papulosis presents with a striking prognostic difference of benign - merely cutaneous - involvement or quickly developing - into less than 1 year - malignant subset, associating cutaneous and visceral lesions and multiorgan involvement in 1/2 of the patients, which leads to premature, disease-specific mortality in 1/4 of the cases. Central nervous system and gastrointestinal tract complications are the major reasons for disease-specific death. Over the years, the diagnosis of severe nervous system involvement has become more common.

[Pathogenesis of hidradenitis suppurativa/acne inversa]. / Pathogenese der Hidradenitis suppurativa/Acne inversa.

Hidradenitis suppurativa/acne inversa (HS) has a multifactorial pathogenesis. In addition to a sporadic form, a familial form is reported in around 40% of patients, for whom an autosomal dominant (AD) inheritance with reduced gene penetrance is assumed. The phenotype of the disease with inflammatory nodules, abscesses and secreting sinus tracts suggests an infectious origin, but the exact role of the bacteria detected in HS pathogenesis remains unclear. Smoking and metabolic syndrome are regarded as important trigger factors in HS, with obesity and hormonal changes playing a pathogenic role in the latter. Ultimately, Toll-like receptors, antimicrobial peptides, immune cells and key cytokines are involved in the excessive inflammatory reaction of HS and are also the targets of future therapies.