Spectrum of comorbid autoimmune diseases in patients with chondrodermatitis nodularis helicis: a 17-year retrospective study of 215 patients in Asturias, northern Spain.

Background: The cause of chondrodermatitis nodularis helicis (CN) (Winkler’s disease) is unknown, but potential associations with autoimmune diseases have been suggested in case reports, however, studies with large case series are lacking.

Objectives: To clarify the frequency of chronic inflammatory and autoimmune diseases (CADs), and associated gender and age distribution, in a large cohort of patients with CN.

Materials & Methods: The frequency of CADs (systemic and cutaneous) was assessed in 215 patients (65.1% males and 34.9% females; median age: 69.6 years) with a histopathological diagnosis of CN (2000-2017). Endocrine diseases were not included. Statistical analysis included Fisher’s exact test and multivariate logistic regression analysis.

Results: Twenty different CADs were diagnosed in 15.34% patients with CN. The most frequent were polymyalgia rheumatica (six patients), psoriasis (four patients, one with psoriatic arthritis), rheumatoid arthritis (three patients), CREST syndrome (two patients), vitiligo (two patients), and chronic dermatitis (two patients). Several CADs were strongly associated with tobacco smoking. Systemic CADs were more frequent in females (OR: 3.814; CI 1.513-9.613; p = 0.005; multivariate logistic regression analysis). Differences according to age at onset were not significant.

Conclusion: We characterize, for the first time, the spectrum of CADs as well as age and gender distribution in patients with CN based on the largest cohort of patients to date. The possible accumulation of different disorders that are strongly associated with tobacco smoking (Buerger’s disease, pulmonary Langerhans cell histiocytosis, rheumatoid arthritis, Lupus erythematosus, and others) merits further investigation, but the rarity of some of them makes this challenging.