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European Journal of Dermatology

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Spectrum of comorbid autoimmune diseases in patients with chondrodermatitis nodularis helicis: a 17-year retrospective study of 215 patients in Asturias, northern Spain Volume 32, numéro 3, May-June 2022

Tableaux

Auteurs
1 Dermatology Department, Hospital Universitario Central de Asturias, Avenida de Roma, s/n. Oviedo, 33011, Asturias, Spain
2 Dermatology Department, Universidad de Oviedo, Asturias, Spain
3 GRIDER (Grupo de Investigación en Dermatología, Universidad de Oviedo)
4 Pathology Department, Hospital Universitario Central de Asturias, Avenida de Roma, s/n. Oviedo, 33011, Asturias, Spain
Reprints: Jimena Carrero Martín

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.