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Background: There is growing evidence that (certain) hidradenitis suppurativa (HS) comorbidities comprise syndromes including HS as a key cutaneous manifestation. These apparently autoinflammatory syndromes and their diagnostic delay might have detrimental effects on affected patients., Methods: A systematic review was performed on the databases MEDLINE, EMBASE, and CENTRAL utilizing a standardized extraction form according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines., Results: Sixty-four eligible articles on syndromic HS were retrieved. The identified syndromes included already described ones (pyoderma gangrenosum-acne-suppurative hidradenitis, pyogenic arthritis-pyoderma gangrenosum-acne-suppurative hidradenitis, psoriatic arthritis-pyoderma gangrenosum-acne-suppurative hidradenitis, pyoderma gangrenosum-acne vulgaris-hidradenitis suppurativa-ankylosing spondylitis, synovitis-acne-pustulosis-hyperostosis-osteitis) and further novel symptom constellations. Cutaneous signs, including HS lesions, usually precede signs from other organs. The cutaneous signs of a considerable proportion of patients appear refractory to conventional treatment, and monotherapy with biologics does not suffice to sustain remission., Conclusion: The results are subsequently discussed with focus on the pathophysiology and treatment of the detected syndromes. The dermatologist's role in the precise diagnosis and early treatment administration of HS is pivotal. The purpose of the treatment should be the effective prevention or delay of the autoinflammatory march and its irreversible consequences., (© 2020 S. Karger AG, Basel.)

Hidradenitis suppurativa (HS) is a chronic, recurrent cutaneous disease of the terminal hair follicle which manifests with deep-seated, painful nodules, abscesses, and sinus tract formation. The pathophysiology of the disease includes among various factors also dermatoendocrinologic variables: Correlations with metabolic syndrome, obesity, sex steroid hormones, and the improvement after antiandrogen therapy are some of the key points presented in this review. Hormonal treatment of HS can be an effective and inexpensive alternative or add-on therapy to classic HS treatments, especially in cases where antibiotics and/or biologics are ineffective or contraindicated.

Background: Hidradenitis suppurativa (HS) is a chronic, inflammatory, recurrent, debilitating skin disease of the hair follicle affecting apocrine glands-rich areas of the body. The disease usually occurs after puberty leading to painful nodules, abscesses, tunnels and scarring. Although uncommonly, HS can also occur in children and adolescents., Objective: Our objective was to describe the epidemiology, characteristics, predisposing factors and clinical course of HS in paediatric patients., Methods: The retrospective cohort study included patients with HS, who have been diagnosed in Dessau Medical Center and reported development of HS during childhood or adolescence, fulfilled the diagnostic criteria for HS and had a follow-up period of at least one year. A systematic review was conducted on MEDLINE, EMBASE and CENTRAL on 19 March 2020 using the terms 'hidradenitis' or 'acne inversa' together with the terms 'children', 'paediatric' and 'adolescence'., Results: Twenty paediatric patients [1 child (0.22%), 19 adolescents (4.25%)] were detected out of 447 patients evaluated (4.5%) with a male to female ratio of 1.86 : 1. The median age at diagnosis was 17 years [interquartile range (IQR) 16-18] and the median age at onset 15 years (IQR: 14-16.5). The majority of the patients suffered from moderate disease were overweight or obese and non-smokers. The most common comorbid disorder was acne vulgaris. The systematic review revealed reports with data heterogeneity and lack of systematic documentation of specific demographic characteristics. Most paediatric patients were female, obese and non-smokers, with considerable comorbid disorders., Conclusions: Hidradenitis suppurativa in Germany may affect children and adolescents exhibiting a particular phenotype of mainly male non-smokers., (© 2020 The Authors. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology.)