<b><i>Background:</i></b> 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. <b><i>Methods:</i></b> 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. <b><i>Results:</i></b> 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. <b><i>Conclusion:</i></b> 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.
<b><i>Background:</i></b> Hidradenitis suppurativa (HS) is an inflammatory, potentially scarring disease of the hair follicle, affecting the apocrine gland-bearing skin areas. The major comorbid disorders associated with the occurrence or the aggravation of the disease are obesity and smoking. Numerous efforts to dissociate these factors led to controversial results. <b><i>Objectives:</i></b> To assess the importance of metabolic disorders/obesity, smoking/environmental toxins, and inflammation in HS by utilizing the differential expression of major relevant protein markers in lesional skin of obese/smoking versus non-obese/non-smoking HS patients. <b><i>Methods:</i></b> Lesional skin specimens deriving from two groups of HS patients (BMI >30 and smokers, <i>n</i> = 12 vs. BMI <30 and non-smokers, <i>n</i> = 10) were stained with antibodies raised against irisin, PPARγ, and IGF-1R, which correlate with metabolic disorders/obesity, EGFR and AhR, associated with smoking, and IL-17, IL-17R, and S100A8, as markers of inflammation. <b><i>Results:</i></b> Metabolic disorders/obesity-related markers exhibited marked differential expression between the two groups, while smoking-associated markers a limited one. IL-17R expression was stronger in obese/smokers, and S100A8 staining exhibited intense strong immunoreactivity in both groups without significant difference. <b><i>Conclusions:</i></b> The notion that obesity plays a role in HS development appears to be supported by the prominent regulation of the associated lesional biomarkers. Tobacco smoking might contribute less to HS than previously suspected.
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.
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