Self-management-competency as a new target in Hidradenitis suppurativa care.

Background: Hidradenitis suppurativa affects approximately 1% of the population.Objective: Highlighting the relevance of self-management-competency as a new therapeutic target.Method: 258 patients from the 'Epidemiology and Care in Acne inversa (EpiCAi)' project were included in the study. Disease burden was measured by patient-rated questionnaires in terms of disease activity, pain, quality of life, depression and insomnia and correlated with the domains of the health education impact questionnaire (heiQ) measuring self-management-competency.Results: 66 male (25.6%) and 192 female (74.4%) patients, with a mean age of 40.3 ± 10.24 years were included. Mean scores of pain on the numeric rating scale (NRS), Dermatology Life Quality Index (DLQI) and Hospital Anxiety and Depression Scale (HADS) were 5.11 ± 2.68, 11.35 ± 7.79 and 13.71 ± 7.57, respectively. The Insomnia severity index (ISI) showed a mean of 9.58 ± 5.76. The HADS has the highest increased total risk across all heiQ domains. With respect to the heiQ domains, the highest exposure can be attributed to improving constructive attitudes and approaches as well as decreasing emotional distress.Conclusion: There is a clear association of self-management-competency with overall disease burden, which underlines the need for psychoeducational support. This study provides ideas to develop new possible strategies of care.

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.

[Topical and novel device-based therapies for mild hidradenitis suppurativa]. / Lokale und neue apparative Therapien der milden Hidradenitis suppurativa.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic inflammatory disease of the skin folds, which requires more outpatient treatment alternatives. Although the disease was previously treated using surgery, new treatment modalities now allow the effective treatment of mild and moderate cases in an ambulatory care setting. AIM OF STUDY: Local and instrument-based therapies are presented and their efficacy and safety profiles are highlighted. MATERIALS AND METHODS: Clinical evidence for each therapeutic modality are presented and current treatment developments are analyzed based on the future treatment of HS patients in Germany. RESULTS: Effective treatments for outpatient care of HS patients include topical clindamycin, resorcinol, and intralesional corticosteroids. New devices such as LAight therapy (combining intense pulsed light [IPL] with radiofrequency) are available, which can be used as monotherapy or adjunct therapy in combination with systemic treatment and/or surgery for the management of HS. CONCLUSION: Evidence-based use of local treatments can provide more efficient outpatient and self-administered strategies, which improves the quality of life of HS patients, especially for patients with recurrent mild and moderate disease.

[Epidemiology, patient quality of life, and treatment costs of hidradenitis suppurativa/acne inversa]. / Epidemiologie, Patientenlebensqualität und Behandlungskosten der Hidradenitis suppurativa/Acne inversa.

Hidradenitis suppurativa/acne inversa (HS) is associated with numerous and relevant restrictions on the quality of life for those affected and their relatives. The exact prevalence of HS varies significantly across studies, but it is likely to be higher than suggested in previous publications. HS care is associated with high costs for the healthcare system and for those affected. The introduction of biologic therapy has led to additional costs, but also to considerable additional benefits in terms of care. In view of the complexity of diagnostics and therapy, there is a particular need for optimized care concepts in order to reduce the burden on those affected, their relatives and the healthcare system.

[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.

[Endocrinological mechanisms in the pathophysiology of hidradenitis suppurativa]. / Endokrine Mechanismen bei der Pathophysiologie der Hidradenitis suppurativa.

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.

Hidradenitis suppurativa in paediatric patients: a retrospective monocentric study in Germany and review of the literature.

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.

Alterations in innate immunity and epithelial cell differentiation are the molecular pillars of hidradenitis suppurativa.

BACKGROUND: The large unmet need of hidradenitis suppurativa/acne inversa (HS) therapy requires the elucidation of disease-driving mechanisms and tissue targeting. OBJECTIVE: Robust characterization of the underlying HS mechanisms and detection of the involved skin compartments. METHODS: Hidradenitis suppurativa/acne inversa molecular taxonomy and key signalling pathways were studied by whole transcriptome profiling. Dysregulated genes were detected by comparing lesional and non-lesional skin obtained from female HS patients and matched healthy controls using the Agilent array platform. The differential gene expression was confirmed by quantitative real-time PCR and targeted protein characterization via immunohistochemistry in another set of female patients. HS-involved skin compartments were also recognized by immunohistochemistry. RESULTS: Alterations to key regulatory pathways involving glucocorticoid receptor, atherosclerosis, HIF1α and IL17A signalling as well as inhibition of matrix metalloproteases were detected. From a functional standpoint, cellular assembly, maintenance and movement, haematological system development and function, immune cell trafficking and antimicrobial response were key processes probably being affected in HS. Sixteen genes were found to characterize HS from a molecular standpoint (DEFB4, MMP1, GJB2, PI3, KRT16, MMP9, SERPINB4, SERPINB3, SPRR3, S100A8, S100A9, S100A12, S100A7A (15), KRT6A, TCN1, TMPRSS11D). Among the proteins strongly expressed in HS, calgranulin-A, calgranulin-B and serpin-B4 were detected in the hair root sheath, koebnerisin and connexin-32 in stratum granulosum, transcobalamin-1 in stratum spinosum/hair root sheath, small prolin-rich protein-3 in apocrine sweat gland ducts/sebaceous glands-ducts and matrix metallopeptidase-9 in resident monocytes. CONCLUSION: Our findings highlight a panel of immune-related drivers in HS, which influence innate immunity and cell differentiation in follicular and epidermal keratinocytes as well as skin glands.

Pilonidal sinus disease: an intergluteal localization of hidradenitis suppurativa/acne inversa: a cross-sectional study among 2465 patients.

BACKGROUND: Hidradenitis suppurativa (HS), also referred to as acne inversa, is a debilitating skin disease characterized by inflammatory nodules, chronic abscesses and tunnels (fistulae and sinuses). The association with pilonidal sinus disease (PSD) is frequently reported but not well documented. OBJECTIVES: To determine the prevalence and characteristics of inflammatory skin lesions located in the intergluteal fold (IGF) of patients with HS. METHODS: This was an international multicentre retrospective cross-sectional study based on data collection from a large cohort of patients with HS with and without histopathology. Results From a total of 2465 patients with HS included in the study, 661 (27%) reported lesions in the IGF. These patients were significantly more often smokers and had more severe HS. Of the 238 patients with an available clinical diagnosis, intergluteal-HS (IG-HS) was diagnosed in 52 patients (22%) and PSD was diagnosed in 186 patients (78%). IG-HS was associated with the localization of HS in the proximity of the IGF, including the buttocks, genitals and the anus. There was a possibility of misclassification bias in this study as a clinical/image-based diagnosis or histopathology of the IGF lesions was not always available. CONCLUSIONS: The high prevalence of PSD suggests a strong link between both entities. Therefore, it may be useful to identify common pathophysiological mechanisms and develop common therapeutic strategies. What's already known about this topic? The occurrence of pilonidal sinus disease has not been clearly reported among patients with hidradenitis suppurativa/acne inversa. What does this study add? This is the first study that investigated the prevalence of pilonidal sinus disease among a large cohort of patients and identified the patient characteristics. Risk factors that might help to improve the management of patients were identified.

[Two annular, atrophic plaques on the abdomen of a 35-year-old man]. / Zwei anuläre, atrophische Plaques am Abdomen bei einem 35-jährigen Patienten.

A 35-year-old man presented with two annular, reddish-brown, atrophic skin lesions in the navel and on the right lower abdomen. The lesions had persisted for more than 4 years and had remained unchanged and asymptomatic. Histology revealed annular atrophic lichen planus with a lichenoid lymphocytic infiltration and cystoid bodies. The patient was treated with local corticosteroids without improvement.

Diabetes mellitus and the skin.

Diabetes is a debilitating, life-threatening disease accounting in 2015 for the death of 5 million people worldwide. According to new estimations, 415 million adults currently suffer from the disease, and this number is expected to rise to 642 million by 2040. High glucose blood levels also affect the skin among systemic organs, and skin disorders can often predict the onset of this metabolic disorder. In this review, we address the pathomechanistic effects of diabetes on the skin and give an overview on the most common skin diseases associated with diabetes.

CD40/CD40 ligand interactions and TNFα treatment reduce activity of P105 promoter of the human papilloma virus-18 in vitro.

BACKGROUND: Cervical carcinoma cells including those infected with the oncogenic human papilloma virus (HPV) and several cervical carcinoma cell lines show a strong expression of the CD40 receptor, unlike benign cervical epithelial cells infected with HPV. The functional relevance of this up-regulated expression in the tumor is not fully understood. Nevertheless, it might offer a unique possibility to target those malignant cells due to the antiviral and antitumoral effects of the CD40/CD40 ligand (CD40L) interactions. AIM: In vitro assessment of the effect of CD40L on HPV 18-P105 promoter activity and the subsequent release of IL-6 by the promoter transfected HeLa(CD40) cells, which express CD40 constitutively. MATERIAL AND METHODS: Transfection of HeLa(CD40) cells was achieved by electroporation after optimizing the parameters by the pCMV-ß-Gal vector and ß-Gal stain. Transfected HeLa(CD40) cells were challenged with BHK(CD40L) and TNFα, in addition to BHK(wt) and medium alone as controls. HPV18-P105 promoter activity was demonstrated by luciferase reporter gene assay while IL-6 was assessed by ELISA. RESULTS: CD40/CD40L interactions and TNFα treatment significantly reduced HPV18-P105 promoter activity (56.0 ± 10.2% and 64.1 ± 9.1% vs. control, respectively; p < 0.001). Likewise, IL-6, which is a sensitive cytokine of CD40 activation, was significantly increased in HeLa(CD40) cells in the same experiments (2.7 fold after stimulation with BHK(CD40L) and 5.2 fold after stimulation with TNFα vs. control; p < 0.01 and p < 0.001, respectively). CONCLUSION: It is likely that the CD40/CD40L interactions and TNFα are effective against cervical carcinomas by repressing transcriptional activity of HPV promoter. This can result in new adjuvant treatments.

[Experimental models of human skin aging]. / Experimentelle Modelle humaner Hautalterung.

The skin is a representative model for the study of human aging. Despite the high regenerative capacity of the skin, skin physiology changes over the course of life. Medical and cosmetic research is trying to prevent aging, to slow, to stop, or to reverse it. Effects of age-related DNA damage and of changing skin structure on pharmacological parameters are largely unknown. This review article summarizes the state of scientific knowledge in the field of experimental models of human skin aging and shows approaches to improve organotypic skin models, to develop predictive models of aging, and improve aging research.