S2k guideline for the treatment of hidradenitis suppurativa / acne inversa - Short version.

The S2k guideline on hidradenitis suppurativa/acne inversa (HS/AI) aims to provide an accepted decision aid for the selection/implementation of appropriate/sufficient therapy. HS/AI is a chronic recurrent, inflammatory, potentially mutilating skin disease of the terminal hair follicle-glandular apparatus, with painful, inflammatory lesions in the apocrine gland-rich regions of the body. Its point prevalence in Germany is 0.3%, it is diagnosed with a delay of 10.0 ± 9.6 years. Abnormal differentiation of the keratinocytes of the hair follicle-gland apparatus and accompanying inflammation form the central pathogenetic basis. Primary HS/AI lesions are inflammatory nodules, abscesses and draining tunnels. Recurrences in the last 6 months with at least 2 lesions at the predilection sites point to HS/AI with a 97% accuracy. HS/AI patients suffer from a significant reduction in quality of life. For correct treatment decisions, classification and activity assessment should be done with a validated tool, such as the International Hidradenitis Suppurativa Severity Scoring System (IHS4). HS/AI is classified into two forms according to the degree of detectable inflammation: active, inflammatory (mild, moderate, and severe according to IHS4) and predominantly inactive, non-inflammatory (Hurley grade I, II and III) HS/AI. Oral tetracyclines or 5-day intravenous therapy with clindamycin are equal to the effectiveness of clindamycin/rifampicin. Subcutaneously administered adalimumab, secukinumab and bimekizumab are approved for the therapy of HS/AI. Various surgical procedures are available for the predominantly non-inflammatory disease form. Drug/surgical combinations are considered a holistic therapy method.

Hidradenitis Suppurativa: Absence of Hyperhidrosis but Presence of a Proinflammatory Signature in Patients' Sweat.

The role of sweat glands in hidradenitis suppurativa has been largely neglected, despite the fact that its original designation, as "hidrosadénite phlegmoneuse", implied an inflammatory malfunction of the apocrine sweat glands as the underlying pathogenic driver. The aim of this study was to evaluate the role of apocrine sweat glands with respect to the proinflammatory environment of hidradenitis suppurativa. Therefore, gravimetric assessment and multiplex cytokine assays from sweat obtained from patients with hidradenitis suppurativa along with immunofluorescence cytokine/chemokine analysis of lesional apocrine glands- bearing hidradenitis suppurativa skin were performed. Gravimetric assessment of 17 patients with hidradenitis suppurativa revealed that the condition is not associated with hyperhidrosis. However, patients seem to be more affected by subjective sweating. The current data identified a complex proinflammatory signature in hidradenitis suppurativa sweat characterized by a significant upregulation of monocyte chemoattractant protein-1, interleukin-8 (CXCL8), and interferon-γ. In agreement with this, a strong in situ expression of these mediators could be observed in apocrine glands of lesional hidradenitis suppurativa skin. These data shed new light on the proinflammatory capacity of apocrine sweat glands in hidradenitis suppurativa, which may lead to reconsideration of the role of sweat glands in hidradenitis suppurativa pathology.

Switching from Adalimumab Originator to Biosimilar in Patients with Hidradenitis Suppurativa Results in Losses of Response-Data from the German HS Registry HSBest.

Since 2021, adalimumab biosimilar ABP 501 can be used alternatively to adalimumab originator (ADAO) in the treatment of hidradenitis suppurativa (HS). Effectiveness and safety data remain scarce. We investigated the impact of switching from ADAO to ABP 501 on disease severity and the occurrence of adverse events (AEs) in patients with HS. We analyzed clinical data on patients enrolled in the German HSBest registry. Evaluation outcomes were assessed at three time points (baseline of originator (t0), prior to switching to biosimilar (t1) and 12 to 14 weeks after switching (t2)) and included patient-reported AEs and disease severity using the International Hidradenitis Suppurativa Severity Score System (IHS4) score. In total, 94 patients were switched from ADAO to ABP 501. Overall, 33.3% (n = 31/94) of the patients developed AEs and/or loss of response (LoR) within 12 to 14 weeks after switching. Of these, 61.3% (n = 19/31) experienced LoR but no AEs, 22.6% (n = 7/31) LoR combined with AEs and 16.1% (n = 5/31) AEs only. Our study showed that switching HS patients from ADAO to ABP 501 does significantly affect treatment effectiveness. Switching patients who are on remission maintenance therapy should be viewed critically.

Economic analysis of the costs associated with Hidradenitis suppurativa at a German University Hospital.

BACKGROUND AND OBJECTIVES: Hidradenitis suppurativa (HS) significantly affects the patient`s quality of life and leads to multiple medical consultations. Aim of this study was to assess the utilization of medical care of HS patients. PATIENTS AND METHODS: All patients presenting in 2017 for an outpatient, day patient and / or inpatient treatment with leading claim type HS at the Department of Dermatology, University Hospital Würzburg, were included. Primary outcome was the economic burden of HS patients, measured by resource utilization in €. RESULTS: The largest share of the direct medical costs for HS were the inpatient costs with a leading surgical diagnosis-related group (DRG). Antiseptics were the predominant topical prescription. While doxycycline was the most frequently prescribed systemic therapy, adalimumab was the main cost driver. The difference between in-patient (€ 110.25) and outpatient (€ 26.34) direct non-medical costs was statistically significant (p < 0.001). With regards to indirect medical costs, a statistically significantly higher loss of gross value added (inpatient mean € 1,827.00; outpatient mean € 203.00) and loss of production (inpatient mean € 1,026.00; outpatient mean € 228.00) could be noted (p < 0.001), respectively. CONCLUSIONS: The present study on disease-specific costs of HS confirms that the hospital care of patients with this disease is cost-intensive. However, the primary goal of physicians is not and should not be to save costs regarding their patients`treatment, but rather the premise to utilize the existing resources as efficient as possible. Reducing the use of costly therapeutics and inpatient stays therefore requires more effective therapy options with an improved cost-benefit profile.

Correction: Economic analysis of the costs associated with Hidradenitis suppurativa at a German University Hospital.

[This corrects the article DOI: 10.1371/journal.pone.0255560.].

[Differences in anatomical area-specific wound healing in hidradenitis suppurativa/acne inversa after surgery].

BACKGROUND: Hidradenitis suppurativa/acne inversa is an inflammatory, debilitating disease for which wide local excision of the affected area with secondary wound healing is considered the treatment of first choice for the inactive scarring form or after adequate anti-inflammatory medical treatment. OBJECTIVES: In this study, we aimed to assess the duration of complete secondary wound healing after surgical intervention for hidradenitis suppurativa/acne inversa. MATERIALS & METHODS: Twenty-three surgical procedures in 17 consecutive patients (eight female, nine male) were evaluated for duration of secondary wound healing at axillary or anogenital/inguinal sites. To investigate the contribution of hair follicle bulge progenitor cells in wound re-epithelialization, tissue samples of lesional and perilesional skin were analysed for expression of the stem cell marker, cytokeratin 15 (CK15), and CD200, a marker for human follicular stem cells that resides in the bulge area. RESULTS: Initial wound size did not differ significantly between surgical wounds in the axillary (mean: 30.0 cm2 ± 5.4) and anogenital/inguinal (mean: 35.3 cm2 ± 5.7) region. However, healing time to complete wound closure was almost twice as fast in the anogenital/inguinal (mean: 132 days ± 10.4) than axilla area (mean: 254 days ± 39.1; p < 0.01). The accelerated wound healing in the anogenital/inguinal region was accompanied by significantly enhanced CK15 and CD200 expression, compared to axillary wounds (p < 0.05). CONCLUSION: The anogenital/inguinal region showed significantly faster secondary wound healing after surgical intervention for hidradenitis suppurativa/acne inversa compared to axillary wounds. We suspect differences in pilosebaceous unit density and thus hair follicle progenitor cells (as mirrored by CK15 and CD200 expression) to be the main driver behind this finding.

Scalp necrosis in giant cell arteritis: case report and review of the relevance of this cutaneous sign of large-vessel vasculitis.

Giant cell arteritis (GCA) is a systemic vasculitis associated with severe complications such as loss of vision and, rarely, scalp necrosis. We present a patient with GCA who had bilateral scalp necrosis and an erythrocyte sedimentation rate of only 21 mm after the first hour. Therapy with systemic steroids, which were slowly tapered over 1 year, led to secondary wound healing without recurrence. As there are no systematic reviews on the occurrence of scalp necrosis in patients with GCA, we performed a literature research and meta-analysis and discovered 78 cases published between 1946 and 2007. Analysis of the data revealed that GCA with scalp necrosis is associated with a higher incidence of vision loss (32%) and other visual defects (37.3%) than GCA without scalp necrosis (visual disturbances in up to 20%). GCA with scalp necrosis is also associated with an increased mortality (standard mortality ratio [SMR], 4.2) in contrast to GCA without scalp necrosis, which has no significantly higher mortality than age-matched controls (SMR 0.8-1.034). In patients with scalp necrosis, the diagnosis of GCA was made about 1 month later than in patients without scalp necrosis, and scalp necrosis was never reported to occur after onset of therapy with glucocorticoids. Thus, for reasons beyond potential loss of vision, physicians should be alert for symptoms of GCA as only timely diagnosis and immediate therapy may prevent serious complications and increased mortality.

Assessing the psychological burden of patients with hidradenitis suppurativa.

Hidradenitis suppurativa (HS) causes a significantly compromised quality of life. Assessment of the psychological burden of HS is crucial for evidence-based allocation of resources. We examined methods to easily assess the psychological burden in HS patients. A total of 110 HS patients were assessed with respect to sociodemographic data, Hurley scale, and modified Sartorius score. Patients were asked to provide information on time of first diagnosis, previous therapies, surgical intervention, and pre-existing conditions. Distributed questionnaires were the Dermatology Life Quality Index (DLQI) and the Skindex-29, the Visual Analogue Scale (VAS) for pain, and the German version of the Hospital Anxiety and Depression Scale (HADS-D) for evaluation of anxiety (HADS-D/A) and depression (HADS-D/D). Of the 110 patients with HS (mean age: 38 ± 12 years; range: 18 to 75), 61 were female and 49 were male. We found a statistically significant correlation between HADS-D/A and VAS (p = 0.009), between Skindex-29 and Sartorius score (symptoms: p = 0.024; emotions: p = 0.019; functional status: p = 0.002), as well as between Skindex-29 and VAS (symptoms: p = 0.000; emotions: p = 0.001; functional status: p = 0.000). Additionally, VAS correlated significantly with DLQI (p = 0.000) and body mass index with Sartorius score (p = 0.038). Our study shows that HS patients experience a high level of psychological distress. Interestingly, a clear correlation between psychological burden and HS patients was inferred by the VAS for pain. HS patients require active management for their physical as well as psychological health.

Surgical treatment of persistent macrocheilia in patients with Melkersson-Rosenthal syndrome and cheilitis granulomatosa.

BACKGROUND: Various conservative methods for treatment of labial swelling in patients with cheilitis granulomatosa have been attempted, often with only moderate success and sometimes with persistent disfiguring lip swelling. Severe macrocheilia can produce an unaesthetic facial deformity associated with functional disturbances. In patients with persistent macrocheilia, reduction cheiloplasty with excision of excess tissue may be indicated when conservative treatment has proven ineffective in reducing swelling but may have been successful in stabilizing disease. OBJECTIVE: To evaluate long-term results after reduction cheiloplasty in patients with macrocheilia caused by Melkersson-Rosenthal syndrome or cheilitis granulomatosa. DESIGN: Follow-up study in 7 patients with severe persisting macrocheilia, including 3 patients with Melkersson-Rosenthal syndrome and 4 patients with cheilitis granulomatosa in a stable state of disease, treated by reduction cheiloplasty at our hospital between January 1, 1987, and December 31, 2002. Preoperative and postoperative medical histories were obtained, and criteria for the success of surgical treatment were evaluated by clinical examination. Different techniques of reduction cheiloplasty are described and demonstrated in representative cases of severe macrocheilia. RESULTS: Surgical treatment in all 7 patients showed satisfying aesthetic and functional outcomes that persisted throughout follow-up (median follow-up, 6.5 years). CONCLUSIONS: Reduction cheiloplasty is an effective method to correct persistent macrocheilia and improve lip aesthetics in patients with Melkersson-Rosenthal syndrome or granulomatous cheilitis in the persistent state of disease. With careful planning, proper sequencing of treatment, and proficiency in the various surgical techniques, optimal results can be achieved.