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.

Proceeding report of the Symposium on Hidradenitis Suppurativa Advances (SHSA).

Hidradenitis Suppurativa (HS) is a chronic debilitating skin condition that impairs the productivity and the quality of patients` lives. HS has recently drawn lots of attention among scholars to further expand their knowledge but it still loads with uncertainties and gaps to be explored. This publication addresses these uncertainties, and provides a road-map for researchers, scholars and clinicians from different disciplines for their future studies about HS. This is a proceeding report of the first Symposium on Hidradenitis Suppurativa Advances (SHSA), and it reviews the scientific sessions about the epidemiology, pathophysiology, presentations, and management of HS. This symposium was a great opportunity for experts in the HS field to exchange their knowledge, and improve their mutual understanding of this disease.

Becker Naevus Syndrome of the Lower Body: One Case and Review of the Literature.

Becker naevus syndrome is a rare epidermal naevus syndrome defined by the co-occurrence of a Becker naevus with various cutaneous, muscular and skeletal anomalies. In the majority of cases, abnormalities exclusively consist of ipsilateral hypoplasia of the breast, areola and/or nipple in addition to the naevus. Here, we report on a 42-year-old woman with an extensive Becker naevus reaching from the left buttock to the left calf verified on histological examination. In addition, there was marked hypoplasia of the fatty tissue of the left thigh confirmed by magnetic resonance imaging in contrast to hyperplasia of the fatty tissue of the left gluteal area. Underlying muscles and bones were not affected. There was no difference in leg lengths. In addition, we review and discuss the features of Becker naevus syndrome with emphasis on 10 reported cases with involvement of the lower body.

Kerinokeratosis Papulosa of Childhood.

BACKGROUND: Kerinokeratosis papulosa (KP) is considered an extremely rare genodermatosis presenting usually as waxy papules on the trunk in childhood. OBJECTIVE: To describe and analyze the clinical, histological and potential etiopathological aspects of KP. METHODS: The dermatoscopic features of a new case of KP of childhood are investigated. The presence of human papillomavirus (HPV) DNA in lesional skin was studied by polymerase chain reaction. Furthermore, all cases of KP of childhood reported so far were reviewed. RESULTS: As a diagnostic tool, we describe for the first time a dermatoscopic feature, namely a cribriform pattern of KP, in an 11-year-old boy. In addition, we detected HPV (type 57) in his KP lesions. CONCLUSIONS: Dermatoscopic examination might be a useful tool to distinguish KP from other skin lesions, e.g. common warts. The detection of HPV type 57 might hint to an etiological role of HPV for KP.

[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.

Smart Scar Care-Industry 4.0 in Individualized Compression Garments: A Randomized Controlled Crossover Feasibility Study.

BACKGROUND: We tested the workflow and comparability of compression garments (CG) automatically knitted from 3D-body-scan data (3DBSD) versus manually measured data for scar treatment. Industry 4.0 has found its way into surgery, enhancing the trend toward personalized medicine, which plays an increasingly important role in CG scar therapy. Therefore, we conducted a study to evaluate the workflow from 3DBSD to fast and precisely knitted CG and compared it with standard of care. METHODS: A randomized controlled crossover feasibility study was conducted as part of the individual medical technology research project "Smart Scar Care." Objective and patient-reported outcome measures were documented for 10 patients with hypertrophic burn scars at baseline and after wearing CG automatically knitted from 3DBSD versus CG from manually measured data for one month. RESULTS: The "scan-to-knit" workflow and the study design were feasible in 10 of 10 patients. No adverse effects were found. 3DBSD showed a bias of half a centimeter compared with manually measured data and wider limits of agreement. With respect to fit, comfort, suitability, Vancouver Scar Scale, Patient and Observer Scar Assessment Scale, stiffness and microcirculation, this was a promising pilot study. Stiffness and blood flow were increased in scars compared with normal skin. The highest rank correlations were found between pain and itch, stiffness and Patient and Observer Scar Assessment Scale, Vancouver Scar Scale, and pain. CONCLUSIONS: These results indicate that automatically knitted CG using 3DBSD could become an alternative to the standard of care, especially with regard to economical and faster patient care. The produced scan data opens the door for objective scar science.

Severe COVID-19 infection in a patient with multiple sclerosis treated with fingolimod.

BACKGROUND: Fingolimod is used for immune therapy in patients with multiple sclerosis. Long-term treatment is associated with a small increase in the risk of herpes virus reactivation and respiratory tract infections. Patients with coronavirus disease 2019 (COVID-19) under Fingolimod treatment have not been described. METHODS AND RESULTS: We report a 57-year old female patient with a relapsing remitting multiple sclerosis under fingolimod treatment who experienced a severe COVID-19 infection in March 2020 (Extended Disability Status Scale: 2.0). Having peripheral lymphopenia typical for fingolimod treatment (total lymphocytes 0.39/nL [reference range 1.22-3.56]), the patient developed bilateral interstitial pneumonia with multiple ground-glass opacities on chest CT. Fingolimod medication was stopped. On the intensive care unit, non-invasive ventilation was used to provide oxygen and ventilation support regularly. Over the following two days, oxygenation improved, and the patient was transferred to a normal ward five days after admission. CONCLUSION: The implications fingolimod has on COVID-19 are complex. As an S1P analogue, fingolimod might enhance lung endothelial cell integrity. In addition, in case of a so-called cytokine storm, immunomodulation might be beneficial to reduce mortality. Future studies are needed to explore the risks and therapeutic effects of fingolimod in COVID-19 patients.

Split-skin grafting from the scalp: the hidden advantage.

BACKGROUND: Split-skin grafting is a routine reconstructive technique associated with large variation in practice. Grafts from the thigh, buttock, or abdomen take a long time to heal and may leave unpleasant, hypopigmented scars. Retrospective reports favor the scalp as a donor site in burn patients. OBJECTIVE: Evaluation of duration of healing, cosmetic outcome, and safety of split-skin grafting from the scalp in patients receiving dermatologic surgery. MATERIAL AND METHODS: One hundred sixty-six consecutive patients (85 men, 81 women) were treated for coverage of chronic leg ulcers or other large skin defects with a split-skin graft taken from the posterior scalp. Area and thickness of the graft, healing time, and adverse events were documented. RESULTS: Mean healing time until complete reepithelization was 5.4+/-1.0 days for a single harvest (median 5 days). No major complications occurred. Spotted alopecia was a rare event. Almost all (96.5%) of the patients would undergo split-skin harvesting from the occipital scalp again if needed. CONCLUSIONS: Advantages of the scalp as a donor site include rapidity of wound healing, low risk of complications, and excellent cosmetic results. The large number of hair follicles containing the epidermal stem cell pool can explain these advantages.

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.

Improvement of impaired diastolic left ventricular function after diet-induced weight reduction in severe obesity.

BACKGROUND/OBJECTIVES: Obesity is independently associated with left ventricular (LV) diastolic dysfunction and altered cardiac morphology. Morbidity and mortality in patients with diastolic dysfunction are similar to values observed in patients with systolic heart failure. We hypothesized that dysfunctional cardiac responses in people with obesity are reversible after weight loss. Thus, we studied the effect of dietary weight reduction on LV diastolic function as well as on cardiac structure using transthoracic echocardiography and tissue Doppler imaging (TDI). SUBJECTS/METHODS: Thirty-two subjects with obesity underwent a 12-week low-calorie fasting phase of a formula diet. Echocardiographic tissue Doppler indices of diastolic function and measurements of cardiac size were obtained prior to and after the fasting phase. RESULTS: A 12-week diet significantly reduced body mass index from 40.3 ± 6.6 kg/m2 to 33.2 ± 6.1 kg/m2 (p < 0.01). Weight loss was associated with a significant reduction in blood pressure and heart rate. Echocardiography revealed diastolic dysfunction in subjects with obesity, which was improved by dieting. After weight loss, trans-mitral Doppler echocardiography showed a significant reduction in A-wave velocity, from 65.8 ± 19.2 cm/s to 57.0 ± 16.8 cm/s, and an increase in E/A ratio from 1.2 ± 0.4 to 1.4 ± 0.5 (p < 0.01). TDI displayed a significantly lower a'-wave velocity (10.3 ± 2.3 cm/s and 8.9 ± 1.7 cm/s; p < 0.01). Left atrial and LV dimensions were normal and remained unchanged after weight loss. CONCLUSION: Obesity is associated with diastolic dysfunction. A 12-week low-calorie diet with successful weight loss can reduce blood pressure and heart rate and partially normalize diastolic dysfunction.

Impact of Severe Obesity and Weight Loss on Systolic Left Ventricular Function and Morphology: Assessment by 2-Dimensional Speckle-Tracking Echocardiography.

Obesity is associated with an increased risk of heart failure. Little is known about the impact of dietary changes on the cardiac sequelae in obese patients. Twenty-one obese subjects underwent a 12-week low calorie fasting phase of a formula diet. Transthoracic two-dimensional speckle-tracking echocardiography was performed to obtain systolic left ventricular strain before and after weight loss. Body mass index decreased significantly from 38.6 ± 6.2 to 31.5 ± 5.3 kg/m(2), and the total percentage fat loss was 19%. Weight reduction was associated with a reduction in blood pressure and heart rate. Left ventricular longitudinal global peak systolic strain was in the lower normal range (-18.7 ± 3.2%) before weight loss and was unchanged (-18.8 ± 2.4%) after 12 weeks on diet with substantial weight loss. Also, no significant change in global radial strain after weight loss was noted (41.1 ± 22.0 versus 43.9 ± 23.3, p = 0.09). Left atrial and ventricular dimensions were in normal range before fasting and remained unchanged after weight loss. In our study obesity was associated with normal systolic left ventricular function. A 12-week low calorie diet with successful weight loss can reduce blood pressure and heart rate. Systolic left ventricular function and morphology were not affected by rapid weight reduction.

Impact of fumaric acid esters on cardiovascular risk factors and depression in psoriasis: a prospective pilot study.

Patients with psoriasis have an increased risk of cardiovascular disease that is partly attributable to chronic systemic inflammation. The aim of our prospective pilot study was to investigate the impact of fumaric acid esters (FAE), a first-line systemic antipsoriatic treatment in Germany, on cardiovascular risk parameters. Participants with moderate-to-severe psoriasis from the University Medical Center Mannheim and the University Hospital Würzburg were treated with FAE for 16 weeks according to standard dosage recommendations. Disease severity, life quality and depression scores as well as biomarkers of inflammation, lipid and glucose metabolism were assessed prior to initiation of FAE and after 16 weeks. Out of 39 participants recruited, 27 completed the study. 44% of all participants and 63% of those completing the 16-week treatment achieved PASI 50 response and 27 or 37% PASI 75 response. Clinical improvement was paralleled by significant improvement in quality of life, high treatment satisfaction and significant reduction of depressive symptoms. Adverse events, most frequently mild gastrointestinal complaints, flush and lymphocytopenia occurred in 89%. FAE did not modify glucose metabolism or inflammatory parameters substantially. However, a highly significant increase in serum levels of the atheroprotective cytokine adiponectin was noted after 16 weeks (median 4.7 vs. 8.9 µg/ml; p = 0.0002). Our study demonstrates a significant beneficial impact of FAE on adiponectin, indicating a potential cardioprotective effect. It will be interesting to verify this finding in larger cohorts and to assess the long-term influence of FAE on cardiovascular risk and disease.

Brooke-Spiegler syndrome complicated by unilateral hearing loss.

Brooke-Spiegler syndrome is an autosomal dominant tumor predisposition disorder. The disease is characterized by the occurrence of multiple skin appendage tumors, including cylindroma, trichoepithelioma, and spiradenoma. In some patients, tumors cover the entire head circumference, thereby causing disfigurement and other complications. Here, we report on a man with multiple cylindroma that were distributed in a turban tumor-like fashion. One of these neoplasms arose in the meatus externus of the right ear leading to unilateral hearing loss, a complication that has been documented only on few occasions in this disease.

Albumin induces NF-kappaB expression in human proximal tubule-derived cells (IHKE-1).

BACKGROUND: Chronic renal diseases with enhanced glomerular protein filtration are accompanied by tubulointerstitial inflammation and progression to renal function deterioration. Filtered proteins, like albumin, seem to be a pathogenic factor per se in the progression of renal diseases. There is evidence that the nuclear factor kappaB (NF-kappaB) is involved in protein-overload stimulated renal inflammatory pathomechanisms. The aim of this study was to investigate albumin-induced NF-kappaB expression as well as NF-kappaB activity upon long long term exposure to albumin in human proximal tubular cells as only acute albumin-induced NF-kappaB activity has been reported so far. METHODS: To investigate the hypothesis, that NF-kappaB may be involved in protein-induced renal inflammatory pathomechanisms, we exposed human renal proximal tubule-derived cells (IHKE-1) to bovine serum albumin (BSA: 50 and 500 microg/ml). The NF-kappaB and TNF-alpha specific mRNA expression was detected by RT-PCR. NF-kappaB specific protein expression was analysed by Western blot. Reporter gene assays were performed to determine the NF-kappaB specific activity. RESULTS: Albumin-exposure induced an increase in NF-kappaB specific mRNA expression, NF-kappaB protein expression and activity. These effects are decreased by the protein kinase C (PKC) inhibitor bisindolylmaleimide I (BIM) and the tyrosine kinase inhibitor herbimycin A. An albumin-induced increase in TNF-alpha specific mRNA expression as biological, inflammatory parameter associated with the albumin-induced NF-kappaB activity was detectable. CONCLUSION: We suggest, that albumin-exposure induces an increase in NF-kappaB and TNF-alpha specific mRNA expression, NF-kappaB specific protein expression and protein activity in renal proximal tubule cells in culture, which is at least in part PKC and tyrosine kinase dependent.