Management of Pilonidal Disease and Hidradenitis Suppurativa.

Pilonidal disease and hidradenitis suppurativa affect healthy young adults, causing discomfort and pain that leads to loss of work productivity and should be approached in a personalized manner. Patients with pilonidal disease should engage in hair removal to the sacrococcygeal region and surgical options considered. Hidradenitis suppurativa can be a morbid and challenging disease process. Medical management with topical agents, antibiotics, and biologics should be used initially but wide local excision should be considered in severe or refractory cases of the disease.

Squamous Cell Carcinoma Arising in Chronic Inflammatory Dermatoses.

Squamous cell carcinoma (SCC) is a known sequela of chronic inflammatory conditions of the skin. Labial discoid lupus erythema-tosus (DLE), oral lichen planus (OLP), and lichen sclerosus have a relatively short lag time from dermatosis onset to manifestation of malignancy; cutaneous DLE, hypertrophic lichen planus, chronic wounds, hidradenitis suppurativa (HS), and necrobiosis lipoidica can be present for decades before an associated malignancy is observed. Vigilant monitoring is essential for orolabial DLE, chronic HS, and chronic wounds because malignancies in these settings are particularly aggressive and often fatal. We summarize what is known about the nature and demographics of SCC arising within chronic inflammatory dermatoses, emphasizing lag time from dermatosis diagnosis to malignancy onset of common inflammatory conditions.

Autoimmune, Autoinflammatory Disease and Cutaneous Malignancy Associations with Hidradenitis Suppurativa: A Cross-Sectional Study.

BACKGROUND: Hidradenitis suppurativa (HS) is a debilitating cutaneous disease characterized by severe painful inflammatory nodules/abscesses. At present, data regarding the epidemiology and pathophysiology of this disease are limited. OBJECTIVE: To define the prevalence and comorbidity associations of HS. METHODS: This was a cross-sectional study of EPICTM Cosmos© examining over 180 million US patients. Prevalences were calculated by demographic and odds ratios (OR) and identified comorbidity correlations. RESULTS: All examined metabolism-related, psychological, and autoimmune/autoinflammatory (AI) diseases correlated with HS. The strongest associations were with pyoderma gangrenosum [OR 26.56; confidence interval (CI): 24.98-28.23], Down syndrome (OR 11.31; CI 10.93-11.70), and polycystic ovarian syndrome (OR 11.24; CI 11.09-11.38). Novel AI associations were found between HS and lupus (OR 6.60; CI 6.26-6.94) and multiple sclerosis (MS; OR 2.38; CI 2.29-2.48). Cutaneous malignancies were largely not associated in the unsegmented cohort; however, among Black patients, novel associations with melanoma (OR 2.39; CI 1.86-3.08) and basal cell carcinoma (OR 2.69; CI 2.15-3.36) were identified. LIMITATIONS: International Classification of Diseases (ICD)-based disease identification relies on coding fidelity and diagnostic accuracy. CONCLUSION: This is the first study to identify correlations between HS with melanoma and basal cell carcinoma (BCC) among Black patients as well as MS and lupus in all patients with HS.

Deroofing: A safe, effective and well-tolerated procedure in patients with hidradenitis suppurativa.

INTRODUCTION: Hidradenitis suppurativa (HS) is a recurrent, debilitating, chronic disorder of the pilosebaceous unit. Although advances in HS treatment have been made, more than 45% of patients remain dissatisfied with systemic treatment, and more than one-third are dissatisfied with surgical procedures. OBJECTIVES: A prospective, observational study on the deroofing procedures in HS with special attention paid to patient satisfaction and complications. METHODS: HS lesions were assessed clinically and by the use of ultrasound. Patients reported outcomes, including pain, itch and satisfaction, were measured at 24 h post-surgery by a numeric rating scale (NRS) ranging from 0 to 10. Additionally, the timeline of objective wound closure reported by patients in (weeks), in addition to the need for any analgesics use, were both evaluated. RESULTS: The mean closure time of the post-deroofing wound was assessed as 4.4 ± 1.9 weeks. A statistically longer time was necessary for complete closure in males than in females (4.9 ± 2.2 weeks and 3.9 ± 1.6 weeks, respectively; p = 0.046). The closure time correlated positively yet weakly with the HS tunnel's width (r = 0.27, p = 0.016) and length (r = 0.228, p = 0.044). Patients assessed mean pain at 24 h post-op as mild with 0.7 ± 1.2 points according to NRS, with no differences between sexes. Similarly, itch in the first 24 h was assessed as mild with 1.8 ± 1.1 points, without differences between sexes. No pain, itch or adverse events were reported after 1 week following deroofing. Moreover, no cases of wound infection were reported. An overall patient satisfaction was assessed as 9.9 ± 0.4 points (range 9-10 points). CONCLUSION: Deroofing is an easy, effective and safe dermatosurgical procedure that does not require surgical experience or operating theatre. It is associated with no complications and very low post-op pain and should be part of holistic HS management.

New treatment of pyoderma gangrenosum and hidradenitis suppurativa: A review.

Pyoderma gangrenosum (PG) and hidradenitis suppurativa (HS) are stubborn inflammatory skin diseases categorized as neutrophilic hypodermal dermatoses. These conditions exhibit connections with other autoinflammatory disorders driven by immune responses. Their pathogenesis is complex, rooted in significant imbalances in both innate and adaptive immune systems, particularly featuring elevated levels of tumor necrosis factor-α (TNF-α), interleukin (IL)-1, IL-8, IL-17, and IL-23. Studies involving skin tissue pathology and serology have indicated that targeting specific cytokines can bring therapeutic benefits. Indeed, many patients in clinical settings have responded positively to such interventions. Yet, given the diverse cytokines in play, focusing on a single one with antibody therapy might not always be effective. When resistance to biologics emerges, a combined approach targeting multiple overactive cytokines with immunosuppressants, for example cyclosporine and Janus kinase inhibitors, could be an option. In the current review, we explore recent therapeutic developments for PG and HS.

Hidradenitis suppurativa disease control associated with type of hormonal contraceptive use.

Hidradenitis suppurativa (HS) is a painful chronic skin condition of apocrine gland regions. This retrospective cohort study aimed to assess the impact of hormonal contraception type on HS disease control in adult women. In total, 160 patients were included, with the majority identifying as Black or African American (73.1%). Multivariate logistic regression showed that oestrogen-progesterone users were 3.14 times more likely to experience stable or improved HS than progesterone-only users (adjusted odds ratio 3.14, 95% CI 1.18-8.35; P = 0.02). Further investigation is needed to elucidate the antiandrogenic mechanisms affecting HS symptom response to hormonal contraceptives.

Use of multiple fasciocutaneous flaps for the management of extensive hidradenitis suppurativa.

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease with a relapsing nature that has a significant impact on the patient's quality of life. The clinical presentation of the disease includes deep dermal abscesses and sinus tracts. Long-term affliction of the disease may lead to contractures, scars and fibrosis. Management is still challenging and varies from medical to surgical options. We report the following case of a man in his 30s who presented with severe HS in the axillae, groins, perineum and lower sacral region. The patient has been treated by excision of the whole lesions followed by using multiple fasciocutaneous flaps as a reconstructive method.

Fibromyalgia in Patients With Hidradenitis Suppurativa.

This case-control study compares the prevalence of fibromyalgia between adults with hidradenitis suppurativa and adults with non­pain-related conditions.

Targeting Metabolic Syndrome in Hidradenitis Suppurativa by Phytochemicals as a Potential Complementary Therapeutic Strategy.

Hidradenitis suppurativa (HS) is a chronic inflammatory disease characterized by the appearance of painful inflamed nodules, abscesses, and pus-draining sinus tracts in the intertriginous skin of the groins, buttocks, and perianal and axillary regions. Despite its high prevalence of ~0.4-1%, therapeutic options for HS are still limited. Over the past 10 years, it has become clear that HS is a systemic disease, associated with various comorbidities, including metabolic syndrome (MetS) and its sequelae. Accordingly, the life expectancy of HS patients is significantly reduced. MetS, in particular, obesity, can support sustained inflammation and thereby exacerbate skin manifestations and the chronification of HS. However, MetS actually lacks necessary attention in HS therapy, underlining the high medical need for novel therapeutic options. This review directs attention towards the relevance of MetS in HS and evaluates the potential of phytomedical drug candidates to alleviate its components. It starts by describing key facts about HS, the specifics of metabolic alterations in HS patients, and mechanisms by which obesity may exacerbate HS skin alterations. Then, the results from the preclinical studies with phytochemicals on MetS parameters are evaluated and the outcomes of respective randomized controlled clinical trials in healthy people and patients without HS are presented.

Hidradenitis suppurativa and follicular occlusion syndrome: Where is the pathogenetic link?

The follicular occlusion tetrad complex encompasses several entities (hidradenitis suppurativa, acne conglobata, dissecting cellulitis of the scalp, and pilonidal cyst) that share common clinical features, risk factors, and pathophysiology. Follicular occlusion is a crucial triggering mechanism in the etiology in each of these disorders, leading to development of distinctive skin lesions such as deep-seated nodules, abscesses, comedones, and draining sinuses, often with accompanying scarring. Despite the fact that the follicular occlusion tetrad components manifest multiple similarities, they also exhibit many differences among themselves and require individual approaches and treatment.

Epidemiology of hidradenitis suppurativa.

Hidradenitis suppurativa (HS) is a chronic, inflammatory, recurrent skin disease affecting hair follicles in predominantly intertriginous areas, characterized by deep, painful nodules and abscesses, fistulas, sinus tracts, and scarring. The estimated global prevalence of HS is highly variable, as revealed in a growing body of published literature, and ranges from 0.053% to 4.1%. In North American and European patients, HS is three times more common in women than men, whereas in South Korea and Japan, male predominance is found. The disease most frequently manifests itself between the ages of 18 and 29. Numerous published studies have reported the association between smoking, obesity, and HS, although there are limitations in confirming the causal relationship due to the retrospective design of the available studies. Case-control studies have frequently evaluated the association between HS, metabolic syndrome, and other systemic comorbidities. Due to increased mental health problems, a higher risk of suicide in patients with HS has been reported. We provide up-to-date evidence about the epidemiology, genetic and environmental risk factors, comorbidities, and quality of life of patients with HS. The divergence in HS frequency, possibly due to differences in populations and methodologies, remains to be explained in future worldwide studies.

A loss-of-function NCSTN mutation associated with familial Dowling Degos disease and hidradenitis suppurativa.

Dowling Degos disease (DDD) is a rare autosomal dominant genodermatosis characterized by acquired, slowly progressive reticulated pigmented lesions primarily involving flexural skin areas. Mutations in KRT5, POGLUT-1 and POFUT-1 genes have been associated with DDD, and loss-of-function mutations in PSENEN, a subunit of the gamma-secretase complex, were found in patients presenting with DDD or DDD comorbid with hidradenitis suppurativa (HS). A nonsense mutation in NCSTN, another subunit of the gamma-secretase, was already described in a patient suffering from HS and DDD but whether NCSTN could be considered a novel gene for DDD is still debated. Here, we enrolled a four-generation family with HS and DDD. Through Whole Exome Sequencing (WES) we identified a novel nonsense mutation in the NCSTN gene in all the affected family members. To study the impact of this variant, we isolated outer root sheath cells from patients' hair follicles. We showed that this variant leads to a premature stop codon, activates a nonsense-mediated mRNA decay, and causes NCSTN haploinsufficiency in affected individuals. In fact, cells treated with gentamicin, a readthrough agent, had the NCSTN levels corrected. Moreover, we observed that this haploinsufficiency also affects other subunits of the gamma-secretase complex, possibly causing DDD. Our findings clearly support NCSTN as a novel DDD gene and suggest carefully investigating this co-occurrence in HS patients carrying a mutation in the NCSTN gene.

Azathioprine in hidradenitis suppurativa with inflammatory phenotype: a case series of 11 patients.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic inflammatory disease that negatively impacts the quality of life of patients. It presents as deep-seated nodules, abscesses, fistulae, sinus tracts, and scars in the axilla, inguinal area, submammary folds, and perianal area. Recently, two phenotypes have been described: a follicular phenotype and an inflammatory phenotype. Numerous medical treatments are available for hidradenitis suppurativa, with particular importance of antitumor necrosis factor antibodies. Due to the association of HS with other conditions with a pro-inflammatory state, particularly Crohn's disease, it has been suggested that azathioprine may have a role in the treatment of HS. OBJECTIVE: To assess the effectiveness of azathioprine monotherapy in patients with moderate-severe HS. METHODS: We retrospectively studied patients with HS treated with azathioprine in monotherapy. We performed both clinical and ultrasound evaluation at baseline as well as in the follow-up visits. Their baseline score on the iHS4 and DLQI scales and 12-16 weeks after starting the treatment were compared. We also registered the number of patients who achieved HiSCR. RESULTS: Six patients presented significant improvement, reducing their score in iHS4 and DLQI scales and achieving HiSCR. Another patient had clinical improvement, meaning reduction in iHS4 and DLQI, but without achieving HiSCR. Two patients stopped the treatment before week 12 because of adverse events. The remaining two patients presented no improvement. The median (Q3-Q1) baseline iHS4 score was 6 (12-6), and follow-up iHS4 score, 4 (6-2), being these differences statistically significant (P = 0.006). Median (Q3-Q1) baseline DLQI scores and 12-16 weeks after treatment were 17 (23-11) and 14 (18-9) although statistically nonsignificant (P = 0.099). CONCLUSION: We present a case series of 11 patients treated with azathioprine with good clinical and ultrasonographic response. We suggest that azathioprine may benefit a certain patient profile with the inflammatory phenotype.

Narrative Review and Update on Biologic and Small Molecule Drugs for Hidradenitis Suppurativa: An Entity With a Promising Future. / [Artículo traducido] Revisión narrativa y actualización en fármacos biológicos y de pequeña molécula para el tratamiento de la hidradenitis supurativa: una entidad con un futuro prometedor.

Hidradenitis suppurativa (HS) is a chronic inflammatory entity characterized by the appearance of multiple nodules, abscesses, and fistulas, predominantly in apocrine regions. In addition to its dermatological involvement, it is associated with multiple systemic comorbidities. Its treatment is combined: topical pharmacological, systemic pharmacological and surgical. Regarding biologic or small molecule drugs, currently only adalimumab is approved. A narrative review of the literature on biological or small molecule drugs used in the treatment of hidradenitis suppurativa is presented. The arsenal we found is large, with multiple targets: inhibitors of tumor necrosis factor alpha (TNF-alpha), interleukin (IL)-17, IL-23, IL-1, inhibitors of the janus kinase (JAK) pathway, and multiple other drugs in study. New prospective studies and comparative trials are needed to analyze the effectiveness and safety of these treatments, in an entity with a promising future.

Serum levels of serum amyloid A, interleukin-6 and C-reactive protein correlate with severity of hidradenitis suppurativa.

BACKGROUND: Validated biomarkers enabling an objective, dynamic assessment of hidradenitis suppurativa (HS) disease severity do not exist. The aim of our study was to determine the serum concentration of four potential biomarkers with respect to HS disease severity. METHODS: We recruited 50 patients with hidradenitis suppurativa. After obtaining informed consent, patients were requested to fill out multiple questionnaires. Severity of HS was determined based on Hurley and Sartorius scores by an experienced dermatologist. Blood sampling included Serum Amyloid A (SAA), Interleukin-6 (IL-6), C-reactive protein (CRP) and S100 protein (S100) in a certified laboratory. RESULTS: Moderate and statistically significant correlations of SAA, IL-6 and CRP with the clinical scores Hurley and Sartorius were observed. The respective Spearman's correlation coefficients (r) were: Hurley 0.38, 0.46, 0.35 and Sartorius 0.51, 0.48, 0.48. No relevant changes were detected when comparing S100 to both Hurley (r=0.06) and Sartorius (r=0.09). CONCLUSIONS: Our data suggest that an association between SAA, IL-6, CRP and HS disease severity could exist. Further research is needed to define their potential as biomarkers for quantifying and monitoring disease activity and response to treatment.