Effective long-term control of refractory hidradenitis suppurativa with adalimumab after failure of conventional therapy

Background Hidradenitis suppurativa is a chronic suppurative condition featuring inflammatory nodules, fistulas and scars. It occurs predominantly in the axillae and groin. The disease is poorly responsive to any treatment and is connected with significant morbidity. Systemic therapy, including oral antibiotics, retinoids and antiandrogens, usually has only limited effect. Surgical treatment of affected areas is necessary in advanced stages. Objectives Several reports support the beneficial effect of tumor necrosis factor‐α (TNF‐α) antagonists for the treatment of severe hidradenitis suppurativa. By contrast with data on infliximab and etanercept, data describing the potential positive influence of adalimumab on disease outcome are limited and refer to only small cohorts of patients. Methods Eight patients with severe, recalcitrant hidradenitis were treated for 1 year with adalimumab in a standard regimen and were subsequently followed for 1 year. Results All patients improved within 4–6 weeks and laboratory parameters of C‐reactive protein (CRP) and leukocyte count reduced significantly during treatment. Three patients demonstrated long‐lasting improvement and five showed recurrences several months after discontinuation of the therapy. The average recurrence‐free interval was 9.5 months. Conclusions Adalimumab is suitable for the long‐term treatment of hidradenitis suppurativa and presents a further conservative treatment approach.     

Lichen sclerosus and hidradenitis suppurativa: Two case reports of a new possible association

We report the cases of two women affected by lichen sclerosus also having clinical signs of hidradenitis suppurativa. Lichen sclerosus is a chronic autoimmune disease, in which activated fibroblasts produce significantly altered collagen leading to fibrosis Hidradenitis suppurativa is a chronic relapsing inflammatory disease affecting folliculopilosebaceous unit and apocrine gland, which lesions are nodules and abscesses. The association between lichen sclerosus and autoimmune disorders is well known, but not the one with hidradenitis suppurativa. We present two case reports of these unusual comorbidities.     

Two diseases one remedy? Systemic amyloidosis secondary to hidradenitis suppurativa: Treatment with infliximab

Hidradenitis suppurativa, known as acne inversa, is a relapsing and chronic inflammatory skin disease affecting the skin folds. During the chronic course of the disease many local complications like fistulae to other tissues or systemic complications including anemia, secondary amyloidosis, lymphedema, nephrotic syndrome, artropathy may take place. Amyloid A amyloidosis is a rare complication of hidradenitis suppurativa, which has been described in a limited number of case reports. Herein, we present such a patient that had developed AA amyloidosis during the course of hidradenitis suppurativa. Both AA amyloidosis and hidradenitis suppurativa have responded to infliximab therapy which was shown by clinical recovery and by the improvement in renal functions.     

Efectividad de la terapia fotodinámica con ácido 5-aminolevulínico en el tratamiento de la hidrosadenitis supurativa. Serie de 5 casos

Hidradenitis suppurativa has been described as a chronic, recurrent, and disabling inflammatory disease involving the entire hair follicle.Several treatments, including photodynamic therapy, have been used, but the results have been inconsistent and recurrence is high.In this prospective study, we evaluated disease severity, quality of life, and treatment tolerance in 5 patients with moderate to severe hidradenitis suppurativa treated with photodynamic therapy using 5-aminolevulinic acid and a 635-nm light source. Treatment effectiveness was evaluated using the Sartorius severity score, the Dermatology Life Quality Index, and a visual analog scale for pain and disease activity.Significant improvements were observed with all 3 instruments and the effects remained visible at 8 weeks. Our results suggest that photodynamic therapy with 5-aminolevulinic acid and a light wavelength of 635 nm could reduce disease severity and improve quality of life in patients with difficult-to-treat hidradenitis suppurativa.     

Hidradenitis suppurativa resulting in systemic amyloid A amyloidosis: a case report and review of the literature

Hidradenitis suppurativa is a chronic, inflammatory disease of the follicular epithelium that presents as tender, subcutaneous nodules in an intertriginous distribution with sinus tract formation. Most commonly, hidradenitis suppurativa results in local complications, such as scarring and infection. However, systemic complications, such as anemia and arthropathies, have also been described. Herein, we report an unusual case of systemic amyloid A secondary to hidradenitis suppurativa. We describe a 39-year-old man with a long history of recurrent, tender, erythematous nodules in the axillary and anogenital regions, resulting in abscesses, sinus tract formation, and large areas of scarring. After 21 years of cutaneous disease with concurrent elevated systemic inflammatory markers, the patient was noted to have significant proteinuria. A kidney biopsy and immunostaining revealed deposits of amyloid A. Echocardiogram and electrocardiogram showed ventricular and atrial wall thickening with an appearance consistent with cardiac amyloid deposition. Systemic amyloid A amyloidosis is a serious, but rare, complication of chronic inflammatory disorders, including hidradenitis suppurativa, with potential multi-organ involvement including renal and cardiac manifestations. Amyloid A amyloidosis should be suspected in patients with chronic inflammatory cutaneous diseases who present with renal abnormalities, especially proteinuria or the nephrotic syndrome.     

Adalimumab: A Review in Hidradenitis Suppurativa

Subcutaneous adalimumab (Humira^®) is a tumour necrosis factor-α blocker that is the only approved agent for the treatment of moderate to severe hidradenitis suppurativa (HS) in several countries worldwide. This article reviews the clinical efficacy and safety of subcutaneous adalimumab in patients with moderate to severe HS. In clinical trials (PIONEER I and II), a greater proportion of adalimumab than placebo recipients reached HS clinical response (HiSCR) at week 12. The main secondary endpoints, such as the proportion of patients with an abscess and inflammatory nodule count of ≤2 at week 12, were significantly greater with adalimumab than with placebo in PIONEER II, but not in PIONEER I. In addition, adalimumab showed the potential to reduce the high health-related quality of life burden of HS and increase patient satisfaction. HiSCR rates were generally maintained in the longer term, and the safety profile of adalimumab in patients with moderate to severe HS was consistent with the known safety profile of the drug for other indications, with no new emerging safety signals. Adalimumab is an effective and generally well tolerated treatment for patients with moderate to severe HS, and is the first agent approved for this difficult-to-treat disease.     

Severe vulval apocrine acne successfully treated with prednisolone and isotretinoin

Apocrine acne, otherwise known as hidradenitis suppurativa, is a chronic inflammatory scarring disease affecting the apocrine gland-bearing skin. We present a case of a 34-year-old woman with severe vulval apocrine acne who was successfully treated initially with prednisolone and then maintained on long-term isotretinoin. This case indicates that long-term treatment with isotretinoin may be more successful than the usual 4-6 months treatment regime.     

Prevalence of hidradenitis suppurativa among patients with Down syndrome: a population‐based cross‐sectional analysis

Hidradenitis suppurativa (HS) is a debilitating, chronic, painful inflammatory disease. Scientists have previously suggested that there is a potential genetic link predisposing Down syndrome patients to the development of HS through the increased formation of the amyloid precursor protein. However, this laboratory based finding had not previously been verified in the population. In this study, we evaluated the commonness of hidradenitis suppurativa within a large group of patients who had Down syndrome, and we used another groups of patients that did not have Down syndrome to compare findings. We observed that the diagnosis of hidradenitis suppurativa was present among 2.1% of patients with Down syndrome over the past five years. Compared to those without Down syndrome, patients with the condition had five times the likelihood of having hidradenitis suppurativa during the period of the study. Hidradenitis suppurativa was most common among Down syndrome patients who were aged 18–29 years. Hidradenitis suppurativa affected males and females, and whites and non‐whites, with Down syndrome equally. The diagnosis of hidradenitis suppurativa is made at a younger age among patients with Down syndrome, compared to patients without the condition. This suggests that hidradenitis may occur earlier in life among patients with Down syndrome, or it may occur more severely and thereby prompt earlier visits to the doctor for this condition. It is also possible that hidradenitis suppurativa is diagnosed earlier among patients with Down syndrome because doctors may be examining patients with Down syndrome more frequently in general.     

[Artículo traducido] Escisión amplia en hidradenitis supurativa. ¿Modifica el curso de la enfermedad

There is a lack of studies assessing whether wide excision surgery in hidradenitis suppurativa affected areas is useful for the global control of the hidradenitis suppurativa. We aimed to find whether surgery results were a better global control on the disease activity in both, the area where the surgery is performed and distant areas. We evaluated the disease course of 17 patients with hidradenitis suppurativa who underwent wide excision of complex fistula tracts between October 2018 and January 2022 at the Hospital Universitario de La Princesa, Madrid. We found that wide excision of complex fistulas produces an overall positive effect on the inflammatory activity in hidradenitis suppurativa that may be important to achieve an adequate control of the disease.     

Wide Excision in Hidradenitis Suppurativa. Does it Modify the Course of Disease?

There is a lack of studies assessing whether wide excision surgery in hidradenitis suppurativa affected areas is useful for the global control of the hidradenitis suppurativa. We aimed to find whether surgery results were a better global control on the disease activity in both, the area where the surgery is performed and distant areas. We evaluated the disease course of 17 patients with hidradenitis suppurativa who underwent wide excision of complex fistula tracts between October 2018 and January 2022 at the Hospital Universitario de la Princesa, Madrid. We found that wide excision of complex fistulas produces an overall positive effect on the inflammatory activity in hidradenitis suppurativa that may be important to achieve an adequate control of the disease.     

Combination hyperbaric oxygen therapy and ustekinumab for severe hidradenitis suppurativa

Hidradenitis suppurativa is a painful chronic inflammatory skin condition characterized by inflammatory nodules that can lead to sinus tracts and scarring. Numerous treatments have been reported, though none have reliable efficacy. Antiinflammatory agents, such as tumor necrosis factor‐alpha inhibitors and interleukin inhibitors, have been used as medical therapy for refractory cases. We describe here a case of severe hidradenitis suppurativa in a pediatric patient successfully treated with a combination of high‐dose ustekinumab and hyperbaric oxygen therapy.     

A gene dysfunction module reveals the underlying pathogenesis of hidradenitis suppurativa: An update

Hidradenitis suppurativa is a chronic skin disease characterised by repeated skin abscesses with sinus tracts and scar formation. Currently, the aetiology and pathogenesis of hidradenitis suppurativa remain unclear. Genetic factors, immune disorders, hormonal abnormalities, skin-microbial dysbiosis, smoking, obesity and mechanical friction all influence hidradenitis suppurativa pathogenesis. Moreover, hidradenitis suppurativa has a familial subset with autosomal dominant transmission proposed and is related to a mutation of γ-secretase component genes. In this review, we analyse and summarise research progress regarding the relationship between the γ-secretase gene dysfunction and the pathogenesis of hidradenitis suppurativa.     

Management of Hidradenitis Suppurativa in Patients with Metabolic Comorbidities

Hidradenitis suppurativa is a chronic inflammatory skin condition associated with an increased prevalence of individual metabolic conditions such as insulin resistance, obesity, hyperlipidemia, hypertension, and with the metabolic syndrome, as a constellation of these risk factors. This places affected patients at an increased risk of early cardiovascular morbidity and mortality. Moreover, many of the therapeutic options, including the newer biologics, used in the treatment of hidradenitis suppurativa have both beneficial and adverse metabolic effects. Therefore, it is critical for physicians to consider the complex interactions between the disease process and the treatment options in the holistic management of these patients with an intrinsically higher risk of metabolic consequences. Other chronic systemic inflammatory diseases such as psoriasis and rheumatoid arthritis have been studied more extensively with regard to their associations and share an underlying link with the metabolic syndrome; we can draw upon the existing knowledge in our understanding and management of hidradenitis suppurativa.     

Risk factors for hidradenitis suppurativa: a pilot study

The hidradenitis suppurativa is a chronic debilitating inflammatory disease whose etiology is not fully understood. We conducted a pilot case-control study matched by sex and age with other dermatological patients to analyze possible risk factors associated with this disease. We included 15 cases and 45 controls, 67% were women. Bivariate and multivariate logistic regression analysis identified significant association with smoking, higher body mass index and family history. The use of hormonal contraceptives was less frequent in women with hidradenitis.     

Long-term efficacy of a single course of infliximab in hidradenitis suppurativa

BACKGROUND: Hidradenitis suppurativa is a chronic inflammatory skin disease characterized by abscess formation, predominantly in the axillae and groins. The disease is difficult to treat and has a severe impact on quality of life. Recently, several case reports have been published describing successful treatment of hidradenitis suppurativa with infliximab and other tumour necrosis factor alpha inhibitors. OBJECTIVES: To evaluate the long-term efficacy of a single course of infliximab. METHODS: Ten patients with severe, recalcitrant hidradenitis were treated with infliximab (three infusions of 5 mg kg(-1) at weeks 0, 2 and 6) and followed up for at least 1 year. The disease activity was measured using laboratory parameters and a recently developed acne score. The patients rated the efficacy of infliximab on a 10-point scale at regular intervals. Quality of life was measured before and after treatment using the Dermatology Quality of Life Index (DQLI). RESULTS: All patients improved within 2-6 weeks. The average acne score diminished from 164+/-50 (mean+/-SD) before treatment to 89+/-49 after 1 year (P=0.002). The mean CRP (C-reactive protein) was reduced from 31.7 mg mL(-1) to 5.5 mg mL(-1) after 1 month (P=0.015). Patients judged the efficacy with a score of 7.9. The mean DQLI was reduced from 18.4+/-7.9 before treatment to 9.3+/-9.1 after 1 year (P=0.007). In three patients long-lasting improvement was observed, with no recurrence of lesions in a 2-year follow-up period. The other patients showed recurrence of lesions after 8.5 months (range 4.3-13.4 months). CONCLUSIONS: Infliximab is an effective treatment in severe hidradenitis suppurativa, leading to reduction of symptoms for a prolonged period.     

Hidradenitis suppurativa in a patient with Warkany syndrome: Sustainability of adalimumab treatment

Mental retardation is a potential limitation to self‐administration of innovative biologic treatment, which is otherwise very effective for devastating chronic inflammatory conditions, such as hidradenitis suppurativa (HS). We report our successful experience with a 34‐year‐old woman, who was genetically affected by Warkany syndrome, which causes intellectual disability. This patient was unable to maintain long‐term antibiotic regimen and was disappointed by the limited results in her 12 years' history of painful draining fistulae and abscesses; however, she enthusiastically adhered to adalimumab 40 mg/once weekly treatment. After 52 weeks, treatment compliance is excellent, the patient is satisfied with the HS improvements, and no adverse effects have been reported.     

Cigarette smoking as a triggering factor of hidradenitis suppurativa.

BACKGROUND: Hidradenitis suppurativa is a chronic inflammatory skin disease involving the axillary, inguinal and anogenital regions and sometimes, in addition, the submammary or sacral areas. The etiology of this condition is unknown. OBJECTIVE: A matched-pair case-control study was performed to evaluate the influence of smoking habits on the manifestation of this disease. METHODS: Patients who had received surgical treatment for hidradenitis suppurativa in two dermatological centers completed a questionnaire dealing with family history, course of the disease and smoking habits. To form a randomized matched-pair control group, an equal number of patients admitted for various other skin diseases such as atopic dermatitis, varicose veins, skin tattoos, alopecia areata or melanoma was matched for sex and age and evaluated for smoking habits. Statistical analysis was performed by use of several chi2 tests in a cross-table setting. Moreover, a comparison to the expected smoking prevalence in Germany based on national statistics was performed. RESULTS: Out of 84 patients treated for hidradenitis suppurativa, 63 subjects (27 men, 36 women) completed the questionnaire. The rate of active cigarette smokers was 88.9% (56 patients), whereas 4 subjects (6.4%) had never smoked. 3 patients (4.8%) stated to be ex-smokers, but 2 of these had quit smoking only recently and after onset of the disease. The rate of smokers in the matched-pair control group was 46%. The significantly higher proportion of active smokers among patients with hidradenitis suppurativa can be expressed by an odds ratio of 9.4, the calculated 95% confidence interval was 3.7-23.7 (p < 0.001). The expected smoking prevalence in Germany was 26.7% according to national statistics. 73% of our patients had no family history of hidradenitis suppurativa whereas 27% reported at least one affected first-degree relative. CONCLUSION: From the exceedingly high rate of smokers among patients with this condition we conclude that cigarette smoking is a major triggering factor of hidradenitis suppurativa. Remarkably, the disease can be categorized as a smoking sequel that is neither of vascular nor neoplastic nature. Because familial occurrence was rather rarely reported, and because an environmental factor in the form of cigarette smoking appears to be of crucial importance to trigger the disease, we assume that the genetic basis of hidradenitis suppurativa is polygenic rather than mendelian. Smoking cessation should be encouraged particularly in patients with hidradenitis suppurativa although it is unknown whether this improves the course of the disease.     

Treating hidradenitis suppurativa with photodynamic therapy

Hidradenitis suppurativa is a chronic, recurring, and disabling inflammatory condition of the skin. There is no cure for hidradenitis suppurativa and treatment must be adapted to each individual patient. Several studies have been published since 2004 on the use of photodynamic therapy to treat hidradenitis suppurativa. The use of superficial or interstitial illumination with 5-amino-levulinic acid (5-ALA) or methylene blue (MB) have been proposed. Injecting 5-ALA or MB followed by illumination with a fiber optic sensor placed inside the lesion appears to be a better method of treating these thick lesions.     

Hidradenitis suppurativa and the metabolic syndrome

The association of the metabolic syndrome (MetS) and its components with immune-mediated chronic inflammatory disorders has attracted much interest within the last two decades. In addition to the well-established association of psoriasis with MetS, recent data point to an association between MetS and hidradenitis suppurativa, as well. The association of hidradenitis suppurativa with MetS and its components, such as diabetes, obesity, and dyslipidemia, has been consistently identified in controlled studies. This relationship is not only limited to older individuals but also applicable to younger patients and those with mild disease. This review documents the link between these disorders and discusses its practical implications, mainly highlighting the importance of prevention and routine screening for early diagnosis to prevent cardiovascular-related morbidity.     

Reticulate pigmented anomaly of the flexures (Dowling-Degos disease): a case report associated with hidradenitis suppurativa and squamous cell carcinoma

We report the first case of reticulate pigmented anomaly of the flexures (Dowling-Degos disease) associated with hidradenitis suppurativa. Our patient also showed a squamous cell carcinoma of the groin, which is a rare complication of hidradenitis suppurativa. The relationship between Dowling-Degos disease and hidradenitis is discussed and the literature on squamous cell carcinoma and hidradenitis suppurativa is reviewed.