化脓性汗腺炎一家系

先证者,男,29岁。面部丘疹、脓疱9年,加重4年。家系中3代15人成员中共有7例患者（男4例,女3例）,均于青春期前后发病,临床特点及病理特点符合化脓性汗腺炎的诊断。     

The nature and consequence of Karl Marx's skin disease

From an analysis of the original correspondence, it has been possible to establish that Karl Marx's incapacitating skin disease was hidradenitis suppurativa, not 'boils' as was universally assumed at the time and since; the psychological effect of this illness on the man and his work appears to have been considerable.     

Clinical,microbiological, immunological and imaging characteristics of tunnels and fistulas in hidradenitis suppurativa and Crohn’s disease

Hidradenitis suppurativa (HS) tunnels and Crohn's disease (CD) fistulas are a challenge to treat. Although pathogenic similarities have been described between HS and CD, recent studies indicate that clinical, microbiological, immunological and imaging characteristics differ between these diseases. This review highlights the differences between HS tunnels and CD fistulas. Next-generation sequencing studies demonstrate a microbiome in HS tunnels dominated by Porphyromonas spp., Prevotella spp. whereas no specific bacteria have been associated with cutaneous CD. Immunologically, TNF has been found upregulated in HS tunnels along with various interleukins (IL-8, IL-16, IL-1α and IL-1β). In CD fistulas, Th1, Th17, IL-17, IFN-ɤ, TNF and IL-23 are increased. US imaging is an important tool in HS. US of HS tunnels depict hypoechoic band-like structure across skin layers in the dermis and/or hypodermis connected to the base of a widened hair follicle. In CD, MR imaging of simple perianal fistulas illustrates a linear, non-branching inflammatory tract relating to an internal opening in the anus or low rectum and an external opening to the skin surface. An increased awareness of the immediate potential differences between HS tunnels and CD fistulas may optimize treatment regimens of these intractable skin manifestations.     

Treatment of severe recalcitrant hidradenitis suppurativa with adalimumab

Hidradenitis suppurativa is a chronic inflammatory skin disease featuring inflammatory nodules, fistulas and discharge of secretions in the intertriginous regions. Without therapy the disease is chronic and progressive. The most effective treatment is the radical wide excision of the affected areas. Systemic antibiotics, immunosuppressants, oral retinoids or antiandrogens have limited beneficial effects. TNF‐α antagonists may represent a new therapeutic approach for patients suffering from severe hidradenitis suppurativa offering promising positive influence on the disease outcome. In contrast to infliximab and etanercept, there are only few reports describing the use of adalimumab in severe hidradenitis suppurativa. Here we report a case of a patient with severe recalcitrant hidradenitis suppurativa successfully treated with adalimumab.     

An open-label phase II study of the safety and efficacy of etanercept for the therapy of hidradenitis suppurativa

OBJECTIVE: To evaluate the safety and efficacy of etanercept for the management of hidradenitis suppurativa. METHODS: In a prospective open-label phase II study, etanercept was administered subcutaneously in a dose of 50 mg once weekly for 12 weeks in 10 patients. They were followed up to 24 weeks and their disease activity and Sartorius score were assessed, with also a self-evaluation by visual analogue scale (VAS). Disease activity was an assessment of the extent of the disease by the attending physicians who were unaware of the protocol. RESULTS: A >50% score improvement was found in six patients at week 12 and in seven patients at week 24. The VAS was decreased compared with baseline in seven patients at week 12 and in six patients at week 24. All changes were statistically significant. All patients reported a decrease of local pain at the site of lesions after week 4. Drainage of pus from the affected areas recurred in eight patients within 4-8 weeks after the end of administration of etanercept. The treatment was well-tolerated. CONCLUSIONS: Etanercept is a safe and effective therapy for hidradenitis suppurativa and decreases the extent of the disease and improves the quality of life. A double-blind, placebo-controlled trial is required to elucidate fully the role of etanercept for hidradenitis suppurativa.     

Outcomes of treatment of nine cases of recalcitrant severe hidradenitis suppurativa with carbon dioxide laser

Background Hidradenitis suppurativa (HS) is a chronic and often a recalcitrant inflammatory skin condition. Objectives To present the results of carbon dioxide (CO₂) laser treatment of recalcitrant HS in nine patients who had failed to improve on medical and other surgical treatments. Methods HS lesions consisting of abscesses, sinuses and granulation tissue were completely excised using the cutting mode of a CO₂ laser, leaving only healthy residual subcutaneous fat. The wounds were closed by primary intention where possible and left to granulate otherwise. Outcomes were determined by clinical review and questionnaire. Results Twenty‐seven sites were treated in 19 sessions on nine patients. Seven procedures were performed under general anaesthesia and 12 under local. All patients rated their postoperative discomfort as less or equal to their preoperative state. Seven of the nine patients had complete remission for 12 months or longer after their last laser treatment and ceased all medications. High levels of patient satisfaction were reported with CO₂ laser treatment. The main complication was axillary scar contracture in two patients but this was insufficient to limit limb movement. Conclusions CO₂ laser treatment should be considered as a treatment option in recalcitrant HS, where multiple medical treatments have been ineffective.     

What causes hidradenitis suppurativa ?—15 years after

The 14 authors of the first review article on hidradenitis suppurativa (HS) pathogenesis published 2008 in EXPERIMENTAL DERMATOLOGY cumulating from the 1st International Hidradenitis Suppurativa Research Symposium held March 30–April 2, 2006 in Dessau, Germany with 33 participants were prophetic when they wrote “Hopefully, this heralds a welcome new tradition: to get to the molecular heart of HS pathogenesis, which can only be achieved by a renaissance of solid basic HS research, as the key to developing more effective HS therapy.” (Kurzen et al. What causes hidradenitis suppurativa? Exp Dermatol 2008;17:455). Fifteen years later, there is no doubt that the desired renaissance of solid basic HS research is progressing with rapid steps and that HS has developed deep roots among inflammatory diseases in Dermatology and beyond, recognized as “the only inflammatory skin disease than can be healed”. This anniversary article of 43 research-performing authors from all around the globe in the official journal of the European Hidradenitis Suppurativa Foundation e.V. (EHSF e.V.) and the Hidradenitis Suppurativa Foundation, Inc (HSF USA) summarizes the evidence of the intense HS clinical and experimental research during the last 15 years in all aspects of the disease and provides information of the developments to come in the near future.     

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.