What should a surgeon know about hidradenitis suppurativa?

Otherwise known as Verneuil's disease, hidradenitis suppurative (HS) is a severe dermatosis of heterogeneous appearance affecting 1% of the population. Its pathophysiology is multifactorial, involving genetic predisposition, inflammatory disorder and environmental elements. Its diagnosis is based on the association of three clinical characteristics: characteristic lesions, typical localizations, and the chronic and recurrent nature of the lesions. Given its diversified aspects, diagnosis can be difficult to achieve. As its manifestations often include abscesses, it is important for the surgeon to know how to detect this pathology. The development of biologics has improved treatment of this disease, treatment that necessitates a multidisciplinary medical and surgical approach involving dermatologists and proctologists as well as surgeons. The objective of this report is to synthesize what a surgeon will need to know so as effectively treat HS patients.

Case Report: Comorbid Hyper-IgD Syndrome and Hidradenitis Suppurativa - A New Syndromic Form of HS? A Report of Two Cases.

Hidradenitis Suppurativa (HS) is a chronic suppurative disease of the pilosebaceous unit. The current model of HS pathophysiology describes the condition as the product of hyperkeratinisation and inflammation at the hair follicular unit. Environmental factors (such as smoking and obesity), gender, genetic predisposition, and skin dysbiosis are considered the main pathogenic drivers of the disease. Autoinflammatory syndromes associated with HS are rare but may help to highlight the potential roles of autoinflammation and dysregulated innate immune system in HS. Therefore, it is of major relevance to increase the awareness about these diseases in order to improve the understanding of the disease and to optimize the management of the patients. Herein, we report for the first time, to our knowledge, two clinical cases of Hyper-IgD syndrome-associated HS. Hyper-IgD is an autoinflammatory syndrome caused by a mevalonate kinase deficiency (MKD), a key kinase in the sterol and isoprenoid production pathway. We describe the potentially shared pathophysiological mechanisms underpinning comorbid MKD-HS and propose therapeutic options for the management of these patients.

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.

Clinical characteristics of pediatric hidradenitis suppurativa: a cross-sectional multicenter study of 140 patients.

Hidradenitis suppurativa (HS) rarely affects pediatric patients. The literature on pediatric HS patients is scarce. This is a cross-sectional study based on case note review or interviews and clinical examination of 140 pediatric patients undergoing secondary or tertiary level care. Patients were predominantly female (75.5%, n = 105) with a median age of 16. 39% reported 1st-degree relative with HS. Median BMI percentile was 88, and 11% were smokers (n = 15). Median modified Sartorius score was 8.5. Notable comorbidities found were acne (32.8%, n = 45), hirsutism (19.3%, n = 27), and pilonidal cysts (16.4%, n = 23). Resorcinol (n = 27) and clindamycin (n = 25) were the most frequently used topical treatments. Patients were treated with tetracycline (n = 32), or oral clindamycin and rifampicin in combination (n = 29). Surgical excision was performed in 18 patients, deroofing in five and incision in seven patients. Obesity seemed to be prominent in the pediatric population and correlated to parent BMI, suggesting a potential for preventive measures for the family. Disease management appeared to be similar to that of adult HS, bearing in mind that the younger the patient, the milder the disease in majority of cases.

Hidradenitis Suppurativa or Hidradenitis Suppurativa-Like Lesions Located on Amputation Stumps? Description of 2 Cases.

Hidradenitis suppurativa (HS) is a chronic and recurrent inflammatory skin disorder affecting mainly the areas rich in apocrine sweat glands, such as the axillae, groins and buttocks. The role of mechanical pressure and friction due to clothing in the pathogenesis of HS lesions has been previously stressed. Here, we report 2 middle-aged men who presented with HS lesions/HS-like lesions on their amputation stump and review 2 additional cases from the literature. Management was challenging as 2 patients needed tumor necrosis αinhibitor while deroofing/surgery was the option for the 2 others. These cases highlight that mechanical pressure and friction are environmental factors that can play a role in the pathogenesis of HS lesions.

How to make a Barrett esophagus: pathophysiology of columnar metaplasia of the esophagus.

Barrett esophagus is defined as a specialized intestinal replacing the squamous epithelium of the esophageal mucosa in response to gastroesophageal reflux. Barrett metaplasia is a healing process that develops to protect the esophagus from further damage. Although mechanisms by which Barrett metaplasia evolves toward dysplasia and adenocarcinoma have been extensively studied, the process by which squamous epithelium is replaced by specialized intestinal metaplasia is poorly understood. Barrett esophagus develops when defense mechanisms in the esophageal mucosa (luminal secretion of mucus, bicarbonate, growth factors, etc.) are overwhelmed by an ongoing cycle of mucosal injury and repair. Hydrogen ion, pepsin, trypsin, and bile acids are considered harmful agents that synergistically invade the esophageal mucosa. Areas of destroyed squamous epithelium are then progressively reepithelized by a columnar epithelium that may originate from multipotent stem cells located within the basal layer of the normal esophageal mucosa or in the ducts of submucosal glands.

Etilefrine use in the management of post-operative chyle leaks in thoracic surgery.

Etilefrine, a sympathomimetic drug, was used 11 times in 10 patients with thoracic (n=8) or abdominal (n=2) chyle leak occurring after thoracic surgical procedures. It was given as a 4.2-5 mg/h intravenous infusion. During the 11 etilefrine administrations, three patients had total parenteral nutrition, three had enteral nutrition, three had oral fat-free diet and medium-chain triglyceride supplementation, and two were fed orally without restriction. Daily chyle flow output decreased in all but one patient who was reoperated. Chyle flow output did not decrease relevantly in one patient who was reoperated. Chylothorax recurred after reoperation and etilefrine then induced significant output decrease. In another patient, etilefrine was stopped despite significant output reduction because of interactions with other sympathomimetic drugs used for heart failure. The mean etilefrine treatment duration was 6.4 days (range 4-7). The mean daily output was from 740 ml before etilefrine infusion to 183 ml on the seventh day of etilefrine use. By inducing contraction of the smooth muscle fibres present in the wall of the main thoracic chyle ducts, etilefrine can be considered as a useful adjunct in the management of post-operative chyle leak.

[Fundic endocrine tumors and atrophic gastritis: the value of antrectomy]. / Tumeurs endocrines fundiques sur gastrite atrophique: intérêt de l'antrectomie.

We report two cases of fundic endocrine tumors associated with fundic atrophic gastritis-related hypergastrinemia and treated by antrectomy. About twenty infracentimetric tumors were observed in each patient and restrictively involved fundic mucosa. Antrectomy induced normalization of gastrinemia and the disappearance of fundic tumors, as soon as the 4(th) and 7(th) month. No recurrence was observed during the 5-year and 18-month follow-up time, respectively. We therefore consider that antrectomy may be regarded as a useful alternative treatment in the management of atrophic gastritis-related fundic endocrine tumors.