Hidradenitis Suppurativa
Hidradenitis suppurativa (HS) is a chronic, follicular occlusive, inflammatory skin condition. It affects the intertriginous skin areas of the axillary, groin, perianal, perineal, and inframammary regions. It presents as recurrent, inflamed nodules and ab
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48
Jacob Bornstein
48.1 Introduction Hidradenitis suppurativa (HS) is a chronic, follicular, occlusive, inflammatory skin condition. It is also called “acne inversa” and “Verneuil’s disease” [1]. HS affects the intertriginous skin areas of the axillary, groin, perianal, perineal, and inframammary regions. It presents as recurrent, inflamed nodules and abscesses, draining sinus tracts and bands of severe scar formation. It is associated with pain, bad odor, discharge, and disfigurement [2]. Its prevalence is 1–4% [3]; most cases are mild. It usually begins before age of 40, in women twice as much as men.
48.2 Pathogenesis Past theory assumed that apocrine glands are involved. Currently follicular occlusion or rupture with an associated immune response is thought to cause the disease. There is a genetic susceptibility: 40% of patients with HS have an affected first-degree family member [4]. A candidate locus for HS is at chromosome 1p21.1–1q25.3.
Other risk factors are pressure and friction on intertriginous skin, obesity, and smoking. Despite abscess formation, the early, unruptured HS lesions are sterile. Older and ruptured lesions and sinuses may grow staphylococci, streptococci, Gram-negative rods, anaerobic bacteria, and coagulase-negative staphylococci. Positive cultures may represent contaminants from normal skin flora or secondary infection. Oral contraceptives containing androgenic progestins, intramuscular medroxyprogesterone acetate, or levonorgestrel intrauterine device may precipitate or worsen HS [5]. Paradoxically, HS has been reported as an adverse effect of anti-TNF- alpha therapies and other biologic treatments given for other chronic inflammatory diseases.
48.3 Clinical Presentation HS develops in intertriginous areas: the axillae, inguinal area, inner thighs, perianal and perineal areas, mammary and inframammary regions, buttocks, pubic region, scrotum, vulva, and trunk. The primary lesions are inflammatory nodules, sometimes with suppurative discharge. Sinus formation, clusters of open comedones and scar formation are seen in
J. Bornstein (*) Department of Obstetrics and Gynecology Galilee Medical Center, Bar-Ilan University Faculty of Medicine, Nahariya, Israel © Springer International Publishing AG, part of Springer Nature 2019 J. Bornstein (ed.), Vulvar Disease, https://doi.org/10.1007/978-3-319-61621-6_48
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48.5 Histopathology Early lesions have characteristics of follicular hyperkeratosis, plugging, follicular dilation, and lymphocytic perifolliculitis [8]. Established areas of disease demonstrate also psoriasiform hyperplasia of the interfollicular epithelium [8] or a dense, mixed inflammatory infiltrate in the dermis. In addition, chronic abscesses, sinus tracts, and granulation tissue—sometimes with foreign body giant cells—may be seen. Destruction of folliculo-pilosebaceous units and fibrosis are characteristics of advanced disease.
48.6 Diagnosis The diagnosis of HS is made by revealing the characteristic history and location of the lesions— in the a
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