Hidradenitis Suppurativa
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A guide to Hidradenitis suppurativa Dr Liezel Griffin, Specialist Registrar in Dermatology, Dermatology Centre, Salford Royal NHS Foundation Trust, Salford Dr David Fitzgerald, Consultant Dermatologist, Dermatology Centre, Salford Royal NHS Foundation Trust, Salford, Manchester M6 8HD Correspondence to: Dr D Fitzgerald ([email protected]) idradenitis suppurativa is a chronic remains unclear (Fitzsimmons et al, 1985; Von Classification inflammatory disease of the hair follicle der Werth et al, 2000; Pink et al, 2013). Hurley system H(Zouboulis et al, 2015). It is also termed Hormonal factors may influence the severity The Hurley system is a grading tool used to acne inversa because of its close association of hidradenitis suppurativa in women, with assess the severity of hidradenitis suppurativa with acne vulgaris. In contrast to acne exacerbation related to menstruation (Jemec, in clinical practice (Hurley, 1989). The three vulgaris, hidradenitis suppurativa primarily 2012) and in some cases to the use of androgen- stages are detailed in Table 1 and Figures 2–4. affects intertriginous sites, most commonly containing contraceptives (Stellon and Wakeling, the genital, perianal, axillary, groin and sub- 1989). Hidradenitis suppurativa can have a Physician’s Global Assessment mammary areas (Jemec, 2012). Hidradenitis significant detrimental effect on quality of life The six-stage Physician’s Global Assessment is suppurativa most commonly develops in early related to chronic painful discharging abscesses. a useful tool for assessing response to treatment adult life, while diagnosis after the age of Depression, social isolation, impaired sexual and in clinical trials (Table 2). 50 years is relatively unusual (Jemec, 2012). relationships and unemployment are all associated There is a female:male ratio of 3:1 (Jemec, with the disease (Jemec, 2012; Zouboulis et Sartorius hidradenitis suppurativa score 2012). Jemec et al (1996) reported a prevalence al, 2015; Janse et al, 2017). Hospital resources The Sartorius hidradenitis suppurativa score of 1% in a Danish population-based study. are also impacted: annual inpatient costs per allows clinicians to take into account the Other studies, using varying methodologies, individual have been estimated at £2027 in a number of abscesses or nodules, sinus tracts have found prevalence rates ranging from retrospective cohort study of 11 359 patients and location of lesions. The distance between 0.053–4% (Jemec, 1988; Revuz et al, 2008; who required outpatient, emergency department discrete lesions and presence of normal skin Cosmatos et al, 2013). and inpatient treatment (Desai and Shah, 2017). is also included. Separate scores are given for Previously, the apocrine sweat glands were each region involved, number of nodules and thought to be central to the pathogenesis of Pathway for diagnosis, referral individual fistulae. hidradenitis suppurativa (Prens and Deckers, and treatment Figure 1. Scarring and draining of multiple fistulas connected 2015). However, research has now confirmed Lack of awareness about hidradenitis via sinus tracts on the breast of a person with severe the hair follicle to be key to the disease process. suppurativa among health-care providers Hidradenitis suppurativa forms part of the leads to delayed diagnosis, compounded by a hidradenitis suppurativa. ‘follicular occlusion tetrad’, along with severe historical perception that no helpful treatment nodulocystic acne (acne conglobata), dissecting options existed. Effective therapy is now cellulitis of the scalp and pilonidal sinus (Vazquez available and early recognition of this chronic et al, 2013). Follicular occlusion in hidradenitis and debilitating disorder is essential to prevent suppurativa results from excessive proliferation morbidity and poor quality of life for this of keratinocytes and resulting rupture of the patient group (National Institute of Health follicle leads to local inflammation with the and Care Excellence, 2015). release of pro-inflammatory cytokines (IL-1β, IL-17 and IL-18) (Kelly et al, 2015). Signs and symptoms Lifestyle, environmental and genetic factors Hidradenitis suppurativa presents as recurrent contribute to the pathogenesis of hidradenitis (more than two episodes in 6 months) painful, suppurativa. The link between hidradenitis subcutaneous inflammatory nodules, usually in suppurativa and obesity is well described flexural sites (Zouboulis et al, 2015). A higher ©AbbVie with a high prevalence of overweight/obesity prevalence of anterior lesions occur in women Table 1. Hurley system documented in several studies (Harrison et (sub-mammary, genito-femoral and axillae), al, 1988; Alikhan et al, 2009; Sartorius et al, whereas men are more likely to have gluteal or Stage Clinical features 2009). Obese patients are also at greater risk of atypical site involvement (back, ears and face) severe hidradenitis suppurativa (Sartorius et al, (Jemec, 2012; Canoui-Poitrine et al, 2013) I Single or multiple abscesses 2009), because of increased mechanical shear, (Figure 1). No sinus tracts or scarring occlusion of hair follicles and androgen excess Lesions may persist for several weeks or II Recurrent single or multiple widely separated (Alikhan et al, 2009). Smoking has also been months. Abscesses can form, which rupture abscesses confirmed as a significant risk factor in several with purulent discharge. As hidradenitis Sinus tracts and scar formation population studies (König et al, 1999; Revuz et suppurativa progresses, sinus tracts form as al, 2008; Sartorius et al, 2009). a consequence of follicular rupture, with III Diffuse involvement OR multiple A family history of hidradenitis suppurativa secondary bacterial infection and malodorous interconnecting abscesses and sinus tracts has been demonstrated in 30–40% of patients discharge. Significant scarring is very common From Hurley (1989) although the exact mechanism of inheritance in severe disease (Jemec, 2012). The production of this guide was supported by AbbVie who funded its publication and were involved in reviewing and approving the content. AXHUD170927 Date of preparation: September 2017 Figure 2. Hurley stage I, solitary axillary nodule. Figure 3. Hurley stage II, axillary scarring with associated Figure 4. Hurley stage III, axillary scarring and multiple fistulae. draining fistulae. ©AbbVie ©AbbVie ©AbbVie Classification of hidradenitis suppurativa Current guidelines and used in practice. Oral rifampicin and clindamycin into three latent classes (LC) based on management 300 mg twice daily each is recommended as first- phenotypic features has been proposed in a The British Association of Dermatologists line in moderate to severe Physician’s Global cross-sectional study of 618 patients (Canoui- guidelines for hidradenitis suppurativa are due Assessment or Hurley stage II and usually Poitrine et al, 2013). LC1 (48%) had axillary- for publication in late 2017. The only formal continued for 10 weeks (Gulliver et al, 2016). mammary hidradenitis suppurativa with guidance currently available is discussed below. Small studies have reported reduction in severity hypertrophic scarring. LC2 (26%) additionally of hidradenitis suppurativa with combination had a greater proportion of chest, ear, leg or European guidelines rifampicin and clindamycin therapy (Mendonça back lesions. Acne, epidermal cysts, pilonidal The European S1 Guideline for the treatment and Griffiths, 2006; van der Zee et al, 2009). sinus and comedones were more common. of hidradenitis suppurativa/acne inversa was A case series of 116 patients found a significant LC3 (26%) patients had gluteal involvement published in 2015 (Zouboulis et al, 2015). benefit (P<0.001) for severe hidradenitis with folliculitis. Both LC2 and LC3 had a Definitive guidance is difficult because of the suppurativa with clindamycin (300 mg twice significantly higher proportion of smokers and multiple presentations and grades of severity. daily) and rifampicin (600 mg daily) for 10 weeks longer duration of symptoms than LC1. LC2 The Hurley, Sartorius and Physician’s Global (Gener et al, 2009). patients were more likely to be male with a Assessment staging systems are recommended higher body mass index (Canoui-Poitrine et al, for clinicians. The impact on the patient should National Institute of Health and Care 2013). The usefulness of this classification in ideally be measured using the Dermatology Excellence guidance clinical practice is yet to be seen. Life Quality Index and visual analogue scale for The anti-tumour necrosis factor-α (TNF-α) pain. A stepwise approach, according to Hurley monoclonal antibody, adalimumab (Humira, Differential diagnosis stage, is recommended for management. AbbVie), is approved by the National Institute and systemic associations of Health and Care Excellence (2015) for Abscesses may be misdiagnosed as Adjuvant therapy and lifestyle advice moderate to severe hidradenitis suppurativa. staphylococcal skin infection (Zouboulis et al, All patients should be given general advice, Randomized, placebo controlled studies 2015) and treated with repeated short courses including wearing loose clothing, use of have found significant improvement in the of antibiotics. Multiple GP or emergency absorbent bandages and lifestyle measures validated Hidradenitis Suppurativa Clinical department attendances are common, leading to (smoking cessation, weight reduction). Response (Jemec et al, 2015; Kimball et al, negative investigation for immunosuppression. Weight loss decreases mechanical