Rapid Communication/Hot Topic Received: March 30, 2015 Accepted after revision: May 6, 2015 Published online: June 30, 2015

Dermatology DOI: 10.1159/000431175

Hidradenitis Suppurativa/Acne Inversa: Criteria for Diagnosis, Severity Assessment, Classification and Disease Evaluation Christos C. Zouboulis a, b Veronique del Marmol a, d Ulrich Mrowietz a, c Errol P. Prens a, e Thrasivoulos Tzellos a, f Gregor B.E. Jemec a, g a

European Hidradenitis Suppurativa Foundation e.V. and b Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, Dessau, and c Department of Dermatology, University Medical Center Schleswig-Holstein, Kiel, Germany; d Department of Dermatology, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; e Department of Dermatology, Erasmus University Medical Center Rotterdam, Rotterdam, The Netherlands; f Department of Dermatology, Faculty of Health Sciences, University Hospital of North Norway, Tromsø, Norway; g Department of Dermatology, Roskilde Hospital, Health Sciences Faculty, University of Copenhagen, Copenhagen, Denmark

Abstract Background: Hidradenitis suppurativa/acne inversa (HS) is a chronic, inflammatory, recurrent, debilitating disease, which inflicts a significant burden on patients and is associated with comorbid disorders, such as significantly reduced quality of life, depression, stigmatization, inactivity, working disability, impairment of sexual health and several cardiovascular risk factors. Aims/Methods: To implement an expert consensus on the diagnostic criteria, severity and classification assessment, and an assessment of anti-inflammatory treatment effectiveness based on current evidence. Results: This article provides criteria for diagnosis, severity assessment, classification and evaluation of HS patients. Conclusion: The provided criteria can be used as tools for

© 2015 S. Karger AG, Basel 1018–8665/15/0000–0000$39.50/0 E-Mail [email protected] www.karger.com/drm

the promotion of uniformity in HS evaluation and facilitation of early and timely identification and referral in the primary care setting and thorough and efficient evaluation in daily clinical practice. © 2015 S. Karger AG, Basel

Nature of the Disease

Hidradenitis suppurativa/acne inversa (HS) is a multifactorial, chronic, inflammatory, recurrent skin disease, which can lead to severe scarring and disability and which can cause a significant, but greatly underestimated, burden on patients [1, 2]. The 1st International Research Symposium on HS, which took place in Dessau from March 30 to April 2, 2006, has formulated the following definition of the disease: ‘HS is a chronic, inflammatory, recurrent, debilitating skin disease (of the terminal hair follicle), that usually presents after puberty with painful, Prof. Dr. med. Prof. h.c. Dr. h.c. Christos C. Zouboulis Departments of Dermatology, Venereology, Allergology and Immunology Dessau Medical Center Auenweg 38, DE–06847 Dessau (Germany) E-Mail christos.zouboulis @ klinikum-dessau.de

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Key Words Acne inversa · Classification · Clinical signs · Diagnostic criteria · Hidradenitis suppurativa · Severity assessment

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deep-seated, inflamed lesions in the apocrine gland-bearing areas of the body, most commonly the axillary, inguinal and anogenital regions (Dessauer definition)’ [2, 3].

Epidemiology

Clinical Findings

HS presents a variable clinical course [15]. One of the main features of the disease is the intertriginous occurrence, although other areas of skin may also be affected [15–17]. The affected areas are in decreasing order of frequency: inguinal, axillary, perineal and perianal as well 2

Dermatology DOI: 10.1159/000431175

Fig. 1. Obligatory body locations of HS in both sexes.

as the submammary and/or intermammary fold in women, buttocks, mons pubis, scalp, area behind the ears and eyelids (fig.  1). The initial transient superficial lesions, namely follicular papules and pustules (folliculitis), are not diagnostic. Transient or persisting nodules, abscesses and finally scars are part of the diagnostic definition, and these can gradually coalesce to form plaques (fig. 2). 23–30% of HS patients are also affected by a pilonidal disease.

HS Diagnosis

For such a chronic, debilitating disease, efficient screening of the population in the primary care setting to detect possible HS cases early is essential, in order to facilitate early referral. This is reflected in the current long mean time to diagnosis of 7 years [1]. Since HS lacks a specific test, implementation of criteria to be used both in the primary care setting and by dermatologists, is of high significance. Recent evidence suggests that a positive reply to the following question could identify HS patients with a sensitivity of 90% and a specificity of 97%: ‘Have you had Zouboulis/del Marmol/Mrowietz/Prens/ Tzellos/Jemec

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The reported prevalence of HS in older studies ranged widely from 0.4 to 4% [4]. A recent study showed a prevalence of 1% in a representative sample of the French population (n = 10,000) [5]. In an earlier Danish study, based on prospective data, a point prevalence of up to 4.1% and a 1-year prevalence of 1% after re-examination of the patients (confidence interval 0.4–2.2%) were found [6]. Selfreported symptoms compatible with the diagnosis of HS occurred in 2.1% (confidence internal 1.88–2.32%) of a population sample of 16,404 persons in Denmark [7]. Women are more commonly affected; the male-female ratio is 1:2.7 to 1:3.3 [5, 6, 8]. An exception to this is the involvement of the perianal area, where men seem to predominate [9, 10]. In contrast to the European studies, the prevalence in the USA has been estimated based on health insurance data only. Using this approach, the prevalence appears to be significantly lower (0.05%) [11]. The average incidence of HS in the Rochester health insurance database is 6.0 patients per 100,000 population per year with an increasing trend (from 4.0 patients per 100,000 population in 1968 to 10.00 patients per 100,000 population in 2008; Rochester, Minn., USA) [12]. HS seems to develop rarely before puberty or after the menopause, although the persistence of existing lesions after the menopause is not unusual. It was reported that only 2% of cases occur before the age of 11 years [13]. However, evidence from a crosssectional study indicates that 7.7% of HS patients reported prepubertal early onset [14]. The average age at onset is 23 years [4]. Apart from the lack of a formal study to determine the prevalence of HS in a population, the reason for the different prevalence estimates is the lack of a commonly accepted definition of diagnostic criteria particularly in early/mild disease and standardized assessment of all body sites including the genital/perianal areas.

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a

b

c

d

Fig. 2. a Follicular papules/pustules (folliculitis). b Nodules (left, arrows, noninflammatory; right, inflammatory). c Abscesses. d Cysts in scar tissue (upper left), fistulae (upper right and lower left) and sinuses (lower right). e Double pseudocomedones (arrows) and scars.

outbreaks of boils during the last 6 months with a minimum of 2 boils in one of the following 5 locations: axilla, groin, genitals, under the breasts and other locations (not specified), e.g. perianal, neck and abdomen?’ [7]. The term ‘boil’ is used since HS patients usually refer to their lesions with it [7]. Since such a definition is very easy to

use in the primary care setting and has shown adequate specificity and sensitivity, it is recommended to be used in such a setting for an early identification of patients. Diagnostic criteria both for the primary care setting and for dermatologists [17] are presented in table 1.

Diagnosis and Assessment of Hidradenitis Suppurativa/Acne Inversa

Dermatology DOI: 10.1159/000431175

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e

Table 1. Diagnostic criteria proposed to be used in the primary care setting and by dermatologists

Primary care setting Outbreaks of boils during the last 6 months with a minimum of 2 boils with 5 different location options [axilla, groin, genitals, under the breasts and other locations (not specified), e.g. perianal, neck and abdomen] Dermatology clinics setting Obligatory criteria – History: recurrent painful or purulent lesions more than twice/6 months – Location: groin, armpit, perineum, buttocks area and submammary/intramammary fold (women) – Clinical signs: Primary lesions like follicular papule/pustule (folliculitis), nodule (inflammatory or noninflammatory), abscess Secondary lesions like cyst, fistula/sinus (exudative or nonexudative), double pseudocomedone, scar (atrophic, net-like, erythematous, hypertrophic, linear or bridged) Additional criteria (not obligatory) – History: HS-positive family history – Microbiology: no evidence of pathogens or presence of normal skin microflora at the predominant primary type of lesions Diagnosis of HS if – All three obligatory criteria are present – One or more obligatory locations are involved – One or more types of obligatory lesions are present (i.e. nodule, abscess, fistula/sinus, scar) Diagnosis of HS requires examining by the naked eye all body areas in which the disease can occur; for this the patients should not be wearing clothes. It is not enough to just ask the patients about skin lesions in these areas.

Table 2. Main differential diagnosis of HS

– Staphylococcal infection (disseminated pustular lesions) – Cutaneous Crohn’s disease (association with intestinal Crohn’s disease) – Abscesses (usually single lesions) – Tumors, primary or metastatic – Lymphogranuloma venereum – Rare diseases: Cutaneous actinomycosis (associated with sinus – intestinal tract disease) Tuberculosis cutis colliquativa

Differential Diagnosis

ence of further signs of inflammation, including temperature increase, a soft tissue infection must be considered and excluded.

Diagnostic Imaging

High resolution skin ultrasonography is appropriate in order to evaluate abscess borders and the formation of fistulae, arising from the deep part of the follicle [19, 20]. Magnetic resonance imaging represents an alternative for the detection of fistulae, especially those also involving other organs (dermointestinal fistulae and genitoanal involvement). The extent of the inflammation can be seen by a thermography of the skin [16].

The major differential diagnoses of HS [17] are shown in table 2. Classification and Severity Assessment

No laboratory markers exist. HS patients with active inflammation may have an increase in the blood cell sedimentation rate or C-reactive protein [18]. In the pres4

Dermatology DOI: 10.1159/000431175

Hurley stage and HS Physician’s Global Assessment are measures that have been used to classify and assess HS disease severity. Hurley staging (fig. 3; table 3) classifies patients into 3 stages and is proposed as a tool to facilitate rational treatZouboulis/del Marmol/Mrowietz/Prens/ Tzellos/Jemec

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Laboratory Markers

Hurley stage

Definition

I

Individual primary lesions and/or cysts without fistulae or scarring

II

Individual primary lesions and/or cysts with presence of fistulae and scarring

III

Confluent primary and secondary lesions at involved surface(s) with fistulae and scars

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Table 3. Hurley staging

Table 4. Hidradenitis Suppurativa - Physician’s Global Assessment

(HS-PGA) a

Clear (score = 0)

No abscesses, no draining fistulae, no inflammatory nodules and no noninflammatory nodules

Minimal (score = 1)

No abscesses, no draining fistulae, no inflammatory nodules but presence of noninflammatory nodules

Mild (score = 2)

No abscesses, no draining fistulae and 1 – 4 inflammatory nodules, or 1 abscess or draining fistula and no inflammatory nodules

Moderate (score = 3)

No abscesses, no draining fistulae and ≥5 inflammatory nodules, or 1 abscess or draining fistula and ≥1 inflammatory nodule, or 2 – 5 abscesses or draining fistulae and 5 abscesses or draining fistulae

b

ment decision-making for surgical approach in a certain body location. It is not useful for the follow-up of patients receiving medical treatment [21]. The HS Physician’s Global Assessment includes 6 stages with clear guidance for disease severity assessment and is appropriate for a dynamic follow-up of patients (table 4) [22].

HS Clinical Response was recently developed and validated for the assessment of anti-inflammatory treatment effectiveness (table 5) [23]. It requires counting inflammatory nodules, abscesses and draining fistulae at baseline and after the intervention. Achievement is identified as (1) at least 50% reduction in the number of abscesses Diagnosis and Assessment of Hidradenitis Suppurativa/Acne Inversa

c

Fig. 3. Clinical severity grades of HS in the axillae. Left: low grade of clinical inflammation; right: inflammation and exudation. a Hurley stage I. b Hurley stage II. c Hurley stage III.

Dermatology DOI: 10.1159/000431175

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Assessment of Anti-Inflammatory Treatment Effectiveness

Type of lesions – Abscesses (fluctuating, draining or not, erythematous, soft or painful to touch/spontaneously painful, round lesion >2 cm) – Inflammatory nodules (solid, erythematous, firm, pyogenic granuloma-like, round lesion

Acne Inversa: Criteria for Diagnosis, Severity Assessment, Classification and Disease Evaluation.

Hidradenitis suppurativa/acne inversa (HS) is a chronic, inflammatory, recurrent, debilitating disease, which inflicts a significant burden on patient...
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