Key points

■ Hydradenitis suppurativa (HS) or Verneuil's disease is a chronic, recurrent inflammatory, painful disease of apocrine-gland-bearing skin

■ HS is an overlooked disease that affects a significant number of patients

■ HS develops in the second or third decade of life

■ Typical lesions are deep-seated nodules, sinus tract and hypertrophic fibrous scars

■ The two main areas involved are axillae and inguino-femoral zones

3.3 Topography 16

3.3.1 Involved Areas 16

3.3.1.1 Two Main Zones 17

3.3.1.2 The Two Less Frequently

Involved Zones 17

3.3.2 Atypical Localizations -

Other Zones May Be Involved 18

3.3.3 Distribution of Lesions 18

3.4 Evolution of the Disease and its Severity.. 18 3.4.1 Age at Onset and Resolution 18

3.5 Clinical Course 19

3.5.1 Intermittent/Benign Course of the Disease 20

3.5.2 Intermediate Course of the Disease 20

3.5.3 Continuous Disease: Moderate, Severe .. .20

3.6 Severity Indexes 21

3.6.1 Hurley's Clinical Staging [2] 21

3.7 Diagnosis 22

3.7.1 Diagnostic Criteria 23

3.7.2 Delay in Diagnosis 23

3.7.3 Differential Diagnoses 23

References 24

There is an extreme variety in the severity of the disease

3.1 Introduction

Contents

Quality of life is severely impaired Hidradenitis suppurativa (HS) is a distinctive chronic disease primarily located to inverse areas of the skin, e.g. axillae and groin. These areas generally may also be said to be apocrine-gland-bearing skin, although the apocrine glands are not primarily involved in the disease.

y j , ,, In the early stages the disease is an inflammato-

Introduction 11 ' °

ry and pustular follicular disease, but subse-

Individual Lesions 12 quently it becomes predominantly suppurative

„ ' T . and scarring. In addition to the objective clinical

Secondary Lesions 13 ° '

Tertiary Lesions 14 manifestations of the disease, the inflammatory

Comedones 15 changes and suppuration cause immediate pain,

Other Lesions 15 soreness and discomfort to the patients. In spite of the distinctness of HS it is commonly misdi-agnosed and frequently poorly managed, which adds to the burden of this disabling chronic disease, which severely impairs the quality of life.

Epidemiological and clinical observations may be of help, as corroborating evidence, in 3 establishing the diagnosis of HS. Generally, HS develops almost always after puberty, usually in the second or third decade of life. It is not an uncommon disease with a prevalence rate of 1% [8]. Women are more frequently affected than men; the sex ratio is 3:1 [1, 7, 8]. This has led to speculation about the aetiological role of endocrine and behavioural factors, although none of these have hitherto been found to be convincing on closer examination. Epidemiological studies however suggest that tobacco may play an aetio-logical or more likely a pathogenic role in the disease, as 84% of patients are current smokers [1]. Another frequently suspected aetiological/ pathogenic factor is obesity. Being overweight is not uncommon but not a unifying characteristic of the patients either: among the 164 patients of a personal series [1] 20% were overweight and 20% obese (body mass index, BMI, >30 kg/m2). The median BMI was, however, 23.6 kg/m2, which may be considered normal. Similarly, another earlier series found no significant deviation from ideal body weight in a series of 76 patients as a whole [6]. It is most likely that these factors play a role as pathogenic elements in the progression of the disease and in the severity of the disease rather than as actual aetiological factors.

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