Hidradenitis Suppurativa (Verneuil’S Disease)

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provided by Elsevier - Publisher Connector Journal of the Saudi Society of Dermatology & Dermatologic Surgery (2013) 17, 1–5

King Saud University Journal of the Saudi Society of Dermatology & Dermatologic Surgery www.ksu.edu.sa www.jssdds.org www.sciencedirect.com

REVIEW Hidradenitis suppurativa (Verneuil’s disease)

Besma Ben Dhaou *, Fatma Boussema, Zohra Aydi, Lilia Baili, Lilia Rokbani

Internal Medicine Department, Habib Thameur Hospital, Tunis, Tunisia Faculty of Medicine, University of Tunis ElManar; Faculty of medicine, Tunis, Tunisia

Received 24 May 2012; revised 15 June 2012; accepted 20 June 2012 Available online 21 July 2012

KEYWORDS Abstract Hidradenitis suppurativa, or Verneuil’s disease, is somewhat common, especially in women, Hidradenitis suppurativa; but is often unrecognized. Clinical manifestations include painful nodules, abscesses, sinus tracts, and Verneuil’s disease ropelike hypertrophic scars in the apocrine gland-bearing areas. It is a chronic disease. Severe dramatic forms are rare. Treatment is both medical and surgical: wide-spectrum antibiotics and excisions tai- lored to the extent of involvement. Quality of life is severely affected even in mild forms. ª 2012 King Saud University. Production and hosting by Elsevier B.V. All rights reserved.

Contents

1. Introduction ...... 2 2. Deﬁnition ...... 2 3. Prevalence ...... 2 4. Risk factors ...... 2 5. Diagnostic criteria ...... 2 6. Clinical features ...... 2 7. Topography ...... 3 8. Severity grading ...... 3 9. Quality of life ...... 3 10. Complications ...... 3 11. Associated diseases ...... 3 12. Treatment ...... 3 13. Conclusion ...... 4 References...... 4

* Corresponding author. Tel.: +216 24144955. E-mail address: [email protected] (B.B. Dhaou). Peer review under responsibility of Saudi Society of Dermatology and Dermatologic Surgery.

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1. Introduction open ‘tombstone’ comedos in secondary lesions; typical topography: axillae, groin, perineal and perianal regions, buttocks, infra- and intermammary folds; and; Hidradenitis suppurativa (HS) was first described in 1854 by a chronicity and recurrences. French surgeon, Aristide Verneuil (Verneuil, 1854). HS is diagnosed by its clinical features and its chronic nature; no lab- All three criteria must be met to establish the diagnosis. Our oratory test or pathology findings identify it. It is defined by patient validates the three criteria. the presence of recurrent, painful, deep-seated, rounded nodules that develop into abscesses and sinus tracts, with suppuration and hypertrophic scarring of apocrine gland-bearing skin. 6. Clinical features

2. Definition Onset usually occurs after puberty, usually during the second or third decade of life. There is frequently a long delay before diagnosis, particularly in mild cases: an abscess may be surgically In San Francisco in March 2009, the second international treated by incision, and the chronic or relapsing nature of the dis- symposium organized by the Hidradenitis Suppurativa Foun- ease not considered at all. Moreover, general practitioners, sur- dation adopted a consensus definition (Hidradenitis Suppura- geons, and even dermatologists frequently know very little about tiva Foundation, 2009): ‘‘HS is a chronic, inflammatory, this disease (Hidradenitis Sup-purativa Foundation, 2009). recurrent, debilitating, skin follicular disease that usually pre- The early lesions are solitary, painful nodules that may per- sents after puberty with painful deep-seated, inflamed lesions sist for weeks or months without any change or with occa- in the apocrine gland-bearing areas of the body, most com- sional episodes of inflammation. Early isolated lesions are monly, the axillary, inguinal, and anogenital regions’’. not specific and are frequently considered to be boils or common abscesses. The shape of these nodules, round rather than 3. Prevalence pointed and with no central necrosis, together with their topography should help to differentiate them from common Although HS is considered a rare disease, several studies re- boils. Their location deep in the hypodermis means they are port a global prevalence of 1% in the general population (Nal- sometimes barely visible. They are extremely painful. Approx- di, 2006). A large French survey confirmed this prevalence imately 50% of all patients experience subjective prodromal (Revuz et al., 2008). The peak prevalence recorded is 4%, in symptoms, which may include burning, stinging, pain, pruri- a population of young women in Denmark. It is likely due tus, heat, and hyperhidrosis, 12–48 h before the overt nodule to the higher prevalence in young adults and in women (sex ra- appears. The mean duration of a single painful nodule is 7– tio: 1/3.3) (Jemec, 1988), confirmed by clinical examination of 15 days. The nodules may remain blind, i.e., fail to burst, patients in another study (Jemec et al., 1996). and either resolve spontaneously or persist as silent nodules with inflammatory recurrences. Most frequently, however, 4. Risk factors they turn into abscesses and rupture externally, draining purulent material. Rupture into neighboring structures is rare. Naldi (2006) has reviewed the risk factors for HS. A case-con- The repetition of attacks may lead to chronic sinus forma- trol study of factors associated with HS in 302 patients (Revuz tion, with intermittent release of serous, purulent, or blood- et al., 2008) confirmed that smoking and overweight are the stained discharges and frequent foul odors from anaerobic two main factors associated with HS. Because no temporal colonization (Fig. 1). These persist for months, sometimes relationship or dose effect has been demonstrated between years. Ulceration or pyogenic granulomas sometimes occur. smoking and HS, this strong association cannot be considered A characteristic secondary lesion is composed of bridged, a true risk factor. ropelike hypertrophic scars resulting from a healing process Furthermore, no specific mechanism has been shown to ex- plain this association, and no study has documented the effect of smoking cessation. Nonetheless, the medical community generally accepts that smoking is the most important triggering factor of HS. Antiperspirant, talc, and deodorant use and depilation with a safety razor have been ruled out as pathogenic factors (Mor- gan and Leicester, 1982). Tight clothing may contribute to discomfort and is considered a triggering factor by some authors.

5. Diagnostic criteria

According to the criteria adopted by the international symposium of the Hidradenitis Suppurativa Foundation in March 2009 (Hidradenitis Suppurativa Foundation, 2009), diagnosis requires the presence of:

typical lesions: deep-seated painful nodules: ‘blind boils’ in early lesions; abscesses, draining sinuses, bridged scars, and Figure 1 Axillary masses ﬁstulated in the skin. Hidradenitis suppurativa (Verneuil’s disease) 3

9. Quality of life

Quality of life (QOL) is profoundly impaired even in mild cases. A study of 114 patients with HS using the Dermatology Life Quality Index (DLQI) recorded a very high score, particularly for question 1, which measures the level of pain, itching, and soreness (Hurley, 1989). A study of 61 HS patients using several questionnaires showed that the impairment of QOL in each ‘‘dimension’’ was signiﬁcantly higher (p < 0.001) than for patients with neuroﬁbromatosis, urticaria, psoriasis, and atopic dermatitis (Von der Werth and Jemec, 2001). This was particularly dramatic for self-perception, daily living activ- ities, mood state, social functioning and physical discomfort.

10. Complications

Figure 2 Axillary sequelae of fistulas. Acute infectious complications such as cellulitis or systemic infection are very unusual. Lymphatic obstruction and lymph- involving dense fibrosis (Fig. 2). Regional lymphadenopathy edema (Wolkenstein et al., 2007) as well as scrotal elephantia- does not appear, surprisingly in view of the inflammatory sis may complicate longstanding inflammation. In men, and infectious features observed (Poli et al., 2006). Pathology squamous cell carcinoma is observed in buttock areas after specimens are usually not taken from patients with HS, as his- several decades of HS. It often starts in deep locations, so diag- topathology is not helpful for diagnostic and/or therapeutic nosis is delayed and the prognosis usually very poor (Faye purposes. et al., 2007).

7. Topography 11. Associated diseases

In decreasing order of frequency, the affected sites are the Association of HS with severe nodular acne (acne conglobata), (Jemec, 1988; Hidradenitis Suppurativa Foundation, 2009; dissecting cellulitis of the scalp, and pilonidal cysts has been re- Wortsman et al., 2009; von der Werth and Williams, 2000): ported. Pilonidal cysts are reported especially frequently: 30% groin, including inner thighs; pubic region; scrotum or vulva; if both true sinuses and midline intergluteal dimples are con- perianal and perineal regions; armpits; inter- and submam- sidered (von der Werth and Williams, 2000). mary folds, and buttocks. These locations are along the milk Reticulate pigmentation of the flexures is sometimes associ- lines of apocrine and mammary tissues, which have the same ated with HS. It is difficult to say if this is a significant associ- embryonic origin. The groin and submammary regions are ation, as usually people do not seek medical advice for these most commonly affected in women, the buttocks and perianal pigment changes, which are discovered during examination skin in men; the anal canal is usually spared. Several sites, fre- of the groin and armpits of HS patients (Maalouf et al., 2006). quently symmetric, may be affected simultaneously; recur- Crohn’s disease (Canoui-Poitrine et al., 2009) is both a dif- rences occur in and around the original site. ferential diagnosis and an associated disease. Cutaneous Cro- hn’s disease must be differentiated from HS in its perianal 8. Severity grading location: the clinician must always be aware that the perianal lesions of Crohn’s disease may mimic HS and should perform Severity is generally assessed by the Hurley classification, the necessary workups. The atypical condyloma-like lesions which was designed for a surgical approach (Canoui-Poitrine seen in Crohn’s disease are not observed in HS; conversely, et al., 2009)(Table 1). specific Crohn’s lesions are unusual outside the perianal re- Although most practitioners know only the most dramatic gion. It is nonetheless impossible to differentiate them on clin- picture of severe disease, the mild forms are more frequent ical grounds only. The presence of epithelioid granulomas in (von der Werth and Williams, 2000). HS is polymorphous; the dermis, away from the site of active inflammation, is sug- its severity and course vary widely between individuals. gestive, but biopsies are not frequently performed in patients with HS. When doubt persists, a colonoscopy must be performed, even in the absence of overt digestive symptoms. True Table 1 Hurley’s classification. associations do exist, including cases of axillary HS. Several case reports and a few series of this association have been re- Grade I Abscess formation, single or multiple without sinus tracts and cicatrization ported (Bedlow and Morti-mer, 1996). Grade II Recurrent abscess with tract formation and cicatrization. Single or multiple, widely separated 12. Treatment lesions Grade III Diffuse or near-diffuse involvement, or multiple Hurley defined the 3 standard clinical stages, as we saw above interconnected tracts and Abscesses across entire area (Canoui-Poitrine et al., 2009)(Table 1). This classification is useful at least as a guide to choose between medical or limited 4 B.B. Dhaou et al. surgical treatment (stage I). Treatment depends on the stage be useful at very high doses (100 mg/day) in a very limited (Hurley’s classification), frequency of exacerbation, and on number of patients. the patient’s goal. A permanent cure can be obtained by wide Finasteride has been used in about ten female patients, with surgical excision, but this procedure should be considered only good results reported (Hessel et al., 2009; Mortimer et al., in the case of advanced disease. Early disease, on the other 1986). Dapsone has been used with good results (Joseph hand, may benefit from medical or medical and surgical ap- et al., 2005). It must be used cautiously in view of its very seri- proaches, simultaneously or successively (Seksik et al., 2006). ous side effects. Some patients suffer from recurrent painful nodules but Only transient efficacy was observed with infliximab in a also experience periods of remission. They may benefit from series of seven patients, where only two patients had sustained various options. Topical treatments, including antiseptics results and three had severe side effects (Farrell et al., 1999). A and antibiotics, are ineffective due to the depth of the lesions. case report of lethal squamous cell carcinoma after treatment A short course of systemic antibiotics may be tried for an with infliximab has been published (Faye et al., 2007). A pro- acute, painful nodule, to shorten the duration of the episode spective open study of 15 patients treated with etanercept and to abort it, avoiding development into an abscess. Various 50 mg weekly found only three responders (Kaur and Lewis, antibiotics have been used for this purpose. Amoxicil- 2006). A small randomized controlled study has shown some lin + clavulanic acid may be the most effective regimen, if it results despite the negative findings for the main outcome mea- is taken very early – within 1 h of the first signs or premonitory sure (Fardet et al., 2007). symptoms. A loading dose should be swallowed immediately Several series of patients have been treated with radiother- and followed by the same dose daily for the next few days. Int- apy at doses up to 8 gray. Considering the high spontaneous ralesional corticosteroids have been advocated. Rapid involu- cancer risk, especially for skin cancers in gluteal locations, this tion (12–24 h) of early lesions may be obtained (Church et al., potentially carcinogenic treatment should be considered with 1993). High doses of systemic steroids may be used to reduce caution (Lee et al., 2009). inflammation and pain. They can be used as an alternative Surgery (Grant et al., 2010) must be performed by an expe- to high-dose antibiotics or together with antibiotics (Revuz, rienced surgeon, aware of the difficulties and failures which 2008). may occur in HS. The proper identification of the entire lesion For an abscess, medical treatment to abort it is ineffective; is important as remnant tissue may facilitate recurrences. the required surgical drainage (incision) procedure should not High-frequency ultrasound examination of the skin or mag- be delayed. Incision of such an abscess is frequently performed netic resonance imaging or both may be helpful. The major ele- in the emergency department. Usually the diagnostic of HS is ment for a good and permanent result is the mapping of sinus missed. The incision is usually followed by packing; nursing in tracts and fistulas, usually done during the surgery. the days that follow and especially the renewal of packing are CO2 laser excision is used in mild to moderate disease with painful at the least and often a nightmare for the patient. A secondary healing. Superiority in comparison with standard limitation on the number of incisions and avoiding packing surgery is a matter for debate (Fro¨hlich et al., 2000; Lapins most of the time should improve patients’ quality of life. Pack- and Emtestam, 2006; Lapins et al., 1994; Lapins et al., 2002). ing is necessary in the case of a very deep cavity, concave area, narrow incision, or incomplete immediate evacuation. In those 13. Conclusion cases, it should not be changed too often and should be stopped as soon as it is dry (Sartorius et al., 2006). HS is an orphan disease, not because of its rarity – for it is fre- Various drugs have been used on a long-term basis. Their quent – but because physicians know too little about it. Treat- goal is to stop the progress of the disease, reduce the relapse ment is not curative, except for wide surgery, appropriate only rate, and avoid pain and chronic suppuration. Due to the for very severe cases; good management, using standard well- probable heterogeneity of the disease, some patients may ben- known treatments, is often more useful than the so-called efit from a drug that is useless for others. innovative treatments, whose risk/benefit ratios are unknown For severely affected patients with a high level of inflamma- today. tion, pain, and discharge, a 10-week regimen of clindamycin and rifampicin (600 mg/day each) Fearfield and Staughton, References 1999; O’Malley et al., 2009; Gener et al., 2009 is very useful. Patients at Hurley stage I or II may benefit from it. A complete Bedlow, A.J., Mortimer, P.S., 1996. Dowling-Degos disease associated remission, sometimes lasting as long as one year, is obtained in with hidradenitis suppurativa. Clin. Exp. Dermatol. 21, 305–306. some patients. In less severely affected patients or after the 10 Canoui-Poitrine, F., Revuz, J., Wolkenstein, P., Viallette, C., Gabison, weeks of clindamycin-rifampicin treatment, when inflamma- G., Pouget, F., et al., 2009. Clinical characteristics of a series of tion and pain are reduced, maintenance treatment with tetracy- 302 French patients suffering from hidradenitis suppurativa, with cline can prevent new attacks. Metronidazole may also be an analysis of factors associated with disease severity. J. Am. Acad. helpful, particularly in the case of foul odors. When surgery Dermatol. 61, 51–57. is indicated, a course of antibiotics beforehand may be useful Church, J.M., Fazio, V.W., Lavery, I.C., Oakley, J.R., Milsom, J.W., to prevent infections, better delineate and identify the lesions 1993. The differential diagnosis and comorbidity of hidradenitis to be excised, and effectively downsize the area to be excised. suppurativa and perianal Crohn’s disease. Int. J. 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