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hidradenitis suppurativa

Monday 28 June 2010

Verneuil disease, acne inversa

Definition: Hidradenitis suppurativa is a chronic inflammatory process involving sites replete with apocrine glands.

Images

- Hidradenitis suppurativa. Sinus tract opens to the surface, inflamed granulation tissue, mixed inflammation with abscess formation, scarring.

Acne inversa (hidradenitis suppurativa) is a chronic, relapsing, inflammatory disorder involving the terminal hairs of one or more apocrine gland-bearing areas, which include the axillae, groins, pubic region, and perineum.

Follicular and intraepidermal apocrine duct occlusion by keratin with perifolliculitis and apocrine adenitis leads to the formation of abscesses.

Chronic inflammatory cells, foreign-body giant cells, granulation tissue, fibrosis, and deep sinus tracts follow destruction of cutaneous appendages.

Cultures usually reveal a spectrum of bacterial pathogens.

Surgery is the main stay of therapy for severe cases.

There are recurrent, deep-seated inflammatory nodules, complicated by draining sinuses and subsequent scarring. The disease causes a high degree of morbidity; pain is often a problem.

Other clinical features include the presence of comedones in retroauricular and apocrine sites, a female predominance, and a genetic predisposition.

Epidemiology

The prevalence estimate is 1 : 300.

Prepubertal onset is rare.

The disease is virtually never seen prior to puberty and has a peak incidence in the third decade of life.

Localization

Hidradenitis suppurativa is a skin disease that most commonly affects areas bearing apocrine sweat glands or sebaceous glands, such as the underarms, breasts, inner thighs, groin and buttocks.

Associations

Associations with lithium therapy, Dowling–Degos disease, cigarette smoking, obesity, and Crohn’s disease have been reported.

Etiology

Recently one Chinese family with the disease was mapped to chromosome 1p21.1–1q25.3. Interestingly, frequent losses in chromosomal region 1p21–p22 have been associated with diffuse malignant peritoneal mesothelioma, a rare neoplasm reported recently in association with acne inversa.

Host defense mechanisms are usually normal, except in some severe cases, where a reduction in T lymphocytes, and the presence of dysfunctional neutrophils have been documented.

Monocytes secrete less TNF-α and IL-6 than do cells from healthy controls, but toll-like receptor 2 is strongly expressed.

There is now good evidence that acne inversa is an androgen-dependent disorder, although how this relates to the poral occlusion by keratinous material is uncertain.

This occlusion is followed by an active folliculitis with, in some cases, a secondary apocrinitis and apocrine destruction.

Apoeccrine glands, which drain directly onto the epidermal surface, appear to be uninvolved.

Coagulase-negative staphylococci are the most common isolate; however, the microbiological flora is not constant.

It is possible that an abnormal immune response against bacteria, colonizing the follicular infundibulum, may be one of the initial events leading to acne inversa.

Synopsis

- acute and chronic inflammation in the dermis
- epithelial downgrowth (probably of follicular origin) ‘draining’ the area
- hemorrhage at the deep edge
- inflammation of the apocrine glands

Microscopy

In established lesions, there is a heavy, mixed inflammatory cell infiltrate in the lower half of the dermis, usually with extension into the subcutis.

Chronic abscesses are present in active cases and these may connect with sinus tracts leading to the skin surface.

The sinuses are usually lined by stratified squamous epithelium in their outer part. They contain inflammatory and other debris. Some of these tracts are probably residual follicular structures.

Granulation tissue containing inflammatory cells and occasional foreign body giant cells is present in up to 25% of cases.

Epithelioid granulomas were present in one case with coexisting Crohn’s disease.

Extensive fibrosis with destruction of pilosebaceous follicles and of apocrine and eccrine glands usually ensues.

Inflammation of the apocrine glands may be present in the axillary region in about 20% of cases.

Perieccrine inflammation is seen in approximately one-third of cases, from all sites.

In early lesions, there is folliculitis and perifolliculitis involving the lower part of the follicle.

The infundibulum is usually dilated and contains keratinous material and inflammatory debris.

These findings support the notion that acne inversa is primarily a follicular disease, but apocrine glands can be primarily involved in a minority of axillary lesions.

Squamous cell carcinoma complicating hidradenitis suppurativa

The development of squamous cell carcinoma is a late and rare complication. The tumors arise from the sinus tracts.

- SCC complicating HS evolves poorly, despite a good histological prognosis.
- Some results sustain the implication of HPV in the malignant transformation of HS.

Treatment

The treatment of acne inversa (hidradenitis suppurativa) is often ineffective.

Initial management is often used in conjunction with other treatment modalities. Initial measures include cessation of smoking, weight loss, and avoidance of irritation in affected areas.

Systemic and/or topical antibiotics are usually used. Topical clindamycin results in significant improvement; it may be given orally, in combination with rifampicin.

Dapsone and corticosteroids have helped some patients, but relapse occurs on the tapering of any steroids. The anti-androgen finasteride has been effective in some patients.

The biologics such as infliximab, efalizumab, and etanercept have helped some patients but their potential side effects need to be considered. Carbon dioxide laser has also been used.

Wide surgical excision, with healing by secondary intention, is the definitive treatment when scarring is present: it has the potential to cure.

The recurrence rate in one report was 42.8% after limited excision, but still 27% after radical excision.

References

- Cutaneous PGP 9.5 distribution patterns in hidradenitis suppurativa. Sartorius K, Emtestam L, Lapins J, Johansson O. Arch Dermatol Res. 2010 Aug;302(6):461-8. PMID: 20091410

- Clinicopathological study of 13 cases of squamous cell carcinoma complicating hidradenitis suppurativa. Lavogiez C, Delaporte E, Darras-Vercambre S, Martin De Lassalle E, Castillo C, Mirabel X, Laurent F, Patenotre P, Gheit T, Talmant JC, Beylot-Barry M, Martinot V, Piette F, Aubin F, Mortier L. Dermatology. 2010;220(2):147-53. PMID: 20029163

- Toll-like receptor 2 is highly expressed in lesions of acne inversa and colocalizes with C-type lectin receptor. Hunger RE, Surovy AM, Hassan AS, Braathen LR, Yawalkar N. Br J Dermatol. 2008 Apr;158(4):691-7. PMID: 18241264

- Lack of association between CARD15 gene polymorphisms and hidradenitis suppurativa: a pilot study. Nassar D, Hugot JP, Wolkenstein P, Revuz J. Dermatology. 2007;215(4):359. PMID: 17911997

- Inversa acne (hidradenitis suppurativa): a case report and identification of the locus at chromosome 1p21.1-1q25.3. Gao M, Wang PG, Cui Y, Yang S, Zhang YH, Lin D, Zhang KY, Liang YH, Sun LD, Yan KL, Xiao FL, Huang W, Zhang XJ. J Invest Dermatol. 2006 Jun;126(6):1302-6. PMID: 16543891

- The clinical genetics of hidradenitis suppurativa revisited. Von Der Werth JM, Williams HC, Raeburn JA. Br J Dermatol. 2000 May;142(5):947-53.PMID: 10809853