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Hidradenitis Suppurativa

Suppurative Hidradenitis

Hidradenitis suppurativa is a chronic skin disease of unknown etiology.


Presentation

Hidradenitis suppurativa is generally seen in the areas having plenty of hair follicles and has many sweat glands (apocrine glands) like underarms, groin and anal area. It is also seen in the regions where there is friction between the skin like between the buttocks and beneath the breast.

The signs and symptoms of hidradenitis suppurativa are as follows. The disease has an insidious onset, where the first sign is erythema; later it becomes painful. Blackheads are commonly seen appearing in pairs also known as a ‘double-barreled pattern’. Red, painful bumps appear which are big in size and leak pus. The pus is foul smelling. Along with these bumps there is severe itching, burning and sweating. Painful, hard, pea-sized lumps form below the skin and may remain for years together. They may enlarge and become inflamed. The leaking lumps heal very slowly and if they heal they lead to scarring and form sinus below the skin. This draining represents persistent nodular hidradenitis suppurativa represented by intermittent discharge of pus and blood.

If left untreated, then over time many abscesses and sinus tracts develop a subcutaneous honeycomb. Ultimately fibrosis, scarring and induration develop. This condition usually begins after puberty presenting as a single painful lump for weeks or months.

Axillary Lesion
  • The clinical course varies from occasional axillary lesions to diffuse abscess formations in multiple sites leading to chronic draining sinuses, as well as indurated, scarred skin and subcutaneous tissues.[aafp.org]
  • Excision and primary closure of the axillary lesions were done in second and third stages. We obtained excellent functional results (convenience during urination etc.) and moderate cosmetic results. Figure 2 Patient 10.[medsci.org]
  • No sex difference is seen in the axillary lesions.[emedicine.com]
Hunting
  • Michael Li, Michelle J Hunt and Christopher A Commens, Hidradenitis suppurativa, Dowling Degos disease and perianal squamous cell carcinoma, Australasian Journal of Dermatology, 38, 4, (209-211), (2007). A.M. Farrel and R.P.R.[doi.org]
Oral Ulcers
  • Setterfield, Oral ulcers as a presentation of secondary syphilis, Clinical and Experimental Dermatology, 43, 8, (868-875), (2018). K. Sardana, G. Verma and R. K.[doi.org]
Skin Lesion
  • RESULTS: The diet demonstrated immediate stabilization of their clinical symptoms, and the skin lesions regressed over the 12-month treatment period.[ncbi.nlm.nih.gov]
  • In this report, one case of HS/AI achieved resolution of skin lesions, ulcer healing and disappearance of symptoms after nine treatments with 5-aminolevulinic acid photodynamic therapy (ALA-PDT).[ncbi.nlm.nih.gov]
  • The scan showed uptake in the left vocal cord malignancy and multiple hypermetabolic subcutaneous foci in the right axilla, right buttocks, and scalp in known locations of skin lesions related to hidradenitis suppurativa.[ncbi.nlm.nih.gov]
  • METHODS: A microarray data set including 30 samples was used to compare the expression of sphingolipid-related enzymes in inflammatory skin lesions from HS patients (n 17) with the expression in clinically healthy skin tissue (n 13).[ncbi.nlm.nih.gov]
  • […] areas), or in the perineum (area around the genitals and anus), AND Your skin lesions must last a minimum of three months even while undergoing continuous treatments as prescribed by your physician.[disability-benefits-help.org]
Acne Vulgaris
  • At center stage is not a suppurative inflammation of the apocrine sweat glands but an occlusion of the hair follicles, comparable to acne vulgaris.[ncbi.nlm.nih.gov]
  • Keri, MD, PhD, Associate Professor of Dermatology and Cutaneous Surgery, University of Miami, Miller School of Medicine; Chief, Dermatology Service, Miami VA Hospital Click here for Patient Education Acne and Related Disorders Acne Vulgaris Hidradenitis[merckmanuals.com]
  • Abstract The ultimate, feral expression of acne vulgaris is acne conglobata. It is a searing, scarring, spectactular disorder which is utterly devastating.[doi.org]
  • Citing Literature Number of times cited according to CrossRef: 43 Sara Wertenteil, Andrew Strunk and Amit Garg, Overall and subgroup prevalence of acne vulgaris among patients with hidradenitis suppurativa, Journal of the American Academy of Dermatology[doi.org]
Hyperhidrosis
  • Lawrence, Axillary hyperhidrosis: eccrine or apocrine?, Clinical and Experimental Dermatology, 28, 1, (2-7), (2003).[doi.org]
  • The distribution, size and density of the apocrine glands in hidradenitis suppuritiva as compared with those in axillary hyperhidrosis and normal controls have been studied.[ncbi.nlm.nih.gov]
  • Carbuncles Poor Hygiene …is definitely not: contagious Herpes STD Cancer Allergies Plague …often happens in conjunction with: PCOS / Insulin Resistance / Androgen Dysfunction Crohns and other inflammatory auto-immune conditions Anemia Hyperhydrosis / hyperhidrosis[hs-usa.org]
  • Early symptoms of HS include pruritus, erythema, and local hyperhidrosis.[journals.lww.com]
Furunculosis
  • […] tracts between infected hair follicles • Staphylococci and anaerobic diphtheroids are most common organisms • Hidradenitis suppurativa is a serious skin infection of the axillae or groin consisting of multiple abscesses of the apocrine sweat glands • Furunculosis[accesssurgery.mhmedical.com]
  • Codes ICD10CM: L73.2 – Hidradenitis suppurativa SNOMEDCT: 59393003 – Hidradenitis suppurativa Look For Subscription Required Diagnostic Pearls Subscription Required Differential Diagnosis & Pitfalls Furunculosis Folliculitis Epidermoid cysts Abscesses[visualdx.com]
  • Early lesions of HS mimic other skin conditions and thus are often misdiagnosed as recurrent furunculosis or boils. The delay in HS diagnosis can be 12 years or longer.[cfp.ca]
  • Furunculosis. Actinomyces spp. Investigations Diagnosis is clinical but investigations may include: FBC: underlying anaemia associated with chronic disease. Blood glucose: identify associated diabetes. Microbiology swabs (usually negative).[patient.info]
Pruritus
  • Abstract A growing body of research has indicated that pruritus is an important feature of hidradenitis suppurativa (HS). This study evaluated pruritus and pain among 103 patients with HS.[ncbi.nlm.nih.gov]
  • Early symptoms of HS include pruritus, erythema, and local hyperhidrosis.[journals.lww.com]
  • Hidradenitis suppurativa typically presents with inflammatory nodules, abscesses, comedones, sinus tracts, or scarring. 1, 3 It has an insidious onset, starting with mild discomfort, erythema, burning, pruritus, and hyperhidrosis.[cfp.ca]
Genital Lesions
  • Alberto Rosenblatt, Homero Gustavo de Campos Guidi and Walter Belda, Nonsexually Transmitted Infections, Male Genital Lesions, 10.1007/978-3-642-29017-6_9, (167-211), (2012). Raed Alhusayen and Neil H.[doi.org]
Meningism
  • Local and systemic infections ( meningitis, bronchitis, pneumonia, etc.), are seen, which may even progress to sepsis.[en.wikipedia.org]
  • A case of meningitis emerged as a complication of hidradenitis suppurativa. Mikrobiyol Bul. 2009 Jan;43(1):153-7 [ PubMed ] 113. Chow ET, Mortimer PS. Successful treatment of hidradenitis suppurativa and retroauricular acne with etretinate.[web.archive.org]

Workup

Diagnosis is mainly done on the basis of clinical presentation but the following clinical tests may help in evaluation of the disease:

  • Complete blood count: Helps in knowing any underlying disease like anemia, the erythrocyte sedimentation rate as well as the white blood cells count may be elevated in case of acute lesions. 
  • Blood glucose levels should be done to find if the patient has underlying diabetes which tends to worsen the condition.
  • Bacteriological analysis of the discharge should be done to identify the microorganisms in the lesions.
  • Ultrasonography of the hair follicles can be done to find any abnormalities in the deeper parts of the follicle.
  • CT scan or MRI can be done prior to the surgery to find out the exact extent of the disease.
Trichophyton Rubrum
  • Li, Ultrastructural changes of Trichophyton rubrum in tinea unguium after itraconazole therapy in vivo observed using scanning electron microscopy, Clinical and Experimental Dermatology, 43, 8, (883-889), (2018). W. Zhong, J. Liu, H. Wang, X.[doi.org]

Treatment

Treatment depends on the presentation and extent of the disease. Following are the various approaches for the treatment of this condition:

Lifestyle

  • Complete cessation of smoking.
  • Reducing weight [22] and changes in food habits are essential to achieve the same. Wear loose clothes. Having hot baths with the mixture of distilled white vinegar and water helps [23].

Medicines

  • Antibiotics: They help in controlling the disease and reducing the risk of future outbreaks. Antibiotics can be given orally or can be used in the form of ointments or creams for local application. Commonly used antibiotics are erythromycin, clindamycin, tetracycline, minocycline and metronidazole.
  • Corticosteroids: Intralesional steroid injections can help in early resolution of the lesion. Orally high doses help in reducing inflammation.
  • Tumor necrosis factor (TNF) alpha- inhibitors: Drugs like infliximab, adalimumab have shown promising effects in treatment of this condition but they increase the risk of infection, heart failure and few types of cancer.
  • Anti-androgen therapy: Hormonal therapy showed beneficial effects with the use of cyproterone acetate and ethinyl estradiol, but the dosages are very high [24].
  • Topical keratolytic agents like resorcinol has shown promising results in many cases due to its effect on follicular keratin plug [25].
  • Zinc supplements also help in reducing inflammation and preventing new outbreaks.

Radiation

  • Using x-rays in decreasing doses for temporary removal of hair from the root helps in treating this condition.
  • Laser hair removal: Use of laser beams for permanent hair removal might aid in the treatment of hidradenitis suppurativa for long term.

Surgical

Surgical intervention becomes necessary in cases of chronic hidradenitis suppurativa. Nonsurgical intervention only supports the treatment but is important many a times before or after the surgery [22] [26] [27] [28]. Early diagnosis of the disease reduces the extent of surgery as well as prevents complications [27].

  • Incision and drainage: When a single small area is involved, surgical drainage of that area should be considered, but this treatment gives only short term relief. 
  • Exposing the sinus tracts: This process is also known as de-roofing; the skin and flesh are removed to uncover the underlying interconnected sinuses that link the separate lesions. The disease has chances of returning in the same area or another area in the body.
  • Surgical removal: This involves wide surgical excision, with margins beyond the clinical borders of activity. Skin grafting may be needed for closure of the wound. 
  • Surgical excision with the help of a carbon-dioxide laser and second-intention healing shows good results and has lesser complications. 
  • Electrosurgery is the best alternative in treatment of hidradenitis suppurativa [29].

Recurrence after the surgery can occur if the excision was not done properly or if the apocrine glands are widespread [8] [27] [30].

Prognosis

Early extensive extraction has good results as it is followed by low recurrence rate [19]. If the disease is not diagnosed in time, and remains untreated, then it can lead to the development of squamous cell carcinoma in the anal and any other affected areas [20] [21].

Etiology

The exact cause of hidradenitis suppurativa is unknown. Sometimes it is linked with autoimmune conditions [2]. As the disease is caused due to blockage and inflammation of hair follicles, there are various factors predisposing this condition like obesity [3], genetic factors [4], hormones [5], drugs and smoking.

Epidemiology

Hidradenitis suppurativa affects 1% of the general population [6] [7]. Increased incidence is seen blacks as they have more number of apocrine glands as compared to whites [8]. It is considered to have predilection for females than in males, with ratio being 2-5:1 [9] [10] but this is controversial [9] [11] [12] as the comedones which are considered to be the precursor lesions for hidradenitis suppurativa are scattered equally in both the sexes and at all places [13].

The age of onset is between 11-50 years, [14] with an average patient age of 23 years [9]. But rarely it can occur before 11 years of age, [15] before puberty [16] and after menopause [17]. The prevalence amidst people older than 55 is much lower compared to younger people [18].

Sex distribution
Age distribution

Pathophysiology

The exact pathogenesis of hidradenitis suppurativa is unclear. The anatomical distribution is suggestive of a disorder in apocrine glands. It begins with blocking of the apocrine or the follicular ducts due to keratinous plugging which in turn leads to ductal dilatation to balance the glandular component.

Infection begins via bacteria travelling through the hair follicle into the apocrine glands which are thereby trapped below the keratinous plug, and multiply rapidly in this
nutrient rich environment of the gland.

Ultimately the gland ruptures, causing inflammation that extends into the surrounding tissue and nearby glands. Bacterial infection with staphylococci and streptococci leads to added local
inflammation, tissue loss and skin damage.

Prevention

Hidradenitis suppurativa cannot be prevented, but depending on the cause the disease can be controlled and flare ups can be avoided. Losing weight certainly helps obese people, even 10% reduction in the body weight can make a difference.

Avoid shaving the areas where the disease is active. Quitting the habit of smoking, wearing loose fitting clothes so as to prevent friction, helps in avoiding the flare-ups. Overheating and sweating can also lead to flaring of this condition, so try and keep your skin cool. You can even use an anti-perspirant to reduce sweating. If the condition is due to a bacterial infection then use antiseptic soaps or bath additives.

Summary

Hidradenitis suppurativa is a chronic skin condition affecting the apocrine gland-bearing skin and areas of friction like the armpits, groin, buttocks, inner thighs and beneath the breasts [1]. It is presented as comedone-like follicular blockage, long-standing relapsing inflammation, discharge containing mucous as well as pus leading to scarring.

Patient Information

Hidradenitis suppurativa is not a contagious disease. It occurs mainly after puberty but can affect any age group. It affects the areas of the body that are rich with sweat glands, hair follicles and are prone to friction like the underarms, groin, anal region, inner areas of thighs, between the buttocks and beneath the breast. It causes foul smelling mucopurulent discharge and often heals with scarring.

It is a very stubborn disease and is also a challenge for the physician to cure it completely. Sexuality of the person can also be affected due to the areas that are affected by this condition. People might also face challenges in their social circle because of the body odor and inappropriate treatment can lead to depression, frustration and isolation. As it is difficult to cure this disease, it is advisable that the patients take care of all the factors like weight, smoking, hygiene, clothes, sweating etc. to keep this condition under control.

References

Article

  1. Alikhan A, Lynch PJ, Eisen DB. Hidradenitis suppurativa: a comprehensive review. J Am Acad Derm. 2009 Apr;60 (4): 539–561.
  2. Cusack C, Buckley C. Etanercept: effective in the management of hidradenitis suppurativa. Br J Dermatol. 2006 Apr;154 (4): 726–9.
  3. Slade DEM, Powell BW, Mortimer PS. Hidradenitis suppurativa: pathogenesis and management. Br J Plast Surg. 2003 Jul;56 (5): 451–61.
  4. Von Der Werth JM, Williams HC, Raeburn JA. The clinical genetics of hidradenitis suppurativa revisited. Br J Dermatol. 2000 May;142 (5): 947–53.
  5. Barth JH, Kealey T. Androgen metabolism by isolated human axillary apocrine glands in hidradenitis suppurativa. Br J Dermatol. 1991 Oct;125 (4): 304–8.
  6. Jemec GB, Heidenheim M, Nielsen NH. The prevalence of hidradenitis suppurativa and its potential precursor lesions. J Am Acad Dermatol. 1996 Aug;35(2 Pt 1):191-4.
  7. Jemec GB. What's new in hidradenitis suppurativa? J Eur Acad Dermatol Venereol. 2000 Sep;14(5):340-1.
  8. Parks RW, Parks TG. Pathogenesis, clinical features and management of hidradenitis suppurativa. Ann R Coll Surg Engl. 1997 Mar;79(2):83-9.
  9. von der Werth JM, Jemec GB. Morbidity in patients with hidradenitis suppurativa. Br J Dermatol. Apr 2001;144(4):809-13.
  10. Jemec GB, Heidenheim M, Nielsen NH. Hidradenitis suppurativa--characteristics and consequences. Clin Exp Dermatol. 1996 Nov;21(6):419-23.
  11. Manolitsas T, Biankin S, Jaworski R, Wain G. Vulval squamous cell carcinoma arising in chronic hidradenitis suppurativa. Gynecol Oncol. 1999 Nov;75(2):285-8.
  12. Fearfield LA, Staughton RC. Severe vulval apocrine acne successfully treated with prednisolone and isotretinoin. Clin Exp Dermatol. 1999 May;24(3):189-92.
  13. Jemec GB. Hidradenitis suppurativa. J Cutan Med Surg. 2003 Jan-Feb;7(1):47-56.
  14. Brown TJ, Rosen T, Orengo IF. Hidradenitis suppurativa. South Med J. 1998 Dec;91(12):1107-14.
  15. Palmer RA, Keefe M. Early-onset hidradenitis suppurativa. Clin Exp Dermatol. 2001 Sep;26(6):501-3.
  16. Mengesha YM, Holcombe TC, Hansen RC. Prepubertal hidradenitis suppurativa: two case reports and review of the literature. Pediatr Dermatol. 1999 Jul-Aug;16(4):292-6.
  17. Barth JH, Layton AM, Cunliffe WJ. Endocrine factors in pre- and postmenopausal women with hidradenitis suppurativa. Br J Dermatol. 1996 Jun;134(6):1057-9.
  18. Revuz JE, Canoui-Poitrine F, Wolkenstein P, et al. Prevalence and factors associated with hidradenitis suppurativa: results from two case-control studies. J Am Acad Dermatol. 2008 Oct;59(4):596-601.
  19. Alharbi Z, Kauczok J, Pallua N. A review of wide surgical excision of hidradenitis suppurativa. BMC Dermatol. 2012 Jun 26;12:9.
  20. Talmant JC, Bruant-Rodier C, Nunziata AC, Rodier JF, Wilk A. Squamous cell carcinoma arising in Verneuil's disease: two cases and literature review. Ann Chir Plast Esthet (in French) 2006 Feb; 51 (1): 82–6.
  21. Short KA, Kalu G, Mortimer PS, Higgins EM. Vulval squamous cell carcinoma arising in chronic hidradenitis suppurativa. Clin Exp Dermatol. 2005 Sep;30 (5): 481–3.
  22. Alikhan A, Lynch PJ, Eisen DB. Hidradenitis suppurativa: a comprehensive review. J Am Acad Dermatol. 2009 Apr;60(4):539-61; quiz 562-3.
  23. Martinez F, Nos P, Benlloch S, Ponce J. Hidradenitis suppurativa and Crohn's disease: response to treatment with infliximab. Inflamm Bowel Dis. 2001 Nov; 7 (4): 323–326.
  24. Mortimer PS, Dawber RP, Gales MA, Moore RA. A double blind controlled cross-over trial of cyproterone acetate in females with hidradenitis suppurativa. Br J Dermatol. 1986 Sep; 115 (3): 263–8.
  25. Boer J, Jemec GB. Resorcinol peels as a possible self-treatment of painful nodules in hidradenitis suppurativa. Clin Exp Dermatol. 2010 Jan; 35 (1): 36–40.
  26. Jansen T, Plewig G. What's new in acne inversa (alias hidradenitis suppurativa)? J Eur Acad Dermatol Venereol. 2000 Sep;14(5):342-3.
  27. Rompel R, Petres J. Long-term results of wide surgical excision in 106 patients with hidradenitis suppurativa. Dermatol Surg. 2000 Jul;26(7):638-43.
  28. Boer J, van Gemert MJ. Long-term results of isotretinoin in the treatment of 68 patients with hidradenitis suppurativa. J Am Acad Dermatol. 1999 Jan;40(1):73-6.
  29. Aksakal AB, Adisen E. Hidradenitis suppurativa: importance of early treatment; efficient treatment with electrosurgery. Dermatol Surg. 2008 Feb;34(2):228-31.
  30. Tanaka A, Hatoko M, Tada H, Kuwahara M, Mashiba K, Yurugi S. Experience with surgical treatment of hidradenitis suppurativa. Ann Plast Surg. 2001 Dec;47(6):636-42.

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Last updated: 2019-07-11 20:36