Hidradenitis suppurativa

From Wikipedia, the free encyclopedia - View original article

Hidradenitis suppurativa
Classification and external resources
ICD-10L73.2
ICD-9705.83
DiseasesDB5892
eMedicineemerg/259 med/2717 derm/892
MeSHD017497
 
Jump to: navigation, search
Hidradenitis suppurativa
Classification and external resources
ICD-10L73.2
ICD-9705.83
DiseasesDB5892
eMedicineemerg/259 med/2717 derm/892
MeSHD017497

Hidradenitis suppurativa (HS) is a skin disease that most commonly affects areas bearing apocrine sweat glands or sebaceous glands, such as the underarms, under the breasts, inner thighs, groin and buttocks.[1]

Contents

Overview

The non-contagious disease manifests as clusters of chronic abscesses, epidermoid cysts, sebaceous cysts, pilonidal cyst or multilocalised infections, which can be as large as baseballs or as small as a pea. It can also start as a single abscess and once it pops, can make tracts of many more abscesses. These cysts can be extremely painful to the touch and may persist for years with occasional to frequent periods of inflammation, culminating in incision and drainage of pus, often leaving open wounds that will not heal. For unknown reasons, people with Hidradenitis develop plugging or clogging of their apocrine glands.[2] HS causes chronic scarring and pus formation of the underarms (axilla) and groin/inner thigh areas. The simple procedure of incision and drainage provides some relief from severe, often debilitating, pressure and pain. Flare-ups may be triggered by perspiration, hormonal changes (such as monthly cycles in women), humidity and heat, and friction from clothing. Persistent lesions may lead to scarring and the formation of sinus tracts, or tunnels connecting the abscesses or infections under the skin.[citation needed] At this stage, complete healing is usually not possible, and progression varies from person to person, with some experiencing remission anywhere from months to years at a time, while others may worsen and require multiple surgeries in order to live comfortably. Wound dehiscence, a premature "bursting" open of a wound, often complicates the healing process.[citation needed] Occurrences of bacterial infections and cellulitis (deep tissue inflammation) may occur at these sites. Hidradenitis suppurativa pain and depression can be difficult to manage.[3]

HS often goes undiagnosed for years because patients are too ashamed to speak with anyone.[3] When patients see a physician or medical practitioner, the disease is frequently misdiagnosed or patients are prescribed ineffective treatments that can be temporary and sometimes even harmful.[citation needed] There is currently no known cure nor any consistently effective treatment. Carbon dioxide laser surgery is currently considered the last resort for those who have advanced to its highest stage, where the affected areas are excised, and the skin is grafted. Surgery does not always alleviate the condition, but may be very expensive.[citation needed]

Several articles and clinics consider this disease as widely misdiagnosed, due to the misunderstanding of the causes and progression of the disease.[4] HS is not caused by poor hygiene.[citation needed] HS is often called an orphan illness due to little current research being conducted on the topic. Since HS is considered a rare disease, its incidence rate is not well established, but has been estimated as being between 1:24 (4.1%) and 1:600 (0.2%).[5]

Areas of involvement with hidradenitis suppurativa

HS patients (n=164, 121 females, 43 males)FemaleMalep (statistical significance)
Axillae70 (58%)30 (70%)NS (not significant)
Mammary and inter-mammary31 (26%)2 (5%)0.006
Inguino-femoral111 (92%)32 (74%)0.007
Perianal and Perineal40 (33%)24 (56%)0.01
Buttocks30 (25%)21 (50%)0.006
Other names for HS

Hidradenitis suppurativa has been referred to by multiple names in the literature, as well as in various cultures. Some of these are also used to describe different diseases, or specific instances of this disease.[3]

Historical overview of hidradenitis suppurativa

A surgeon from Paris, Velpeau described an unusual inflammatory process with formation of superficial axillary, sub-mammary and perianal abscesses in 1839. One of his colleagues also located in Paris, named Verneuil, coined the term “hidrosadénite phlegmoneuse” approximately 15 years later. This name for the disease reflects the former pathogenetic model of acne inversa, which is considered inflammation of sweat glands as the primary cause of hidradenitis suppurativa. In 1922 Schiefferdecker suspected a pathogenic association between acne inversa and apocrine sweat glands. In 1956 Pillsbury postulated follicular occlusion as the cause of acne inversa, which they grouped together with acne conglobata and perifolliculitis capitis abscendens et suffodiens (dissecting cellulitis of the scalp) as the "acne triad". Plewig and Kligman added another element to their acne triad, pilonidal sinus. Plewig et al. noted that this new "acne tetrad" includes all the elements found in the original "acne triad", in addition to a fourth element, pilonidal sinus. In 1989, Plewig and Steger introduced the term "acne inversa", indicating a follicular source of the disease and replacing older terms such as "Verneuil disease".

Historical view of hidradenitis suppurativa

AuthorYearFindings
Valpeau1839First description of the hidradenitis suppurativa
Verneuil1854"Hidrosadénite phlegmoneuse"
Pillsbury1956Acne triad (hidradenitis suppurativa, perifolliculitis capitis abscendens et suffodiens)
Plewig & Kligman1975Acne tetrad (acne triad + pilonidal sinus)
Plewig & Steger1989Acne inversa

Dermatohistological view of hidradenitis suppurativa

AuthorYearMajor Features
Plewig & Steger[15]1989Initial hyperkeratosis of the follicular infundibulum. Bacterial super-infection and follicle rupture. Granulomatous inflammatory reaction of the connective tissue. Apocrine and eccrine sweat glands secondarily involved.
Yu & Cook[16]1990Cysts and sinus tracts lined with epithelium, in part with hair shafts. Inflammation of apocrine sweat glands only if eccrine sweat glands and hair follicles are also inflamed.
Boer & Weltevreden[17]1996Primary inflammation of the follicular infundibulum. Apocrine sweat glands are secondarily involved.

Stages

HS presents itself in three stages.[3][18] Due to the large spectrum of clinical severity and the severe impact on quality of life, a reliable method for evaluating HS severity is needed. Evaluation should take into account the number, type and size of lesions, evolution, pain and repercussions for the quality of life of the patient.[citation needed] Such a comprehensive instrument does not yet exist, but there have been two successful attempts proposed to classify patients with HS according to the severity of their disease.[citation needed]

Hurley's staging system

This is historically the first classification system proposed, and is still in use for the classification of patients with skin/dermatologic diseases (i.e., psoriasis, HS, acne). Hurley separated patients into three groups based largely on the presence and extent of cicatrization and sinuses. It has been used as a basis for clinical trials in the past and is a useful basis to approach therapy for patients. These three stages are based on Hurley's staging system, which is simple and relies on the subjective extent of the diseased tissue the patient has. Hurley's three stages of hidradenitis suppurativa are as follows:[19]

StageCharacteristics
ISolitary or multiple isolated abscess formation without scarring or sinus tracts. (A few minor sites with rare inflammation; may be mistaken for acne.)
IIRecurrent abscesses, single or multiple widely separated lesions, with sinus tract formation. (Frequent inflammation restrict movement and may require minor surgery such as incision and drainage.)
IIIDiffuse or broad involvement across a regional area with multiple interconnected sinus tracts and abscesses. (Inflammation of sites to the size of golf balls, or sometimes baseballs; scarring develops, including subcutaneous tracts of infection – see fistula. Obviously, patients at this stage may be unable to function.)

Sartorious staging system

The Sartorious staging system is more sophisticated than Hurley's, and is likely to supplant it as a means for conducting clinical trials during research. Sartorius et al. have suggested that the Hurley system is not sophisticated enough to assess treatment effects in clinical trials. The need for uniform outcome variables when reporting treatment effects has led to the proposition of a score by Sartorious and colleagues. This classification allows for better dynamic monitoring of the disease severity in individual patients and therefore forms a complementary system to the Hurley classification. They suggest a system that incorporates several of the involved areas of consideration:[20]

Points are accumulated in each of the above categories, and added to give both a regional and total score. In addition, the authors recommend adding a visual analog scale for pain or using the dermatology life quality index (DLQI, or the Skindex) when assessing HS.[21] This system will likely be the basis of most future clinical trials and research studies.

Causes

As this disease is poorly studied, the cause of Hidradenitis suppurativa remains unknown and experts disagree over proposed causes.[22]

Hidradenitis suppurativa occurs when apocrine glands become plugged. Lesions occur in areas of the body with numerous apocrine glands such as the axilla, groin, and perianal region. This theory includes most of the following potentials indicators:[23]

The historical understanding of the disease is that there are dysfunctional apocrine glands[18] or dysfunctional hair follicles,[27] possibly triggered by a blocked gland, creating inflammation, pain, and a swollen lesion.

Triggering factors

There are a number of triggering factors that should be taken into consideration, as it is advisable to avoid such triggers.

Predisposing factors

Severe complications

In disease stage III, fistulas left undiscovered, undiagnosed, or untreated, can lead to the development of squamous cell carcinoma, a rare cancer, in the anus or other affected areas.[37][38] Other stage III chronic sequelae may also include anemia, multilocalized infections, amyloidosis, and arthropathy. Stage III complications have been known to lead to death, but clinical data is still uncertain.

Potential complications

Treatments

Treatments may vary depending upon presentation and severity of the disease. Due to the poorly studied nature of this disease, the effectiveness of the drugs and therapies listed below is unclear, and patients should discuss all options with their physician or dermatologist. Nearly a quarter of patients state that nothing relieves their symptoms.[45] Possible treatments include:

Electron beam radiotherapy has been a successful treatment of hidradenitis, especially in Europe; it is not a common treatment option in most of the United States, as radiation oncologists generally refuse to treat patients with non-malignant diseases because of the potential for secondary radiation-induced tumors in the long term.

When the process becomes chronic, wide surgical excision is the procedure of choice. Wounds in the affected area do not heal by secondary intention, and immediate application of a split thickness skin graft is more appropriate.[23]

References

  1. ^ Alikhan; Lynch, Eisen (2009). "Hidradenitis suppurativa: a comprehensive review". J Am Acad Derm 60 (4): 539–561. doi:10.1016/j.jaad.2008.11.911. PMID 19293006. 
  2. ^ Mayo Clinic Staff. "Causes". Mayo Clinic. Retrieved 19 September 2012. 
  3. ^ a b c d "HS-USA :: What is Hidradenitis Suppurativa?". Retrieved 2007-07-08. 
  4. ^ Gao M; Wang PG; Cui Y et al. (2006). "Inversa acne (hidradenitis suppurativa): a case report and identification of the locus at chromosome 1p21.1-1q25.3". J. Invest. Dermatol. 126 (6): 1302–6. doi:10.1038/sj.jid.5700272. PMID 16543891. 
  5. ^ "HS-USA: Prevalence of Hidradenitis Suppurativa". Retrieved 2007-07-08. 
  6. ^ Sellheyer K; Krahl D. (2005). ""Hidradenitis suppurativa" is acne inversa! An appeal to (finally) abandon a misnomer". International Journal of Dermatology 44 (7): 535–40. doi:10.1111/j.1365-4632.2004.02536.x. PMID 15985019.  More than one of |number= and |issue= specified (help)
  7. ^ Bazex, J, Bayle, P, San, B. (2007). "Hidradenitis suppurativa is acne inversa". International Journal of Dermatology 46: 330. doi:10.1111/j.1365-4632.2007.02872.x. PMID 17343599. 
  8. ^ Scheinfeld N (2006). "Hidradenitis should not be renamed acne inversa". Dermatol. Online J. 12 (7): 6. PMID 17459292. 
  9. ^ Verneuil AS (1854). "Etudes sur les tumor de la peau". Arch Gen Med (in French) 94: 693. 
  10. ^ Velpeau A. Aissele. In: Bechet Jeune Z: Dictionnaire de médecine, on Repertoire Générale des Sciences Medicals sous le Rapport Theorique et Pratique. 1839.
  11. ^ Verneuil AS (1854). "Etudes sur les tumeurs de la peau et quelques maladies de glandes sudoripares". Arch Gen Med 94: 693–705. 
  12. ^ Schiefferdecker B. Die Hautdrüsen der Menschen und der Säugetiere, ihre histologische und rassenanatomische Bedeutung sowie die muscularis sexualis. In: Schweizerbart E (Hrsg), Stuttgart: 1922.
  13. ^ Pillsbury DM, Shelley WB, Kligmann AM. Bacterial infections of the skin. In: Pillsbury DM: Dermatoloy. 1st ed. Philadelphia: 1956: 482–9.
  14. ^ Plewig G, Kligman A. Acne. Morphogenesis and Treatment. Berlin: Springer, 1975: 192–3.
  15. ^ a b Plewig G, Steger M. Acne inversa (alias acne triad, acne tetrad or hidradenitis suppurativa). In: Marks R, Plewig G: Acne and related disorders. London: Martin Dunitz; 1989: 345–57.
  16. ^ Yu C. and Cook M. (1990). "Hidradenitis suppurativa: a disease of follicular epithelium, rather than apocrine glands". Br J Dermatology 122 (6): 763–69. doi:10.1111/j.1365-2133.1990.tb06264.x. PMID 2369556. 
  17. ^ Boer J, Weltevreden EF. "Hidradenitis suppurativa or Acne inversa. A clinicopathological study of early lesions". Br J Dermatol 1996 135: 721–5. 
  18. ^ a b DermNet acne/hidradenitis-suppurativa
  19. ^ Hurley HJ. Axillary hyperhidrosis, apocrine bromhidrosis, hidradenitis suppurativa, and familial benign pemphigus: surgical approach. In: Roenigk RK, Roenigk HH, editors. Dermatologic surgery. Marcel Dekker, New York, 1989, pp. 729–739.
  20. ^ Sartorius K, Lapins J, Emtestam L, Jemec GB (2003). "Suggestions for uniform outcome variables when reporting treatment effects in hidradenitis suppurativa". Br J Dermatol 149 (1): 211–213. doi:10.1046/j.1365-2133.2003.05390.x. PMID 12890229. 
  21. ^ Wolkenstein, P; Loundou, A; Barrau, K; Auquier, P; Revuz, J; Quality of Life Group of the French Society of Dermatology (2007). "Quality of life impairment in hidradenitis suppurativa: a study of 61 cases". Journal of the American Academy of Dermatology 56 (4): 621–3. doi:10.1016/j.jaad.2006.08.061. PMID 17097366. 
  22. ^ Medline Plus (2012). "Hidradenitis suppurativa". U.S. National Library of Medicine. Retrieved 19 September 2012. 
  23. ^ a b Schawartz's principles of surgery, 8th edition, self assessment an board review, chapter 15, the skin and subcutaneous tissue, question 16
  24. ^ Mayo Clinic Staff (2012). "Definition". Mayo Clinic. Retrieved 19 September 2012. 
  25. ^ ClinicalTrials.gov NCT00329823 Etanercept in Hidradenitis Suppurativa
  26. ^ Cusack C, Buckley C (2006). "Etanercept: effective in the management of hidradenitis suppurativa". Br J Dermatol 154 (4): 726–9. doi:10.1111/j.1365-2133.2005.07067.x. PMID 16536817. 
  27. ^ "HSF – What is Hidradenitis Suppurativa? What is HS?". Retrieved 2007-07-08. 
  28. ^ a b Slade DEM, Powell BW, Mortimer PS (2003). "Hidradenitis suppurativa: pathogenesis and management". Br J Plast Surg 56 (5): 451–61. doi:10.1016/S0007-1226(03)00177-2. PMID 12890458. 
  29. ^ a b c Jemec GBE. Body weight in hidradenitis suppurativa. In: Marks R, Plewig G, editors. Acne and Related disorders. London: Martin Dunitz; 1989. pp. 375–6.
  30. ^ Koning A, Lehmann C, Rompel R, Happle R (1999). "of Hidradenitis suppurativa". Dermatology 198 (3): 261–4. doi:10.1159/000018126. PMID 10393449. 
  31. ^ Morgan WP, Leicester G (1979). "The role of depilation and deodorants in hidradenitis suppurativa". Br J Surg 66 (12): 853–6. PMID 509057. 
  32. ^ Stellon AJ, Wakeling M (1989). "Hidradenitis suppurativa associated with use of oral contraceptives". Br Med J 298 (6665): 28–9. doi:10.1136/bmj.298.6665.28. 
  33. ^ Gupta AK, Knowles SR, Gupta MA, Jaunkalns R, Shear NH (1995). "Lithium therapy associated with hidradenitis suppurativa: case report and a review of the dermatologic side effects of lithium". J Am Acad Dermatol 32 (2 Pt 2): 382–6. doi:10.1016/0190-9622(95)90410-7. PMID 7829746. 
  34. ^ Medscape: Medscape Access. Emedicine.medscape.com. Retrieved on 2011-11-06.
  35. ^ Der Werth JM, Williams HC, Raeburn JA (2000). "The clinical genetics of hidradenitis suppurativa revisited". Br J Dermatol 142 (5): 947–53. doi:10.1046/j.1365-2133.2000.03476.x. PMID 10809853. 
  36. ^ Barth JH, Kealey T (1991). "Androgen metabolism by isolated human axillary apocrine glands in hidradenitis suppurativa". Br J Dermatol 125 (4): 304–8. doi:10.1111/j.1365-2133.1991.tb14162.x. PMID 1954117. 
  37. ^ Talmant JC, Bruant-Rodier C, Nunziata AC, Rodier JF, Wilk A (2006). "[Squamous cell carcinoma arising in Verneuil's disease: two cases and literature review]". Ann Chir Plast Esthet (in French) 51 (1): 82–6. doi:10.1016/j.anplas.2005.11.002. PMID 16488526. 
  38. ^ Short KA, Kalu G, Mortimer PS, Higgins EM (2005). "Vulval squamous cell carcinoma arising in chronic hidradenitis suppurativa". Clin. Exp. Dermatol. 30 (5): 481–3. doi:10.1111/j.1365-2230.2005.01875.x. PMID 16045671. 
  39. ^ Hurley HJJ. Apocrine glands. New York: McGraw Hill; 1979.
  40. ^ Tennant F Jr, Bergeron JR, Stone OJ, Mullins JF (1968). "Anemia associated with hidradenitis suppurativa". Arch Dermatol 98 (2): 138–40. doi:10.1001/archderm.98.2.138. PMID 5667225. 
  41. ^ Williams ST, Busby RC, DeMuth RJ, Nelson H (1991). "Perineal hidradenitis suppurativa: presentation of two unusual complications and a review". Ann Plast Surg 26 (5): 456–62. doi:10.1097/00000637-199105000-00008. PMID 1952719. 
  42. ^ Lapins J, Ye W, Nyren O, Emtestam L (2001). "Incidence of cancer among patients with hidradenitis suppurativa". Arch Dermatol 137 (6): 730–4. PMID 11405761. 
  43. ^ Moschella SL (1966). "Hidradenitis suppurativa. Complications resulting in death". JAMA 198 (1): 201–3. doi:10.1001/jama.198.1.201. PMID 5953172. 
  44. ^ Vasey FB, Fenske NA, Clement GB, Bridgeford PH, Germain BF, Espinoza LR (1984). "Immunological studies of the arthritis of acne conglobata and hidradenitis suppurativa". Clin Exp Rheumatol 2 (4): 309–11. PMID 6241861. 
  45. ^ "About Hidradenitis Suppurativa". abscesses.org. Retrieved 2007-07-08. 
  46. ^ a b Martinez F, Nos P, Benlloch S, Ponce J (2001). "Hidradenitis suppurativa and Crohn's disease: response to treatment with infliximab". Inflammatory Bowel Dis 7 (4): 323–326. doi:10.1097/00054725-200111000-00008. PMID 11720323. 
  47. ^ Gener G; Canoui-Poitrine F; Revuz JE et al. (2009). "Combination therapy with clindamycin and rifampicin for hidradenitis suppurativa: a series of 116 consecutive patients". Dermatology (Basel) 219 (2): 148–54. doi:10.1159/000228334. PMID 19590173. 
  48. ^ Mortimer PS, Dawber RP, Gales MA, Moore RA (1986). "A double blind controlled cross-over trial of cyproterone acetate in females with hidradenitis suppurativa". Br J Dermatol 115 (3): 263–8. doi:10.1111/j.1365-2133.1986.tb05740.x. PMID 2944534. 
  49. ^ Cusack C, Buckley C (2006). "Etanercept: effective in the management of hidradenitis suppurativa". Br. J. Dermatol. 154 (4): 726–9. doi:10.1111/j.1365-2133.2005.07067.x. PMID 16536817. 
  50. ^ Scheinfeld N (2006). "Treatment of coincident seronegative arthritis and hidradentis supprativa with adalimumab". J. Am. Acad. Dermatol. 55 (1): 163–4. doi:10.1016/j.jaad.2006.01.024. PMID 16781316. 
  51. ^ Anabelle Brocard, Anne-Chantal Knol, Amir Khammari, Brigitte Dréno (2007). "Hidradenitis Suppurativa and Zinc: A New Therapeutic Approach". Dermatology 214 (4): 325–7. doi:10.1159/000100883. PMID 17460404. 
  52. ^ Clemmenson OJ (1983). "Topical treatment of hidradenitis suppurativa with clindamycin". Int J Dermatol 22 (5): 325–8. doi:10.1111/j.1365-4362.1983.tb02150.x. PMID 6347922. 
  53. ^ Boer J (2010). "Resorcinol peels as a possible self-treatment of painful nodules in hidradenitis suppurativa.". Clin Exp Dermatol 35 (1): 36–40. PMID 19549239. 

External links