Author Topic: Hidradenitis Suppurativa Articles  (Read 96072 times)

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Celery Peach

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Hidradenitis Suppurativa Articles
« on: Monday March 08, 2004, 12:32:08 PM »
 :hi: Guys.

I have started this thread, after conflabs with Nick, so I can just post articles of interest to HS sufferers. Its good for me to have all these in one place. HS, being a 'so called' orphan illness, means that these articles are tucked in here & there all over the web.

I hope Jo (missmash) & others will join me in posting HS info here, but I would ask, can we keep it to articles rather than chit chat (no offence to anyone) I would just like to keep it strictly info.  
« Last Edit: Thursday February 03, 2005, 12:49:08 PM by Celery Peach »

Celery Peach

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Re:Hidradenitis Suppurativa Articles
« Reply #1 on: Monday March 08, 2004, 12:36:44 PM »
Hidradenitis suppurativa. An update
A. J. Papadopoulos, G. Kihiczak and R. A. Schwartz
   

   Summary
Hidradenitis suppurativa is a chronic and often disabling disease characterized by intermittent periods of inflammation and abscess formation in apocrine sweat gland-containing skin. Various therapies have been implemented in its treatment with variable results. Pathogenesis, histopathology and therapy of this disease are reviewed.
   
  Introduction
Hidradenitis suppurativa (HS) is a chronic disease of unknown etiology that mainly affects the genitofemoral, perianal and axillary regions (1-4). The disease is believed to be follicular in origin (1). HS has a higher prevalence in women than men and is estimated to affect 4 percent of women in the general population (5).  

Etiology
HS often occurs in multiple members within families, suggesting a genetic predisposition (5). Various etiological agents have been associated. The increased incidence of HS in obese women with acne led to the theory of HS being associated with an hyperandrogenic endocrine disorder (6,7). One study did not find evidence for biochemical hyperandrogenism in women affected with HS (8). Thus, the role of androgens in HS is still not clear. Smoking (9), lithium (10) and oral contraceptives (11) may also be associated with HS, possibly as triggering factors. Nonetheless, the etiology of the disease is unknown.  

Pathogenesis
Historically, apocrinitis was believed to be the defining histologic feature and pathogenetic mechanism of HS (12,13). Characteristic lobular abscesses in the apocrine gland, demonstrated histologically, seemed to further implicate apocrine gland involvement in the manifestation of disease (12-14). Recently, histological evidence points to HS being a follicular disease (1,15-19). Histological examination in the majority of specimens reveals follicular involvement, including poral occlusion and folliculitis (1). Apocrinitis as the dominant histological feature is found in only a small number of specimens (1,17). Furthermore, a paucity of apocrine glands was demonstrated in the genitofemoral region, one area commonly affected by HS (1). This finding supports the theory that apocrine gland inflammation is not the etiological and pathogenetic mechanism of HS, but rather a secondary manifestation of follicular involvement (1).  

Clinical features
HS may arise singularly or multifocally in the genitofemoral, perianal and axillary areas (1-4). The lesions are painful and have a foul odor attributed to bacterial colonization. Erythematous dermal abscesses form that measure up to 2 cm in diameter. Untreated abscesses will gradually increase in size and may drain to the surface. The course of HS is chronic and remitting, with new abscesses arising in previously unaffected areas or in regions of past involvement. Scarring, fibrosis and sinus tract formations are manifestations of late disease. Strictures can occur secondary to sinus tracts; fistulas may also complicate HS. Squamous cell carcinoma is a rare sequella of longstanding HS (20-24). These cancers may be locally aggressive with distant metastases and a high mortality rate (25).

Clinical associations
The follicular occlusion triad consists of HS, acne conglobata and perifolliculitis capitis abscedens et suffodiens (26-28). Arthritis of peripheral joints and the axial skeletion may rarely be associated with HS (29-32). HS has also been linked to Crohn's disease (33-35). One study reported 24 out of 61 patients with HS were also diagnosed with Crohn's disease, which predated the HS by an average of 3.5 years (35). Acanthosis nigricans and Fox-Fordyce disease may predispose to HS (36). Pyoderma gangrenosum (37,38) and pyoderma vegetans (27) have also been associated with hidradenitis suppurativa.

Therapy
Treatment of hidradenitis suppurativa is challenging. Late stage disease, evidenced by the formation of sinus tracts, fibrosis and scarring, usually necessitates surgical intervention. Early HS is often best treated with antibiotics in our experience, although few clinical trials are available (39). Three months' of treatment with topical clindamycin decreased the number of abscesses, inflammatory nodules and pustules in twenty-seven patients with chronic HS (40). Systemic tetracycline therapy has shown similar clinical effectiveness (39). Others and also we often recommend intrealesional corticosteroids in early stage disease (41). The use of cyproterone acetate and ethinyl estradiol achieved successful clinical results in four women with chronic HS (42); clinical improvement with cyproterone acetate and ethinyl estradiol was also described in another study (43). Isotretinoin is only slightly effective in controlling the disease; clinical improvement is seen in patients with mild HS (44). The clearing of chronic, refractory perianal HS was seen after treatment with cyclosporin for concomitant pyoderma gangrenosum (38).
Medical therapy is of limited value once HS has progressed past its early stage (2). The surgical option of choice for late stage HS is wide local excision with healing by secondary intention (41). One study examined patients with chronic HS who had undergone surgery between the years of 1976 and 1997 (4). An estimated 72-month follow-up revealed that 45% of the patients had recurrence of local HP (4). A 100% recurrence rate was reported after drainage procedures, while limited and wide local excision techniques had a recurrence rate of 42.8% and 27%, respectively (4). More recently, carbon dioxide laser excision has been proposed as a better alternative to conventional surgery (2,45). Carbon dioxide laser excision offers better hemostasis and visualization of abscessed tissue than conventional surgical techniques, allowing more accurate excision (2).
 

Celery Peach

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Re:Hidradenitis Suppurativa Articles
« Reply #2 on: Monday March 08, 2004, 12:41:22 PM »
Acne inversa in Crohn's disease]

[Article in German]

Goischke HK, Ochsendorf FR.

Hartwald-Rehabilitationsklinik der BfA, Bad Bruckenau.

We report 2 patients with the association of Crohn's disease and acne inversa (= hidradenitis suppurativa). A 50-year-old woman with a 10-year-history of Crohn's disease developed suppurative nodules and abscesses in the genitoanal region. Over several years these lesions were hidden from everybody including the family physician. In a 42-year-old female patient abscesses appeared in the axilla 4 years after the diagnosis of Crohn's disease. During the next years these draining lesions also developed in both groins and the perineum. The differential diagnosis included cutaneous manifestations of Crohn's disease in both cases. The clinical picture with draining abscesses and sinuses with communicating channels, cystic nodules, hypertrophic scars and foul-smelling discharge were typical findings of acne inversa. In the last years this entity was reported several times in patients with Crohn's disease. Acne inversa starts with a compact hyperkeratosis of the infundibulum in terminal hair follicles, followed by a segmental rupture of the follicular epithelium and a spreading inflammatory reaction. As pathogenic factors genetic disposition and smoking were discussed. The only curative therapy is excision of the affected area. The reported association should be known in order to recognize the disease and institute the right therapy.

Celery Peach

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Re:Hidradenitis Suppurativa Articles
« Reply #3 on: Monday March 08, 2004, 12:42:04 PM »
[Acne inversa. Pathogenesis and genetics]

[Article in German]

Kuster W, Rodder-Wehrmann O, Plewig G.

Hautklinik, Heinrich-Heine-Universitat Dusseldorf.

Acne inversa is a chronic inflammatory disorder of sebaceous follicles and terminal hair follicles and is one type of acne diseases. The pathogenesis of acne inversa is identical with that of the other types: Hyperkeratosis of the follicular infundibulum leads to a comedo. Bacterial infections result in a rupture of the follicular canal followed by a granulomatous inflammatory reaction with abscesses, panniculitis and draining sinuses. Acne inversa has so far only occasionally been observed in two or more members of the same family. The familial presentations of acne inversa published in the literature and two observations of familial occurrence among the authors' own patients reveal an autosomal dominant inheritance with high penetrance.

Celery Peach

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Re:Hidradenitis Suppurativa Articles
« Reply #4 on: Monday March 08, 2004, 12:43:01 PM »
Acne inversa (alias hidradenitis suppurativa).

Jansen I, Altmeyer P, Piewig G.

Department of Dermatology, Ruhr-University Bochum, Germany.

Acne inversa is a recurrent, suppurative disease manifested by abscesses, fistulas, and scarring. Once considered to be a disease of the apocrine glands, it is actually a defect of follicular epithelium. Thus, the term hidradenitis suppurativa is a misnomer and should be abandoned. In cases of familial acne inversa, the pattern of transmission and number of affected individuals are consistent with autosomal dominant inheritance. Aetiological factors such as hyperandrogenism, obesity, smoking and chemical irritants are not consistently associated with the affection. Bacterial involvement is not a primary event in acne inversa, but is secondary to the disease process. Potential complications include dermal contraction, local or systemic infection due to the spread of microorganisms, systemic amyloidosis, arthropathy, and squamous cell carcinoma. As spontaneous resolution is rare and progressive disability is the rule, early definitive surgical intervention is advisable. The surgical procedure of choice in most cases is wide local excision and healing by secondary intention. Pharmacotherapeutic drugs, including synthetic retinoids and antiandrogens, do not prevent progression of the disease

Celery Peach

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Re:Hidradenitis Suppurativa Articles
« Reply #5 on: Monday March 08, 2004, 12:47:10 PM »
[Acne inversa (hidradenitis suppurativa): early deetection and curative surgery]

[Article in German]

Herrmann A, Preusser KP, Marsch WC.

Universitatsklinik und Poliklinik fur Dermatologie und Venerologie, Martin-Luther-Universitat Halle-Wittenberg.

Acne inversa (synonyms are hidradenitis suppurativa, pyodermia fistulans sinifica) is a chronic inflammatory disorder of the terminal hair follicles of the intertrigines. Abscesses and fistules develop mostly in both axillae, in the anogenital region, and under the breasts. The disease is common, but the right diagnosis is often missed. Local incision gives only a short relief of pain. The therapy of choice is wide local excision. We report on 12 patients with acne inversa who were surgically treated from 1991-1999 in our department. Surgical results and patient satisfaction were assessed on an average of 4.2 years. In all, 20 excisions in the axillae and 5 excisions anogenital were done. Seven patients were treated preoperatively with isotretinoin over at least 2 months. All patients could move their limbs without trouble. The cosmetic result was good in nine patients and three expressed dissatisfaction. Nine patients did not develop new lesions in the treated area. Three patients had new inflammations in both treated and previously uninvolved and therefore not resected regions. Wide local excision gives very good functional and cosmetic results. It usually heate long-lasting disease for nearly disabled patients. Isotretinoin reduces the disease activity and allows radical surgical treatment.

Celery Peach

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Re:Hidradenitis Suppurativa Articles
« Reply #6 on: Monday March 08, 2004, 12:48:23 PM »
[Reticular pigmented anomaly of the flexures. Dowling-Degos disease of the intertrigo type in association with acne inversa]

[Article in German]

Kleeman D, Trueb RM, Schmid-Grendelmeier P.

Dermatologische Klinik, Universitatsspital Zurich, Gloriastrasse 31, CH-8091 Zurich.

Reticulated pigmented anomaly of the flexures (Dowling-Degos disease) is a rare autosomal-dominant genodermatosis with variable penetrance that is characterized by reticulated hyperpigmentation of the flexures. Acne inversa has been previously described in the literature under several synonyms, such as acne triad, acne tetrad and hidradenitis suppurativa. Acne inversa is an inflammation affecting the pilosebaceous units of the flexures. We report a case of Dowling-Degos disease in association with acne inversa. The association of these diseases has been described several times and it appears not to be coincidental. Pathogenetic mechanisms leading to this coincidence are discussed.

Celery Peach

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Re:Hidradenitis Suppurativa Articles
« Reply #7 on: Monday March 08, 2004, 12:49:23 PM »
The differential diagnosis and comorbidity of hidradenitis suppurativa and perianal Crohn's disease.

Church JM, Fazio VW, Lavery IC, Oakley JR, Milsom JW.

Department of Colorectal Surgery, Cleveland Clinic Foundation, Ohio.

Over the last 8 years, 61 patients with hidradenitis suppurativa (HS) have been treated at this institution. Twenty-four have also had a diagnosis of Crohn's disease (38%). This dual pathology is examined in detail in this retrospective review. There were 11 males and 13 females with a mean age of 39 years (range 18 to 75 years). The Crohn's disease was ileal in 1 patient, ileocolic in 4, and affected the large bowel only in 19. The diagnosis of Crohn's disease predated that of HS by an average of 3.5 years. At the time of review, 22 patients had a stoma, 23 had undergone laparotomy and 17 had lost their rectum. Hidradenitis suppurativa occurred in the perineal or perianal area in all patients but involved other sites in 20 cases. Skin grafting had been done in 9 and local procedures in 19 patients. Granulomas were found in excised skin in 6 cases but this finding was not associated with a poor outcome. At a mean follow-up of 3.2 years from the most recent surgery for HS (range 1 to 11 years) 11 were asymptomatic for HS, 11 had symptoms and no follow up was available in 2. These data show that HS may coexist with Crohn's proctocolitis, complicating the diagnosis and management of patients in whom it occurs. An increased appreciation of the possibility is recommended

Offline Nick

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Re:Hidradenitis Suppurativa Articles
« Reply #8 on: Monday March 08, 2004, 12:51:40 PM »
I have taken the liberty of making this thread "sticky" as I think it will be a continuing project for the forum :up:

Celery Peach

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Re:Hidradenitis Suppurativa Articles
« Reply #9 on: Monday March 08, 2004, 12:51:43 PM »
[Acne inversa: a dapsone-sensitive dermatosis]

[Article in German]

Hofer T, Itin PH.

Abteilung fur Dermatologie, Kantonsspital Aarau.

Acne inversa is a chronic disease with a major impact on the quality of life. Therapeutic options were long restricted to local disinfectants and systemic antibiotics, as well as repeated incision and drainage which produce only short term benefits. Retinoids, antiandrogens and radiation therapy are only partially successful. The best approach appears to be surgical removal of the entire apocrine sweat gland apparatus. Dapsone is used in dermatology to treat inflammatory dermatoses such as dermatitis herpetiformis and pyoderma gangrenosum, and was formerly the treatment of choice for acne conglobata. We report its successful use in acne inversa. Five female patients aged 23-40 years with acne inversa for a mean of 9.6 years were included. All patients showed an almost complete resolution of their symptoms within 2-4 weeks. All patients rated the treatment results with dapsone as good or very good. The treatment was well tolerated and no important side effects occurred. Because of its lack of teratogenicity, dapsone may be the most favorable treatment option in young women with acne inversa.

Celery Peach

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Re:Hidradenitis Suppurativa Articles
« Reply #10 on: Monday March 08, 2004, 12:58:27 PM »
Nodulocystic acne and hidradenitis suppurativa treated with acitretin: a case report.

Scheman AJ.

North Shore Center for Medical Aesthetics, Northbrook, Illinois 60062, USA.

Nodulocystic acne is a dermatologic disease that can result in significant damage to the skin of the face, chest, and back. Hidradenitis suppurativa is a scarring disease of the skin that causes deep cysts and abscesses on the axillae and anogenital areas. We review a case of a patient with severe nodulocystic facial acne and hidradenitis suppurativa that was treated with 2 full courses of isotretinoin. Although the patient's condition improved, some draining cysts persisted on the face and groin. Because of the inability of isotretinoin to achieve long-term remission of the patient's condition, acitretin was considered as a possible maintenance drug. The patient was almost completely improved after 5 months' therapy with acitretin, which also was effectively used for ongoing maintenance. Acitretin may be a promising treatment for severe nodulocystic acne and hidradenitis suppurativa, which require long-term suppression when isotretinoin fails to give long-term remission.

Celery Peach

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Re:Hidradenitis Suppurativa Articles
« Reply #11 on: Monday March 08, 2004, 01:04:14 PM »
Hidradenitis suppurativa polyposa.

Wrone DA, Landeck A, Dibbell DG, Xie H, Warner TF.

Department of Medicine, University of Wisconsin Hospital and Clinics, Madison 53792, USA.

A case of severe chronic hidradenitis suppurativa of the perineum complicated by disfiguring fibrous, polypoid lesions is presented. The patient, a 41-year-old woman, had a long history of axillary hidradenitis which subsequently involved the perineum. Draining sinuses, scars and large pendulous masses of the vulva developed over 10 years. Cutaneous scars, ridges, papules and large fibrous polyps were present. Deep clefts, sinuses, dense fibrous scars and foci of chronic inflammation were seen. Rarely, large fibrous polyps may develop in chronic hidradenitis suppurativa and may be due to chronic local lymphedema. Careful pathologic examination is necessary to exclude squamous cell carcinoma.

Celery Peach

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Re:Hidradenitis Suppurativa Articles
« Reply #12 on: Monday March 08, 2004, 01:05:59 PM »
Immunohistochemical study of cytokeratins in hidradenitis suppurativa (acne inversa).

Kurokawa I, Nishijima S, Kusumoto K, Senzaki H, Shikata N, Tsubura A.

Department of Dermatology, Hyogo Prefectural Tsukaguchi Hospital, Hyogo, Japan. ikuro@alles.or.jp

In 14 cases of hidradenitis suppurativa, cytokeratin (CK) expression was studied immunohistochemically, using six antikeratin antibodies against CK1, CK10, CK14, CK16, CK17 and CK19, respectively. The draining sinus tract epithelium of hidradenitis suppurativa lesions was divided into three components: infundibular-like keratinized epithelium (type A), non-infundibular keratinized epithelium (type B), and non-keratinized epithelium (type C). In type A samples, CK17 (which is found in normal infundibulum) was not detected, suggesting fragility of this epithelial type. Keratin expression in types B and C epithelia was similar to that observed in the outer root sheath in normal hair follicles. Our results suggest that the draining sinus epithelium may possess characteristics of fragility, undifferentiation and hyperproliferation, as shown with CK expression.

Celery Peach

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Re:Hidradenitis Suppurativa Articles
« Reply #13 on: Monday March 08, 2004, 01:10:09 PM »
Perineal hidradenitis suppurativa: presentation of two unusual complications and a review.

Williams ST, Busby RC, DeMuth RJ, Nelson H.

Department of General Surgery, Oregon Health Services University, Portland.

Two patients with advanced perineal hidradenitis suppurativa, complicated by fecal incontinence and squamous cell carcinoma, are presented. The first patient was a 58-year-old man who had a 30-year history of chronic recurring perianal abscesses and perineal sinuses. At the time of presentation, he had extensive perineal suppurative disease, and scarring and fixation of the anal sphincters with resultant fecal incontinence. He was treated with wide excision and skin graft closure. The second patient was a 27-year-old man with an 11-year history of recurrent gluteal abscesses and perineal sinuses. At the time of presentation, his inflammatory disease was only mildly active, but he had a nonhealing gluteal lesion. The nonhealing lesion was diagnosed as a squamous cell carcinoma and was managed with wide excision and primary closure. The inflammatory disease was excised and grafted. Complications of advanced hidradenitis suppurativa can be debilitating and life threatening. We review the etiology, pathophysiology, complications, and treatment options of hidradenitis suppurativa, including a literature review of the association with malignancy. We propose that the incidence of disabilities and complications may be reduced by early diagnosis and treatment, by emphasis on prevention of recurrence, and by more aggressive surgical intervention for recurrent and extensive disease.

Celery Peach

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Re:Hidradenitis Suppurativa Articles
« Reply #14 on: Monday March 08, 2004, 01:13:34 PM »
Skin disease tied to greater squamous cell cancer risk. (Hidradenitis Suppurativa).
OB/GYN News, Dec 15, 2001, by Bruce Jancin

MUNICH -- Hidradenitis suppurativa is associated with a markedly increased risk of squamous cell carcinoma, Dr. JeanPaul Ortonne said at the 10th Congress of the European Academy of Dermatology and Venereology.

This chronic inflammatory skin disease may also be associated with increased risks of buccal and primary liver cancer, although this possibility needs to be interpreted with caution, said Dr. Ortonne of the National Institute of Health and Medical Research, Nice.

He cited a retrospective study by Dr. Jan Lapins and associates at Stockholm's Karolinska Institute. This was the first large-scale follow-up study of patients with hidradenitis suppurativa.

The investigators identified all 2,119 patients discharged with a diagnosis of hidradenitis suppurativa from any Swedish hospital during 1965-1997 and linked those records to the Swedish National Cancer Registry After excluding all cancers diagnosed within 1 year after hospitalization on the grounds that the malignancy might have predated the hidradenitis suppurativa, the Swedish investigators determined that patients with hidradenitis suppurativa were at 4.6-fold increased risk of subsequently developing squamous cell carcinoma. Women faced an 8.2-fold increase; for men, the increase was 1.7 fold (Arch. Dermatol. 137[6]:730-34, 2001).

This study validates prior anecdotal reports suggesting a possible association between hidradenitis suppurativa and nonmelanoma skin cancer, Dr. Ortonne said. The suspicion is that the chronic inflammatory bacterial infections and foreign body reactions that figure so prominently in hidradenitis suppurativa can trigger epidermal proliferative changes, including malignancy The higher skin cancer risk in women than men with hidradenitis suppurativa could be related to hormonal influences, or to the fact that women tend to get the inflammatory skin disease at an earlier age than men and hence have lengthier exposure to the disease's effects.

The Swedish study also demonstrated that patients with hidradenitis suppurativa were at 5.5-fold increased risk of buccal cancer and 10-fold elevated risk of primary liver cancer.

Celery Peach

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Re:Hidradenitis Suppurativa Articles
« Reply #15 on: Monday March 08, 2004, 03:30:41 PM »
Hidradenitis Suppurativa
Surgery

Hidradenitis suppurativa refers to a chronic relapsing cutaneous inflammation of the apocrine glands of the body. Chronic hidradenitis suppurativa can affect, in order of preference, the axilla, perineum, scrotum, inguinal, scalp, palms of the hand, soles of thefeet and the submammary region. Hidradenitis suppurativa usually manifests itself afterpuberty (androgen-dependent disorder). Females are more commonly affected than males.The inflammatory process begins as a local occluding spongiform infundibulo-folliculitis of a sweat gland with subsequent rupture and secondary bacterial infection. Symptomsinclude pain, swelling, purulent discharge, and pruritus of the affected region. Associated medical conditions include diabetes and obesity. Initial management consists of general hygienic measures with antibiotics, antiandrogens and estrogens. Surgery is needed when the condition is at an advanced stage with cellulitis and scarring. Non operative treatment is disappointing. Total excision of all apocrine-bearing axillary tissue with primary closure is the treatment of choice. Operative treatment can be safely accomplished even when draining sinuses are present. Recurrence results from inadequate excision or an unusually wide distribution of apocrine glands, but physical factors such as obesity, local pressure,and skin maceration play a role. Radical surgery gives good symptomatic control of severe hidradenitis suppurativa of the axilla, inguino perineal, and perianal regions but is less satisfactory for submammary disease

Celery Peach

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Re:Hidradenitis Suppurativa Articles
« Reply #16 on: Monday March 08, 2004, 03:45:51 PM »
 :up: I thought this one was very informative

Hidradenitis Suppurativa
JoergPeter Ritz MD and Heinz J Buhr MD FACS
Department of Visceral Vascular and Thoracic Surgery, Hindenburgdamm 30, University Hospital Benjamin Franklin Free University of Berlin, Berlin, 12200, Germany

Current Treatment Options in Infectious Diseases 2001, 3:379-385

--------------------------------------------------------------------------------
   
Outline   Opinion statement  

Hidradenitis suppurativa (HS) is a chronic fistula- and abscess-forming disease of the cutis and subcutis of unknown etiology. Disease recurrence is frequent and may cause severe complications. This article reports on the information in the literature regarding epidemiology, pathogenesis, clinical manifestations, and treatment options of HS. HS occurs mainly in the third to fourth decade of life and seems to be associated with obesity, smoking, chemical irritants, and hyperandrogenism; bacterial involvement is secondary. Although acute intermittent lesions of early HS often respond to conservative therapy, such treatment most likely does not alter the clinical course of the disease. Patients may occasionally derive symptomatic benefit from long-term antibiotics, but relapse is almost inevitable when treatment is withdrawn. Therefore, surgery is the only effective therapy for severe HS, especially in view of possible complications after long-standing disease. Incision and drainage of each lesion and abscess may temporarily improve symptoms but do not cure the underlying sinuses and infected apocrine glands. Drainage procedures and limited resections lead to an unacceptable rate of recurrence with an unnecessary risk of life-threatening complications such as squamous cell carcinoma. Recurrence is mostly due to limited resection and inadequate eradication of sweat glands. Radical wide excision of the HS-affected cutis is associated with the lowest recurrence rate. In general, HS recurs the earlier the less infected and abscess- containing skin is resected. Obesity, local pressure, and skin maceration are important promoting factors. Approximately 20 to 25% of HS progresses to a previously unaffected area, which cannot be influenced by therapy options. Patients with HS need to be followed up and treated over a long period of time to exclude late- developing recurrences and to detect a malignant degeneration in an early stage by histopathologic examination of suspicious areas.

Outline   Introduction    

Hidradenitis suppurativa (HS) was first described as a clinical entity by Velpeau in 1839 as a peculiar inflammatory process with superficial abscess formation, affecting tissues of the axillary, mammary, and perianal regions. In 1864, the French surgeon Verneuil was the first to associate HS with the sweat glands [1-3]. In 1922, Schiefferdecker classified sweat glands in eccrine or apocrine and subsequently localized HS to the apocrine glands because those areas rich in these glands (such as axillary, inguinal, genital, perianal, and perineal regions) are affected by the disease [4¢¢,5].

The prevalence of HS is unknown. Jemec [6]reported a point prevalence of 4.1% based on objective findings in a younger adult population. The disease develops mainly in men in the third and fourth decades of life, which coincides with postpubertal increases in androgen levels [1,4¢¢,7]. Onset before puberty is rare. In women, HS sometimes persists into the climacteric; onset after menopause is rare. HS in the perianal region is 10 times more common in men than in women, whereas axillary disease is more prevalent in women [2,4¢¢,6-8]. Some authors believe that HS is more common in the black population, but some report no racial predilection [6-8].

Although its exact etiology is unknown, HS seems to be associated with obesity, proneness to acne, excessive sweating, endocrine disorders such as Cushing's syndrome or diabetes, poor personal hygiene, and smoking [4¢¢,8,9]. Many authors consider chemical irritants such as deodorants, mechanical irritation, depilation, and shaving to be factors. Morgan and Leicester [10] retrospectively compared 40 patients who had HS with 40 age-matched controls and found no significant difference in the use of these items. Since Schiefferdecker, the disease has been considered a disorder of apocrine glands. This theory was supported by an experimental model that induced poral occlusion by manual skin depilation and application of atropine-impregnated tape. This resulted in dilation, inflammation, and bacterial invasion of the apocrine duct. However, the lesions did not progress to the characteristically chronic condition of HS [11]. More recent studies have identified HS as a disorder of follicular rather than apocrine occlusion. Attanoos et al. [5] studied the skin biopsies of patients with HS and reported a consistent finding of follicular occlusion in all specimens when compared with controls. They discussed that inflammation of the apocrine glands did not occur in the absence of an adjacent folliculitis. Thus, apocrine gland involvement seems to be secondary to the primary involvement of the terminal hair follicle [12]. In addition, several reports in the literature link HS to a single gene transmission. Fitzsimmons et al. [13,14¢] reported a pattern of autosomal dominant transmission in their study of three families with 21 affected members.

The disease onset is insidious, with early symptoms of pruritus, erythema, and local hyperhidrosis. Later, a firm pea-sized nodule appears and may rupture spontaneously, yielding a purulent discharge. Gland rupture spreads the infection to surrounding tissues. The cycle is repeated in the adjacent tissue, leading to localized abscesses, chronic draining sinuses, and finally scarring and fibrosis. The disease course is characterized by exacerbations and remissions, with the extent of affected skin gradually increasing [4¢¢,5,7,12]. There are numerous reports of severe complications in chronic HS, including anemia, intestinal keratitis, osteomyelitis, fistulous communications to pelvic organs, and even death [15¢,16]. It remains unclear whether radical excision can prevent some of these complications. Squamous cell carcinoma (SCC) is a dreaded but rare complication with only 26 reported cases since the first description by Anderson in 1958 [16,17]. SCC tends to occur in patients with peri-anal and gluteal disease and is less frequent in axillary cases. In a review of all the published cases by Perez-Diaz et al. in 1995 [18], the average age at diagnosis was 47 years with a male predominance. The mean duration from the onset of HS to SCC was 20 years. Only four patients had a history of fewer than 10 years. Treatment varies from wide local excision to abdominoperineal resection, sometimes combined with radiotherapy or chemotherapy. According to follow-up data, 30% of the patients were alive and recurrence free at 1 year and 41% developed recurrent cancer [16-18].

   
Outline   Treatment  
 
Controversy exists regarding the appropriate treatment for HS. Consequently, there is no standard treatment and most studies lack a long-term follow-up after initial therapy. However, the care and observation of patients with HS remains an important factor because the disease can recur even years after initial therapy [16,19]. Conservative management ranges from antiseptic cleaning or topical steroid creams to systemic anti-biotic therapy [1-3,4¢¢,19,20¢,21¢]. Combined anti-androgen and estrogen therapy in women has been reported to control HS. Acute intermittent lesions of early HS often respond to conservative therapy, but it is unlikely that such treatment alters the long-term clinical course of the disease [19,20¢,21¢,22-23]. Patients may occasionally derive symptomatic benefit from long-term antibiotics, but relapse is almost inevitable when treatment is withdrawn. Therefore, surgery is the only effective therapy for severe HS, especially considering the possible complications after long-standing disease [24¢¢,25]. Selection of a treatment approach is facilitated by preliminary clinical staging (1). All patients, irrespective of the stage or extent of their HS, require proper maintenance care of the involved areas with emphasis on avoidance of local frictional trauma to prevent new outbreaks or recurrences. Thus, continued medical surveillance is usually necessary, even in most patients with definitive surgery for stage III disease.

Pharmacologic treatment

Because of the required extensive nature of surgical treatment, patients are often first administered medical treatment. Patients may have symptomatic relief with a long-term course of antibiotics. Antibiotics are not curative but may diminish unpleasant odor and discomfort in the patient. Conservative medical treatment is applied mainly in stage I disease (1). In general, after withdrawal of conservative treatment, relapse of HS is almost inevitable. The topical use of clindamycin has been favored in the literature. Hormonal therapy is of no value in HS. Isotretinoin or 13-cis-retinoid acid administered in 1 mg/kg daily doses is helpful in some patients with stage II or III disease but is not as beneficial as it is in acne vulgaris [12,19,27]. Anti-androgenes such as spironolacton and cyproterone acetate have been recommended to help prevent recurrent HS but are not routinely helpful.

Systemic antibiotics

Standard dosage

Tetracycline (1-1.5 g/day), minocycline (100-200 mg/day), or ciprofloxacin (250-500 mg/day) should be administered only in patients with early lesions with signs of local inflammation. The treatment should be maintained until all signs of the inflammation are gone, usually for 7 to 10 days.

Contraindications

None.

Special points

Bacterial culture with antibiotic sensitivity testing should be performed on available drainage material. Alternative antibiotic therapy should be based on the results of these studies [20¢]. Tetracycline and ciprofloxacin can be administered orally for 7 to 10 days in patients with early stages of the disease and does not require surgical incision or drainage. Treatment should be adapted according to antibiotic sensitivity testing.

Complications

High recurrence rate after withdrawal of therapy, complications of antibiotic treatment (ie, diarrhea).

Local treatment

Standard procedure

Gentle daily cleaning with a germicidal soap such as chlorhexidine gluconate is advised, as is daily application of a topical antibiotic (2% clindamycin).

Contraindications

None.

Special points

Systemic therapy with tetracyclines did not show better results than topical therapy with clindamycin in patients with HS.

Complications

None.

Surgery

Surgery is the basis for successful treatment of HS, especially in cases of chronic or recurrent disease (stages II and III). Radical surgery is considered the only curative therapy for HS, although the particular area excised can be cured. HS can recur in patients in remote or untreated regions because it may affect any apocrine gland-bearing area. Patients should be appropriately cautioned [16,21¢,24¢¢,25,28]. Currettage and electrofulguration can be used in the base of the exteriorization wounds to destroy residual disease epithelial elements or for hemostasis but are not routinely necessary. Because of the thick fibrotic scarring in these areas, satisfactory anesthesia may be difficult or impossible to achieve with local anesthetics. The surgical strategy can be subdivided into drainage procedures, limited regional exision, and radical wide excision.

Incision and drainage

Defined as simple abscess incisions or drainage of the affected area, this procedure can temporarily improve symptoms and offers a transient benefit. However, it does not cure the underlying sinuses and infected area. It is a simple surgical method with a low complication rate, but acute infection recurs within 1 to 3 months in nearly all patients (1 and 2) [16,24¢¢,29].

Contraindications

This procedure can be performed easily under local anesthesia on an outpatient basis in almost every patient without contraindications. The patient needs to be informed about the partial and temporary improvements of this procedure.

Complications

High recurrence rate.

Limited excision

Defined as resection of the infected abscess and fistula containing cutis in the affected region. This procedure carries a high risk of local recurrence in 40% to 50% of patients, occurring after a median of 9 to 11 months [16,24¢¢,25]. We found no advantage to limited regional excision of the affected skin (1 and 2).

Contraindications

None.

Complications

High recurrence rate.

Radical wide excision

Radical wide excision includes all hair-bearing skin (with or without signs of HS) of the affected region down to soft normal tissue with margins well beyond the clinical borders of activity (1 cm). This procedure is considered to be most effective in treating HS, with a recurrence rate of 20% to 30%. Most authors agree that radical wide excision of the affected skin minimizes recurrence. In a series of 82 patients with wide excision and a mean follow-up of 47 months, Harrison et al. [30] found a 26% recurrence rate in all cases, which is comparable to the 27% recurrence rate in our series of radical wide excision [16].

Standard procedure

Excision of the involved areas down to soft, normal tissue with clear margins of at least 1 cm in the clinically unaffected skin under general anesthesia.

Contraindications

Patient must tolerate general anesthesia, postoperative immobilization, and long-term follow-up.

Complications

Acceptable recurrence rate. Contracture (1%-2%), bleeding, long-term wound healing, vascular/nerve injury.

Skin closure

Controversy exists regarding closure of the skin defect. Primary closure and rotation flaps are rarely used because of the extensive nature of the excisions. The method of wound management may influence the course of the disease. In a surgical series, reoperation was necessary in 54% of the cases with primary wound closure, in 19% after flap repair, and in 13% after grafting [29]. This confirms our experience, which indicated that primary skin closure may inadvertently compromise the excision margin and increase the risk of recurrence. Immediate or delayed split-skin grafting offers the advantage of rapid healing with complete wound closure in 2 to 4 weeks. Grafting is most successful in axillary disease. Healing by granulation is associated with a predictable result that is as good as or superior to that obtained by skin grafting. However, complete wound closure may take up to 2 to 3 months [31].

Standard procedure

Open wound healing by granulation in affected areas. Delayed skin grafting in selected patients with axillary HS 1 to 2 weeks after excision to avoid missing infected skin not completely excised.

Contraindication

Grafting in perianal, anal, or perineal affection of HS.

Complications

Unsightly cosmetic results and poor cosmesis at the donor site. Immobilization of the affected limb for 5 to 7 days. High failure rate in the perianal and perineal area. Grafts in the anal area may contract and lead to anal stenosis.

Radiotherapy

X-irradiation in doses sufficient to produce temporary epilation (single doses of 0.5 to 1.5 Gy up to total doses of 3.0 to 8.0 Gy) appears to be less effective than the approaches cited previously [32].

Lifestyle factors and prevention

An essential part of treatment includes avoidance of tight garments such as T-shirts, body-shirts, snug-fitting blouses, blue jeans, panty hose, and the straps and seams of undergarments and athletic equipment, all of which produce frictional trauma and exacerbate the disease. Whenever possible, women may have to forego wearing a brassiere in favor of a cotton undershirt. The patients must maintain this approach throughout their lives to sustain a quiescent state.

Generally, the local application of adhesive tape, which can promote poral occlusion and is irritative, should be avoided in these patients or used for a limited time. Shaving of regional hair, as in the axillae, is permissible but must be done gently and carefully with a clean sharp razor with prior cleansing of the area. Antiperspirant-deodorant formulations, preferably the liquid or spray varieties (not the roll-on, mat applicators, or stick types) may be used in the axillae. Several reports found a clear correlation between HS and nicotine intake, thus patients should quit smoking
 

Celery Peach

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Re:Hidradenitis Suppurativa Articles
« Reply #17 on: Monday March 08, 2004, 04:10:32 PM »
Combination treatment using Clindamycin & Rifampicin

E.A.O Hindle, B.Kirby & C.E.M. Griffiths
Hope Hospital, Salford, UK - Published in the British Journal of Dermatology 2002

Combination treatment with clindamycin 300 mg twice daily and rifampicin 300 mg twice daily for 10 weeks has previously been reported as successful in folliculitis decalvans.

We report our experience with this regimen in seven patients with HS and three patients with acne keloidalis nuchae. A 10 week course of both agents was initiated in all patients, with liver function monitoring at baseline and during therapy. Four patients with HS tolerated the combination, and three of these responded well: one patient remained clear for 12 months, one reprted a 70% improvement and the third patient has not attended for further review following a good initial response.

Three Patients with HS were unable to tolerate the combination due to diarrhoea, nausea & vomiting. Clindamycin was discontinued in the two patients who experienced diarrhoea due to the association with Clostridium difficile, and minocycline was substituted. One of these patients subsequently developed raised liver aminotransferases, and rifampicilin was discontinued.

All three patients with acne keloidailis nuchae responded completely and this has been maintained for up to 26 months without relapse.

We conclude that this treatment appears effective in these shronic and usually difficult to treat conditions and thus represents a significant theraputic advance.


Offline missmash

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Re:Hidradenitis Suppurativa Articles
« Reply #18 on: Tuesday March 30, 2004, 02:11:54 PM »
Lapins, Jan
Hidradenitis suppurativa: With special reference to carbon dioxide laser surgery

Fredagen den 4 maj 2001, kl. 10.00.
Föreläsningssal I 42, Huddinge Universitetssjukhus.
 
ISBN: 91-628-4722-8     Diss: 01:227  


Abstract:

Hidradenitis suppurativa (HS) is a chronic inflammatory disease with recurrent abscesses. In most cases, it involves the axillar and anogenital regions. In industrialised countries, the prevalence is of 0.3% to 4%, and the disease is over-represented in young adult females. Various medical treatments have been used but they are seldom effective. Surgical treatment is recommended as soon as the condition is diagnosed, but wide excisions very well outside the clinical borders of activity, are mandatory. A simple local incision is of no value. According to Hurley's clinical classification, stage I consists of one or more abscesses with no sinus tract and cicatrisation and stage II consists of one or more widely separated recurrent abscesses, with a tract and cicatrisation. The severest cases (stage III) have multiple interconnected tracts and abscesses throughout an entire area.

    In this thesis we report a method in which horisontal vaporisation with carbon dioxide laser is used to remove the inflamed infiltrating abscesses with precision. Patients classified as Hurley stage H were selected consecutively, in 1989 and by the year 2000 that had undergone this treatment. Most patients were females, in their twenties and thirties and had had the disease for a mean of more than ten years. By using a radical but selective, tissue-sparing technique, we removed the inflamed, foreign body-like tissues of HS, including its squamous epitheliumlined and keratin- containing sinuses. Initially, we used a free hand and more surgeondependent technique that was later abandoned for a scanner assisted technique providing better accuracy, safety and faster ablation. The use of these carbon dioxide laser, rapid beam, optomechanical scanner systems in a continuous mode gives a fast and even ablation with better visualisation of the macro- pathology during surgery. The surgical results were satisfactory cosmetically, functionally and as regards quality of life. We believe that this technique offers a safe and efficient strategy for many colleagues who treat HS.

    Squamous cell carcinoma is a rare but serious complication of HS. To investigate this association and the risk of other malignancies, we performed a population-based retrospective cohort study on 2119 HS patients selected from a computerised database of hospital discharge diagnoses in Sweden during 1965-1997. We found a significantly increased risk of non- melanoma skin cancer in patients with HS. The risks of buccal cancer and primary liver cancer were also higher.

    Aerobic and anaerobic cultures from superficial and deep levels were taken during laser surgery in 24 patients. In all cases, bacterial cultures were positive for one or more specimens from at least one level and from deep levels in all but three cases. Sixteen species or subspecies were found. Staphylococcus OBS aureus OBS and coagulase-negative OBS staphylococci OBS (CNS) were the most frequently found species which suggests that CNS is a true pathogen.

    In our comparison of forty-two unrelated Swedish patients with HS and 250 controls we found no association with HLA-A, -B or -DRB1 alleles using the genetic tissue typing technique. Genetic factors associated with the HLA class I or II regions do not seem to contribute significantly to the possible genetic susceptibility of HS.

    We also compared the release of oxygen radicals and of primary granula from in vitro activated peripheral neutrophils from HS patients with inactive disease to that from a group of healthy controls. Our findings suggest that dysfunctional neutrophils may be involved in the pathogenesis of hidradenitis suppurativa.
The greatest danger for most of us is not that our aim is too high and we miss it, but it is too low and we reach it...  michelangelo

Celery Peach

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Re:Hidradenitis Suppurativa Articles
« Reply #19 on: Tuesday March 30, 2004, 06:13:59 PM »
HS Article written March 2004 by Prof Jean Revuz

Needs adobe reader:

http://www.orpha.net/data/patho/GB/uk-hidradenitis-suppurativa.pdf