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Severe hidradenitis suppurativa


Těžká hidradenitis suppurativa

Úvod:
Hidradenitis suppurativa je chronické, recidivující zánětlivé onemocnění způsobující rozsáhlé změny a poškození kůže. Axila a anogenitální oblast jsou predilekčními místy. Těžké formy onemocnění s chronickým průběhem mohou způsobit závažné fyzické a psychické poškození pacienta.

Kazuistika:
V prezentaci demonstrujeme dvě pacientky s těžkou formou hidradenitis supurativa postihující axilu a anogenitální oblast. Pacientky byly neúspěšně léčeny osm, respektive čtyři roky antibiotiky ve spojení s limitovanými chirurgickým zákroky. Uzávěr defektu po nezbytné radikální chirurgické excizi si vyžádal různé postupy, což vyplývalo z rozsahu a lokalizace poškozené kůže.

Závěr:
Pouze radikální široká excize postižené kůže je vhodný chirurgický postup, které zabrání recidivě onemocnění. Výběr léčebných postupů vedoucích k uzávěru defektu musí být proveden velmi pečlivě, s ohledem na rozsah a individuální charakter onemocnění.

Klíčová slova:
hidradenitis supurativa – axila − anogenitální oblast − chirurgická terapie


Authors: J. Gatěk 1 ;  B. Dudesek 1;  A. Kratka 1;  D. Vrána 2;  J. Duben 1
Authors place of work: Department of Surgery Hospital Atlas Zlin, University of Tomas Bata in Zlin Head of the Department: MUDr. J. Gatěk Ph. D 1;  Department of Oncology, Faculty of Medicine and Dentistry, Palacky University Olomouc Head of the Department: prof. MUDr. B. Melichar Ph. D 2
Published in the journal: Rozhl. Chir., 2014, roč. 93, č. 9, s. 468-471.
Category: Case Report

Summary

Introduction:
Hidradenitis suppurativa is a chronic, recurrent inflammatory disease causing significant changes and damage to skin. Predilection sites are the axilla and the anogenital region. Chronic and severe forms of the diseases may cause both physical and psychological injury to the patient.

Patients:
We present the cases of two female patients with severe hidradenitis suppurativa in the axillae and in the anogenital region. The patients had been unsuccessfully treated for eight and four years respectively with antibiotics and by limited surgery. Different methods of closing the defects following radical surgical excision (local flap reconstruction, healing by granulation) had to be used with respect to the extent of the disease and the site of involved skin.

Conclusion:
Radical wide excision of the skin area involved is the only appropriate surgical procedure which prevents recurrence of the disease. Selection of the therapeutic method must be done very carefully with respect to the individual characteristics of the disease and the patient’s attitude to therapy.

Key words:
hidradenitis suppurativa − axilla − anogenital region − surgery

Introduction 

The term hidradenitis suppurativa inaccurately refers to a disease having its origin in the sweat glands. The term hidrosadénite phlegmoneuse was coined in 1865 by French surgeon Verneuil who attributed the process to inflammation of the sweat glands [1]. The exact pathogenesis of hidradenitis still remains unclear. However, the most widely accepted hypothesis is that the disease starts with occlusion of the follicular ducts. Stasis of the secretion causes dilatation and ensuing bacterial infection with subsequent involvement of the apocrine glands [2,3]. As hair follicles are the primary site of the process, the term acne inversa is more appropriate for the disease. In spite of the pathogenesis, the name hidradenitis suppurativa is used more frequently [4].

Hidradenitis suppurativa is a chronic, recurrent, inflammatory disease presenting sinus tracts with abscesses, fibrosis and scarring process in the affected region. [5] Female smokers with a higher body mass index are at a higher risk [6,7,8]. The predominantly affected sites are the axilla and the anogenital region; however, other areas may be involved too [9].

Conservative therapies with antibiotics and local surgical procedures such as incision, decission and deroofing of the sinus tract have only a short-time effect and recurrences are very frequent. Most authors agree that radical wide excision of the affected skin is the best way to cure the disease and reduces the recurrence rate to a minimum [10,11]. Many methods of wound closure have been described in relation to severe hidradenitis suppurativa treatment. However, wound closure may be difficult in some cases, especially in the anogenital area [4,11,12,13,14]. An appropriate surgical method must be carefully selected with respect to the individual characteristics of the disease, the location of the affected area and also the psychological status of the patient. The aim of the study is to present two cases treated by different surgical procedures, focusing also on the treatment results.

Case report 1  

A 38-year-old woman was admitted to the Department of Surgery at the Atlas Hospital in Zlin with severe hidradenitis in both axillae and the anogenital region. The woman’s history was without serious disease, her body mass index was high and she was a heavy smoker. Several unsuccessful attempts at treatment had been made in the last eight years at different institutions using antibiotics and limited surgery such as discission, deroofing and drainage of the abscesses. Intensive local therapy was needed due to the extent of involved tissue with massive foul-smelling purulent secretion. This resulted in the exclusion of the patient from work and social life and her marriage fell apart. Bacterial culture revealed Streptococcus beta haemolyticus, Proteus mirabilis and Staphylococcus aureus without any special resistance to antibiotics. Our therapy started with antibiotics and simultaneously with radical excision of all involved tissue in the axillae. Primary closure was not performed because of acute inflammation. After two months of local therapy, local flaps were used in both axillae. Partial dehiscence complicated healing but therapy in the axial regions was completed after four months. There were no recurrences. In the anogenital region, we had to change the treatment strategy because the area involved was too extensive and included the genitalia (labia major and minor) and the anus (periproctal fistula). Use of flaps and also grafting was practically impossible in this situation. Therefore, we preferred healing by granulation after radical excision of all the inflammatory tissue. Colostomy was performed first, followed by excision of sinus tracts, abscesses and all involved skin in several steps. The periproctal fistula was cured by the seton. Unfortunately, colostomy was complicated by parastomal hernia. The colostomy was closed after definitive healing of all wounds when there was no risk of recurrence. The total duration of therapy was three years and it was very demanding for both sides. The woman has had no recurrence and she is very satisfied with the result. She has found a boyfriend and has been able to return to sexual life (Fig. 1,2,3,4).

Fig. 1: Both axillae before and after radical excision
Fig. 1: Both axillae before and after radical excision

Fig. 2: Axilla after local flap repair and final result
Fig. 2: Axilla after local flap repair and final result

Fig. 3: Anogenital region before radical excision, after excision, granulation, and final result
Fig. 3: Anogenital region before radical excision, after excision, granulation, and final result

Fig. 4: Diverting colostomy, parastomal hernia, colostomy closing and hernia reconstruction
Fig. 4: Diverting colostomy, parastomal hernia, colostomy closing and hernia reconstruction

Case report 2 

A 52-year-old woman was admitted with hidradenitis in the left axilla. Her history was also without conspicuousness, her body mass index was higher and she was a smoker, too. The patient had been treated unsuccessfully for four years at different institutions. She had undergone several conservative surgeries with antibiotics resulting in recurrences. The whole axilla was macerated with many abscesses and sinus tracts. Bacterial culture revealed Escherichia coli and Streptococcus agalactiae. Foul-smelling purulent secretion required continual local care which excluded the patient from personal and social life. She was admitted at the Atlas hospital and a radical excision of the affected area under antibiotic therapy was performed. We tried to reconstruct the extensive defect by primary closure but it was not successful. In spite of the relative sufficiency of the skin in the axilla, the tension on the suture was too high. Local therapy induced granulation after dehiscence and we prepared conditions for the second attempt at closing the defect. One month later, local flap reconstruction was performed. The procedure was very easy because there was enough skin tissue in the axilla and therefore the wound healed without complications (Fig. 5).

Fig. 5: Axilla before radical excision, after excision, local flap repair a final result
Fig. 5: Axilla before radical excision, after excision, local flap repair a final result

Discussion

Hidradenitis suppurativa is a chronic, recurrent inflammatory disease with extensive changes and damages to the skin in the involved areas. Chronic long-term disorders cause physical and psychological injuries to the patient; especially in severe cases. The changes due to this medical condition may be so severe that it may exclude the patients from the community and cause job loss. It is remarkable that most cases are still treated conservatively for many years without any effects and radical surgery is refused or postponed [15]. Initially, the disease may be underestimated. Conservative therapy with antibiotics, retinoids and limited surgery may last for months or years. Both our patients represent typical cases and they had been treated for eight and four years before radical surgery. The patient with affected axillary and anogenital regions was in terrible mental condition. She had lost hope to be cured. It was therefore very difficult to convince her to undergo radical therapy. She changed her mind after successful therapy of the axilla. Nevertheless, every step had to be discussed in detail. The other patient was in a rather different mental condition. She was not so depressed, she tried to find the best medical institution for her therapy and she was ready to undergo any procedure.

The exact etiology of hidradenitis is unknown. Some publications have presented a higher prevalence among heavy cigarette smokers. Increasing severity of the disease correlates with smoking intensity [6,7]. Both our patients were heavy smokers and also had a high body mass index. Obesity is another factor which may play a role in hidradenitis etiology [8].

Hidradenitis in its early stages is mostly treated conservatively with antibiotics, hormonal therapy, retinoids, immunosuppression and limited surgery. Results of therapy with hormones, retinoids and immunosuppression are less encouraging and further trials are needed to prove efficiency [16,17,18,19,20]. Although conservative therapy may bring temporary relief, relapse is very frequent and occurs in up to 100% of cases. Simple incision, drainage, deroofing and marsupialization are used in the acute stages of the disease but complete surgical excision of sinus tracts, fistulas and all involved areas is the only effective procedure [10,21,22]. Radical therapy reduces the probability of recurrence. There is a controversy about the optimal surgical procedure, especially as regards closure of the defect [23]. The choice of surgical procedure depends on the extent and location of the defect. Acute or chronic status at the time of presentation also influences the therapeutic plan. Primary closure, various skin flaps, grafting and healing by granulation are the most utilized methods of closing the defect. [4,11,12,14,15,22,23,24]. Each method has its advantages and drawbacks and should be selected with regard to the real status of the disease. Healing by granulation may have a very good ultimate cosmetic effect with patient’s satisfaction and sometimes the results may be better than in grafting [11,24]. Grafting and local flaps are usually not suitable in the anogenital region [21]. In both our cases we had to select a different method of reconstruction with respect to local status of the involved area. Reconstruction with local flaps in the axilla is easier than in the anogenital region and so it was used after deep excision of the involved skin. Skin inflammation and destruction in the anogenital region was complicated by fistulas leading into the vagina and to anal tissue with typical periproctal fistula [25]. With regard to to the extent of inflammation, we decided for excision of all tracts, fistulas and healing by granulation. Colostomy made local treatment much easier and more comfortable for the patient [26]. The mental condition of the patient improved dramatically after a short time because she began to believe in being cured. Healing by granulation had very encouraging results and patient satisfaction was high, but it was time consuming. This type of therapy requires patience on both sides.

Conclusion

Only early diagnosis followed by radical surgery enables successful therapy without recurrence. Selection of the most appropriate therapeutic procedure must be individual, depending on the location of the disease and attitude of the patient. Therapy with local flap reconstruction is the most effective. In situations where it cannot be used, healing by granulation is a good alternative. An important condition for successful cure is extreme patience and mutual trust between the patient and the surgeon.

MUDr. Jiří Gatěk PhD

Fügnerovo nábřeží 5476

760 01 Zlín

e-mail: gatekj@gmail.com


Zdroje

1. l. Verneuil A. De l´hidrosadénite phlegmoneuse et des abscés sudoripares. Arch Gén Méd 1864;2:537−557.

2. Attanoos RL, Appleton MA, Douglas –Jones AG. The pathogenesis of hidradenitis suppurativa: a closer look at apocrine and apoeccrine glands. Br J Dermatol 1995;133: 254−258.

3. Parks RW, Parks TG. Pathogenesis, clinical features and management of hidradenitis suppurativa. Ann R Coll Surg Engl 1997;79:83−89.

4. Rompel R, Petres J. Long-term results of wide surgical excision in 106 patients with hidradenitis suppurativa. Dermatol Surg 2000;26:638−643.

5. Brown TJ, Rosen T, Orengo IF et al. Hidrosadenitis suppurativa. South Med J 1998;91:1107−1114.

6. Wiltz O, Schoetz DJ Jr., Murray JJ, et al. Perianal hidradenitis suppurativa. The Lahey Clinic experience. Dis Colon Rectum 1990;33:731−734.

7. König A, Lehman C, Rompel R, et al. Cigarette smoking as a triggering factor of hidradenitis suppurativa. Dermatology 1999;198:261−4.

8. Endlich RF, Silloway KA, Rodehaver GT, et al. Epidemiology, pathology, and treatment of axillary hidradenitis suppurativa. J Emerg Med 1986;4:369−378.

9. Mortimer PS, Lunniss PJ. Hidradenitis suppurativa. J R Soc Med 2000;93:420−422.

10. Jemec GB. Effect of localized surgical excisions in hidradenitis suppurativa. J Am Acad Dermatol 1988;18:1103−1107.

11. Kuo HW, Ohara K. Surgical treatment of chronic gluteal hidradenitis suppurativa: reused skin graft technique. Dermatol Surg 2003;29:173−178.

12. Morgan W, Harding K, Hughes LA. Comparison of skin grafting and healing by granulation, following axillary excision for hidradenitis suppurativa. Ann Royal Coll Surg Engl 1983;65:235−236.

13. Bienek A, Matusiak L, Okulewicz D, Szepietowski J. Surgical treatment of hidradenitis suppurativa: Experiences and Recommendations. Dermatol Surg 2010;36:1998−2004.

14. Kishi K, Nakajima H, Imanishi N Reconstruction of skin defect after resection of severe gluteal hidradenitis suppurativa with fasciocutaneus flaps. J Plast Reconstr and Aesthet Surg 2009;62:800−805.

15. Parks RW, Parks TG. Pathogenesis, clinical features and management of hidradenitis suppurativa. Ann R Coll Surg Engl 1997;79:83−89.

16. Ather S, Chan D, Leaper D, Harding K. Surgical treatment of hidradenitis suppurativa: case series and review of the literature Int Wound J 2006;3:159−169.

17. Slade DM, Powel BW, Mortimer PS. Hidradenitis suppurativa: pathogenesis and management. Br J Plast Surg 2003;56:451−461.

18. van der Zee HH, Orens EP, Boer J. Deroofing: A tissue-saving surgical technique for the treatment of mild to moderate hidradenitis suppurativa laesions. J Am Acad Dermatol 2010;63:475−80.

19. Alikhan A, Lynch PJ, Eisen DB. Hidradenitis suppurativa: A comprehensive review. J Am Accad Dermatology 2009;60:539−61.

20. Krebs AC. Current understanding and management of hidradenitis superlative. J Am Accad Nurse Pact 2007;19:228−234.

21. Rubin RJ, Chinn BT. Perianal hidradenitis suppurativa. Surg Clin North Am 1994;74:1317−1325.

22. Ritz JP, Runkel N, Haier J, et al. Extend of surgery and recurrence rate of hidradenitis suppurativa. Int J Colorectal Dis 1998;13:164−8.

23. Ramasastry SS, Conklin WT, Granick MS, et al. Surgical Management of massive perianal hidradenitis suppurativa. Ann Plast Surg 1985;15:218−223.

24. Weyandt G. Operative Therapie der Acne inversa. Der Hautarzt 2005;56:1033−1039.

25. Culp CE. Chronic hidrosadenitis suppurativa of the anal canal a surgical skin disease. Dis Colon Rectum 1983;26:669−676.

26. Church JM, Fazio W, Lavery, et al. The differential diagnosis and comorbidity of hidradenitis suppurativa and perianal Crohn´s disease. Int J Colorectal Dis 1993;8:117−119.

Štítky
Surgery Orthopaedics Trauma surgery
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