The role of radiotherapy in the treatment of hidradenitis suppurativa: Case report and review of the literature
Mark Trombetta MD, E Day Werts PhD, David Parda MD
Dermatology Online Journal 16 (2): 16

Department of Radiation Oncology, Allegheny General Hospital, Pittsburgh, Pennsylvania. mtrombet@wpahs.org
Drexel University College of Medicine, Allegheny Campus, Pittsburgh, Pennsylvania



I. Introduction

Hidradenitis Suppurativa (HS) is a chronic condition that can be debilitating for patients. Standard therapy ranges from improving skin hygiene to antibiotic therapy to radical surgery. In patients for whom standard therapy is ineffective there are few options. We reviewed the literature and identified only rare anecdotal experience with external irradiation in such patients. In the report, we describe a case of HS, refractory to conventional therapy and multiple radical surgeries, which was finally successfully treated with external irradiation.


II. Case report

A 53-year-old white female with a 5 to 6 year history of HS presented to the radiation oncology department for evaluation. Since her diagnosis of HS, originally affecting only the bilateral inguino-femoral regions, the patient had been treated for several years with conservative conventional therapy including improved hygiene counseling and antibiotic therapy. Two years prior to this consultation, the disease became refractory to conventional antibiotic management and progressed to involve the bilateral axillae and the perianal region. Finally, surgical resection of all sites was performed.

Microscopic pathology was consistent with HS. Following a quiescent period of only 9 months, the disease recurred in all sites and surgical re-excision of all sites was performed. Antibiotic administration with multiple regimens once again failed to improve the clinical symptoms. Three months following the last surgical procedure, the disease re-presented and the patient was sent for a radiation consultation.

Her past medical history was significant hypertension, a superficial melanoma of the right lower extremity (successfully treated by wide local excision 5 years earlier), hypothyroidism, hyperlipidemia, and chronic anxiety and depression. Her family history included basal cell cancer in one daughter before the age of 40, “liver and brain” cancer in her father, and an unknown type of “skin” cancer in her mother. There was no family history of HS. Her medications included wellbutrin, lithium, spironolactone, risperdal, lipitor, synthroid, and xanax. She had a history of allergy to an unknown anti-depressant. On examination her weight was 145 Kg., blood pressure 142/90, pulse 88, and respirations 16. Her height was 154.4 cm. Her Eastern Cooperative Oncology Group (ECOG) performance score was 1. Initial examination revealed a moderately obese woman in moderate discomfort at all affected sites. Bilateral 10 cm axillary incisions were apparent with multi-focal purulent drainage sites along each incision. Findings in the bilateral inguino-femoral regions were similar to those noted in the axillae.

To the best of our knowledge the use of radiotherapy in refractory cases of HS has not been reported in the American literature and there was only a single reference in the European literature. Our patient had no reasonable alternatives to therapy. After extensive discussion with the patient about the paucity of literature on this subject, she agreed to proceed with low dose radiotherapy. External beam radiotherapy was directed to all sites utilizing 6 MeV electrons prescribed to the 90 percent focus of the electron curve. A 5mm tissue equivalent bolus was added to the skin surface and a 1.0 cm margin around the visible disease was added to the Planning Treatment Volume (PTV). A total of 450 cGy at 150 cGy per fraction was delivered over 2 elapsed days. Three weeks following radiotherapy, marked improvement was seen in all areas. However, persistent sub-centimeter punctuate foci of disease were evident in the left groin and an additional 600 cGy was prescribed at 200 cGy per fraction utilizing the same treatment parameters as before. The patient tolerated the procedures well without sequelae and one month following treatment, all areas of disease were clinically resolved. Three months later, she presented with 2 new sub-centimeter lesions, one in the left proximal medial thigh and one in the right perineal region. Two additional recurrent lesions were identified, one in each groin, but at different locations from the original boost areas. Additional irradiation at 200 cGy per fraction to 600 cGy was delivered to the previously treated areas. To the new areas, the dose was increased to 750 cGy at 250 cGy per fraction. Once again, the electron energy, field parameters and prescription depth were similar. All treatment was delivered over 2 elapsed days. She manifested no acute side effects and two weeks following the radiotherapy all lesions had resolved. Following the conclusion of radiotherapy, the patient began to follow a low-fat diet and has lost 45 Kg over 1.5 years time. At the time of this report she is now 2.75 years from treatment and has had no recurrence of lesions. She has not developed any chronic side effect.


III. Discussion

Hidradenitis Suppurativa is a debilitating disease that affects approximately 1 percent of the population [1], although a Danish study group demonstrated a 4 percent rate specifically among women [2]. The disease was first described in1864 by the French surgeon, Verneuil as an inflammatory condition of the apocrine glands and classified as Verneuil's Disease [3]. Wilson noted early on that the disease was exacerbated by poor hygiene [4]. More recently, it has been observed that the disease may arise from the hair follicles [5]. Sellheyer performed a meticulous review of 176 pathologic specimens at different phases of the disease and suggested that the term HA should be abandoned in favor of Acne Inversa [6], although this designation has not been universally adopted.


III.A. Disease presentation

The diagnosis is made both clinically and pathologically. The clinical presentation involves the apocrine-gland bearing areas of the skin. A hallmark of the clinical disease is follicular plugging and inflammatory pustules, which become suppurative and causes severe cicatrization and sinus tracking of the affected areas. The inflammatory changes are accompanied by severe pain and localized discomfort and can severely impact the quality of life of those affected. The disease rarely presents prior to puberty, seemingly exacerbated by the pubescent hormonal changes. It is most prevalent in females (3:1) usually in the second or third decade of life. Obesity and tobacco use have been implicated as potential risk factors [1] and some authors have suggested a familial genetic predisposition with up to 38 percent of patients having a relative affected by the disease [7]. Recently, immunosupression secondary to chemotherapy has been implicated in the etiology of this disease [8, 9]. Sites of involvement are commonly the inguino-femoral regions (most common), the axillae, the areola, the perineum, the perianal area, and the peri-umbilical region. Chronic persistence after conservative local treatment and recurrence after “successful” treatment are characteristic of HS. Patients affected by HS are commonly affected by cervico-facial acne and folliculitis of the scalp. The term “follicular occlusion triad” has been used to describe this [10]. The differential diagnosis of HS is outlined in Table 1. Sequelae of the disease can be severe. Severe disfigurement, cicatrization with resulting distal lymphedema , acute hypocalcaemia, and even the development of squamous cell carcinoma in the affected areas have all been reported [11].

Microbiologic cultures will frequently grow a multi-organism yield predominated by staphylococci and streptococci, but saprophytes are also frequently noted. Hidradenitis suppurativa is a progressive disease and is graded by the Hurley system (Table 2) [12]. Some authors have suggested that soluble Interleukin-2 receptor serum levels may be a useful marker in the staging of HS [13].


III.B. Treatment

Improvement in local hygiene is helpful, but usually not curative; therefore broad spectrum antibiotic coverage is required. Tetracycline, erythromycin, or cephalosporins are the usual antibiotics of choice. Cultures should always be taken to identify the pathogens and rule out the presence of methicillin-resistant Staphylococcus aureus (MRSA). Topical antibiotic or retinoic creams are rarely effective. Systemic retinoids (Accutane®) offer only marginal benefit.

A large proportion of patients will fail conservative antibiotic therapy and the mainstay of salvage therapy in this circumstance is surgical resection with or without graft placement [14, 15]. Even with surgical resection, recurrences can be seen in up to 50 percent of patients. The use of laser therapy has significantly improved the local control rate with up to 73 percent of patients treated showing symptomatic improvement [16]; one study demonstrated a local failure rate of only 14 percent [17]. Unfortunately, distal lymphedema often results from surgical therapy.


III.C. The use of radiotherapy

Still, there is a significant failure rate associated with this disease and patients who fail all other therapies have limited options. Our patient fit this group. We offered her radiotherapy in lieu of continuing on with persistent disease. To the best of our knowledge there are only two documented reports of the use of radiotherapy for HS in the modern literature. Frölich et al., have the largest reported experience over more than 20 years [18]. In their report, 231 patients who had failed other therapies were offered radiation. Multiple fraction schedules were used with multiple fraction sizes (0.5 Gy - 1.5 Gy per fraction to total doses of 3.0 Gy to 8.0 Gy). For chronic recurrences, total doses in excess of 10.0 Gy were delivered. No patients developed acute side effects. Complete symptomatic relief was noted in 38 percent of patients at the end of therapy and 40 percent of patients overall had improvement in symptomatology. Two patients demonstrated no response. Our patient had a more protracted improvement, but her response was durable. As her weight loss occurred months following radiotherapy, we believe that her response was due to irradiation. We also believe that her significant weight loss from a dedicated dietary management program has helped to prevent recurrences.

As the use of radiation in non-malignant diseases may be mutagenic [19, 20]; care should be taken to select patients well and minimize the volume of treatment in patients so selected.


Conclusions

Conservative management of HS with oral antibiotic therapy and a strict weight loss regimen is an optimal first line approach. However, when more radical and invasive surgical options fail, radiotherapy is a viable option. We recommend doses of 7.5 Gy at 2.5 Gy per fraction. Caution should be exercised when offering this therapy to younger patients.

References

1. Jemec GB, Revuz J, Leyden JJ., eds. Hidradenitis Suppurativa, Berlin-Heidelberg, Springer Verlag, 2006.

2. Jemec GB: The symptomatology of hidradenitis suppurativa in women. Br J Dermatol. Sep 1988; 119(3): 345-50. [PubMed]

3. Verneuil A: De L'hidrosadenite phlegmoneuse et des abces sudorpares. Arch Gen Med 2:537-557, 1864.

4. Wilson E: On diseases of the skin. A system of cutaneous medicine. John Churchill, London, 1867, pp 808-809.

5. Yu CC, Cook MG: Hidradenitis suppurativa: A disease of the follicular epithelium, rather than the apocrine glands. Br J Dermatol. Jul 2006; 122 (6); 763-69. [PubMed]

6. Sellheyer K, Krahl D: Hidradenitis suppurativa is acne inversa! An appeal to finally abandon a misnomer. Int J Dermatol. 2005; 44:535. [PubMed]

7. von der Werth JM, Willaims HC: The natural history of hidradenitis suppurativa. J Eur Acad Dermatol Venereol. Sep 2000; 14(5):389-92. [PubMed]

8. Koppel RA, Boh EE. Cutaneous reactions to chemotherapeutic agents. Am J Med Sci. May 2001; 321 (5); 327-35. [PubMed]

9. Guillot B, Bessis D, Deneure O. Mucocutaneous side effects of antineoplastic chemotherapy. Ex Opin Drug Saf. Nov 2004; 3 (6); 579-87. [PubMed]

10. Chicarilli ZN. Follicular occlusion triad: hidradenitis suppurativa, acne conglobata, and dissecting cellulitis of the scalp. Ann Plast Surg. 1987; 18 (3):230-7. [PubMed]

11. Miquel J, Adamski H, Faujour G, Dinulescu M, Le Gall F. Acute hypercalcemia and multiple squamous cell carcinomas arising in hidradenitis suppurativa. Presse Med. Jul-Aug 2009; 38 (7-8); 1177-80. [PubMed]

12. Hurley HJ: Axillary hyperhidrosis, apocrine bromhidrosis, hidradenitis suppurativa and familial pemphigus. Surgical approach. In: Roenigk RK, Roenigk HH Jr. Dermatologic Surgery. Principles and Practice. Marcel Dekker, New York; 1996.

13. Matusiak L, Bieniek A, Szepietowski J: Soluble interleukin-2 receptor serum level is a useful marker of hidradenitis suppurativa clinical staging. Biomarkers. Sept 2009; 14 (6): 432-37. [PubMed]

14. Slade DE, Powell BW, Mortimer PS. Hidradenitis suppurativa: pathogenesis and management. Br J Plast Surg. 2003; 56 (5): 451-61. [PubMed]

15. Parks RW, Parks TG. Pathogenesis, clinical features and management of hidradenitis suppurativa. Ann R Coll Surg Engl. Mar 1997; 79 (2): 83-9. [PubMed]

16. Tierney E, Mahmoud BH, Hexsel C, Ozog D, Hamzavi I. Randomized control trial for the treatment of hidradenitis suppurativa with a neodymium-doped yttrium aluminum garnet laser. Derm Surg. August 2009;35 (8); 1188-98. [PubMed]

17. Lapins J, Sartorius K, Emtestam L. Scanner assisted carbon dioxide laser surgery: A retrospective follow-up study of patients with hidradenitis suppurativa. J Am Acad Derm. August 2002; 47 (2); 280-85. [PubMed]

18. Frölich D, Baaske D, Glatzel M. Radiotherapy of hidradenitis suppurativa - still valid today? Strahlenther Onkol June 2000; 176 (6); 286-9. [PubMed]

19. Bhatia S, Yasui Y, Robison LL. High risk of subsequent neoplasms continues with extended follow-up of childhood Hodgkin's disease: report from the Late Effects Study Group. J Clin Oncol. 2003; 21: 4386-94. [PubMed]

20. Schneider AB, Shore-Freedman E, Ryo UY, et al. Radiation induced tumors of the head and neck following childhood irradiation. Medicine (Baltimore). 1985 Jan; 64 (1):1-15. [PubMed]

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Title:

The role of radiotherapy in the treatment of hidradenitis suppurativa: Case report and review of the literature

Journal Issue:

Dermatology Online Journal, 16(2)

Author:

Trombetta, Mark;
Werts, E Day;
Parda, David

Publication Date:

2010

Publication Info:

Dermatology Online Journal, UC Davis

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