British Journal of Dermatology (1990) 123, 527-531. Squamous cell carcinoma complicating hidradenitis suppurativa A.V.ANSTEY, J.D.WILKINSON AND P.LORD* Departments of Dermatology and "Surgery, Wycombc General Hospital, High Wycombe, Bucks, U.K. Accepted for publication i May 1990 SUMMARY We describe a patient who suffered over 40 years from persistent, extensive hidradenitis suppurativa (HS) and who developed squamous cell carcinomas at two different sites. Hidradenitis suppurativa (HS) most commonly affects the skin of the groin and perineum, axillae and less commonly the buttocks and upper thighs. It results infibrosis,sinus formation and scarring. In common with other chronic inflammatory skin conditions, it is rarely complicated by squamous cell carcinoma (SCC). So far there arc 15 such cases reported in the literature. CASE REPORT A 67-year-old Caucasian man was referred to us with a 40-ycar history of multiple discharging sinuses involving the buttocks and groins. His skin condition had deteriorated markedly over the previous 12 months. Physical examination revealed an obese man of 95 kg with severe HS involving the buttocks, and perianal and perineal skin, extending onto the upper inner thighs and inguinal skin. There were multiple abscesses and suppurating, discharging sinuses as well as extensive scar tissue from healed lesions. Investigations Hacmatological investigations revealed a mild iron deficiency anaemia with a haemoglobin of 10 2 g/dl, MCV of 67 fi and an iron deficient picture on the blood film. The white cell count ranged between 13-1 and 15-6 x io**/!, usually with a moderately raised neutrophil count. The erythrocyte sedimentation rate was elevated from 81 to 90 mm/h. Renal and liver function tests were normal. Immunoglobulins were diffusely raised with IgA 11 4 g/1, IgG 24 g/1 and IgM i 6 g/i. Complement studies revealed a low C4 at o 13 g/1 with a normal C3 at o 90 g/1. Rheumatoid factor {RA latex) was positive at i in 8. Correspondence: Dr A.V. Anstey, Department of Dermatology, Wycombe General Hospital, High Wycombe, Bucks HPii 2TT, U.K. 527
528 A.V.Anstey et al. FIGURE I. Biopsy of sacral ulcer showing infiltrating, well to moderately differentiated squamous cell carcinoma (Haematoxyiin and eosin x 132). Microbiology. Skin swabs taken from suppurating lesions during the first 6 months cultured five main pathogenic organisms: anaerobic Streptococcus, Proprionobacterium acnes, Bacteroides corrodens, Bacteroides melaninogenicus and Bacteroides fragilis. All five organisms were sensitive to metronidazole and amoxycillin with clavulanic acid. Histology. A skin biopsy taken from the inguinal and buttock hidradenitis showed a heavy mixed inflammatory reaction in the dermis extending to the subcutaneous tissues. Occasional foreign body giant cells were present and the findings were compatible with the clinical diagnosis of HS. A biopsy of an indolent sacral ulcer in September 1984 revealed an infiltrating, well to moderately differentiated squamous carcinoma (Fig. i). The tumour cells extended through to the deep dermis and in some areas involved the subcutaneous tissues. A second area of non-healing chronic ulceration of the left hip was biopsied in January 1985 and showed a moderately differentiated squamous cell carcinoma of the skin. The tumour extended to the deep dermis, but did not involve the subcutaneous tissues. Radiology. Chest X-ray was normal. A barium enema was performed to investigate the mild anaemia, and to exclude the possibility of occult Crohn's disease contributing to the inguinal and perineal fistulae; it showed diverticulosis but was otherwise normal.
SCC in hidradenitis suppurativa 529 FIGURE 2. Squamous cell carcinoma over the sacrum. Treatment and progress The patient was admitted to hospital for treatment which included surgical incision and the marsupialization of five sinuses. The remaining hidradenitis was treated by cleaning with an antiseptic twice daily and a 4-week course of antibiotics. During the next 2 years he required frequent admission to hospital, treating in rotation each involved area. His condition had improved until the development of an indolent non-healing area over the sacrum (Fig. 2). A biopsy revealed a squamous cell carcinoma which was treated by deep excision using a diathermy loop. The large ulcerated area was then pinch grafted, which initially appeared to take, but which was later rejected. Further biopsies from the granulation tissue revealed a recurrence of the squamous cell carcinoma. In January 1985 he developed a new area of what appeared to be granulation tissue in a different area of hidradenitis suppurativa over the left hip. A biopsy confirmed a second squamous cell carcinoma. His general health deteriorated rapidly and he died with bronchopneumonia in February 1985, 2 years after the initial referral. A post mortem examination was not performed. DISCUSSION Hidradenitis suppurativa (HS), acne conglobata and dissecting periofolliculitis of the scalp are three similar diseases which are referred to as the 'follicular occlusion triad'. Squamous cell carcinoma (SCC) has been reported as a late complication in three conditions.'"' Despite being a relatively common skin disease, hidradenitis suppurativa is rarely compli-
530 A.V.Anstey et al. s S ^ I E -g, -6 -S 0 > Q a J: x; J: j= 0 Q Q P a F " c ^? I II II "I E ^ > -6 a j< MM m 3 3 g 3 J Q
SCC in hidradenitis suppurativa 531 cated by squamous cell carcinoma. There are 15 cases on record of which 11 have been reported in detail (Table i). The length of time from onset of HS to diagnosis of SCC varied from less than I year to 32 years. In the majority of cases, the SCC have occurred in the anogenital region, with single case reports of SCC arising in the axilla and thigh. Death occurred in at least seven of the 15 cases. The prognosis for cases of SCC occurring in HS is poor (Table i). This may be explained by the often advanced state of the SCC at the time of diagnosis and difficulty in obtaining a diagnostie biopsy. In one case there was an 8-month delay between the development of SCC and its removal by definitive surgery. * In another case there was an 8-month delay between development of SCC and its surgical excision, during which time seven negative biopsies were obtained before a positive histological diagnosis was made.^ This ease illustrates the importance of repeat biopsies in any atypical non-healing ulcer occurring at a site of chronic inflammation of skin. Squamous cell carcinomas arising in ehronieally scarred inflamed skin are, in general, more malignant than other SCCs arising de novo or on a background of sun-damaged skin, as demonstrated by their rapid growth, local aggressiveness and early metastases.^ Surgical treatment of longstanding, extensive hidradenitis suppurativa should be considered in preference to medical management as this is likely to reduce the incidence of SCC. Long-term regular follow-up of extensive HS is important as the aggressive nature of secondary SCCs make early diagnosis and surgical intervention essential." Following surgery, provision should be made for close follow-up to detect evidence of metastatic disease. In summary, our patient had longstanding, severe and extensive hidradenitis suppurativa which we managed conservatively with a combined surgical and medical approach. This proved to be unsuccessful as he developed two separate areas of squamous cell carcinoma arising from the HS. Attempts at surgical excision were unsuccessful and the aggressive nature of the SCC was illustrated by his rapid decline and death. ACKNOWLEDGMENT We wish to thank Mrs B.Knott for preparing the manuscript. REFERENCES r Camisa C, Squamous cell carcinoma arising in acne conglobata. Cutis 1984; 33: 185-90. 2 Curry SS, Gaither DA, King LE. Squamous cell carcinoma arising in dissecting perifoilictilitis of the scalp. J' Am Acad Dermaio! 1981; 4: 673-8. 3 Humphrey LJ, Playforth H, Leavell UW. Squamous cell carcinoma arising in hidradenitis suppurativum. Arch Dermatat 1969; ioo: 59-62. 4 Jackman RJ. Hidradenitis suppurativa: diagnosis and management of perianal manifestations, Proc R Society Med (Suppll 1959; 52: 110-12. 5 Donsky HJ, Mendelson CG. Squamous cell carcinoma as complication of hidradenitis suppurativa. Arch Dermatol 1964; 90: 488-91. 6 Gordon SW. Squamous cell carcinoma arising in hidradenitis suppurativa: case report. Plasi Rtxorutr Surg 1977; 60: 800-2. 7 Alexander SJ. Squamous cell carcinoma in chronic hidradenitis suppurativa. Cancer 1979; 43: 745-8. 8 Johnston WH, Miller TA, Frileck SP. Atypical pseudoepitheliomatous hyperplasia and squamous cell carcinoma in chronic cutaneous sinuses and fistulas. Plast Recomtrr Surg 1980; 60: 395-9. 9 Mora RG, Perniciaro C. Cancer ofthe skin in blacks, i. A review of 163 blacks patients with cutaneous squamous cell carcinoma, J Am Acad Dermatol 1981; 5: 535-43. 10 Black SB, Woods JE. Squamous cell carcinoma complicating hidradenitis suppurativa._7,vhrgohc()/1982; 19: 25-6. 11 Sparks MK, Kuhiman DS, Prieto A, Callen JP. Hypercalcaemia in association with cutaneous squamous cell carcinoma. Arch Dermatot 1985; I2i: 243-6. 12 Zachary LS, Robson MC, Rachmaninoff N. Squamous cell carcinoma occurring in hidradenitis suppurativa. An Plast Surg 1987; 18: 71-3.