Epidermoid Carcinoma Occurring in Acne Conglobata *

JOHN S. DILLON," M.D., HARLAN J. SPJUT,***'M.D.

From the Departments of Surgery and Surgical Pathology, Washington UniversitySchool of Medicine, and Barnes Hospital, St. Louiis, Mlissouiri

ACNE CONGLOBATA, a severe form ofchronic acne, was first described bySpitzer," in 1903, as "Dermatitis Folliculariset Perifollicularis Conglobata." A variantof it, "Acne Aggregata Seu Conglobata,"in which the lesions communicate with oneanother by fistulae was described by Reit-mann,5 in 1908. Since then, reports con-cerning this relatively rare dermatitis havebeen few, being case reports, bacteriologicsttudies and discussions of its resemblanceto cutaneous tuberculosis. The best de-scription of it is that of Michelson andAllen.4Although Selisky believed acne conglo-

bata to be merely an extreme form of acneoccurring in patients of the so-called sebor-rheic type, several features serve to dis-tinguish it from acne vulgaris. Acne con-globata is practically an affliction of menonly, beginning during late puberty andcontinuing throughout life. At times it ap-pears to be familial. It may affect any partof the body, but especially the hairiestparts such as the chest, shoulders and back.The disease starts with the appearance

of comedones that are accompanied by in-flammation. Large papules, or papulo-pus-tules and, at times, large sebaceous reten-tion cvsts, are subsequently generated. Thedisease proceeds indolently, forming ex-panding elevated plaques of inflammationin which abscesses develop and rupture.The abscesses may coalesce forming ser-piginous spreading ulcers. Frequently theulcers communicate with one another sub-cutaneously forming cutaneous fistulas.Whenever the ulcers do heal, they form

Suibmitted for publication February 8, 1963.*4 Present address: Georgetown University

Schlool of MIedicine, Washington 7, D. C.... Present address: Department of Surgical

Pathology, Baylor University, Houiston, Texas.

pitted, irregular keloid scars, the so-calledbridge scars of Lang.3 These scars oftenenclose sebaceous cysts, comedones, or pus-tules. Usually all of the various stages ofthe lesions are to be found interspersed.Hyperkeratosis of hair follicles and theepidermal surface, attenuation of the reteridges, near absence of the granular celllayer epidermal cornified whorls and kera-totic cysts characterize the individual le-sions. Excision of the severely afflictedskin and covering the cutaneous defectswith split skin grafts is the only effectivetreatment known. Irradiation and antibi-otics have failed to control the disease.

If chronicity of inflammation promotesor generates neoplasia, acne aggregata seuconglobata should be associated more thanrarely with cutaneous carcinoma. One caseof epidermoid carcinoma associated withacne conglobata has been reported.3 An-other, ambiguously described as epitheli-onza, may have been epidermoid carcinomaassociated with acne conglobata.2During the past ten years, two patients

have been seen at Barnes Hospital withsquamous cell carcinoma located in regionsof extensive involvement with acne seuconglobata.

Case ReportsCase 1 F. B. (BH 213434) A 39-year-old

white mllan was first seen during 1952. He badhad acne for many years, especially involving thebuttocks, arms, chest, perineumil and scrotum. Hehad felt a lump over the sacrum for two years,which drained six weeks before we saw him, buta tender mass that drained pu!s persisted.

The skin of most of the body contained manypitted scars. There were sinuses in the skin ofboth buttocks, the perineum, and the base of thescroturm. The medial aspects of the buttocks wereindurated and a 6 x 4 cm., elevated, fungatingmass overlay the left side of the sacrum. Several1 x 3 cmii. lymplh nodes were palpable in the

451

452 I)ILLON AN!) S'1JUT

FIG. 1. Case 2. Photo, taken in 1957, shows themyriad scars, pits, and draining sinuses. Wash. U.Ill. 57-4211.

groins. A biopsy specimen of the mass containedepidermoid carcinoma. On September 3, 1952, aresection of the skin and subcutaneouis tissue fromthe femoral trochanters laterally to the anus in-feriorly and to the second lumbar vertebra supe-riorly was performed. The coccyx and lower twosacral vertebrae were also removed. Split-thicknessskin grafts were applied to the defect primarily.Epidermoid carcinoma was found in the skin ofthe buttocks. Bone was not invaded.

The patient was re-admitted three times fordrainage of abscesses of the perianal region,axilla, and neck between 1952 and 1959. In Juneof 1959, a cyst and sinus tract adjacent to theantis were excised and contained epidermoid car-cinoma. The wound drained pus continuously andfailed to heal. During December, 1959, the ulcerwhich was adjacent to the antis wa., found tocontain epidermoid carcinoma. On December 8,1959, a modified abdominoperineal resection wasperformed. No metastases were found in 14 lymphnodes.

In August, 1960, a sinus tract located near the

Annals of StirgeryMarch 1964

base of the scrotum was excised; in it epidermoidcarcinoma was found. The patient was alive andvisibly free of carcinomna in Februiary, 1962.

Case 2 G. B. (BH 289662) A 47-year-oldwdhite mlan was first adlmitted to Barnes Hospitalin 1957. He had had severe acne conglobata for35 years (Fig. 1). Acne of the buttocks wastreated with an indetermiiinate dosage of x-rays in1950. For four months preceding admission henoted tenesmus. For three months an ulcer on theright buttock enlarged steadily.

Active acne, deep pits, and suppuration afflictedthe trunk, face, all extremities, back, lower abdo-men, groin and chest. The skin of the buttocksand perianal region was greatly thickened andcontained many sinuses exuding pus. A 2 cm.condylomatous lesion was present in the midlinejust superior to the anus. The right buttock borean 8 x 6 cm. irregular ulcer with raised under-mined edges (Fig. 2). The ischiorectal fat wasindurated. Hard inguinal nodes were felt bi-laterally. The ulcer of the right buttock con-tained epidermoid carcinoma.

On May 25, 1957, a sigmoid colostomy wasperformed and biopsies of the perianal, thigh andabdominal skin taken. All contained acne con-globata. On June 4, 1957, the skin of both but-tocks and part of both gluteus maximus muscles.were excised, and the denuded surface coveredby split-thickness grafts. The carcinoma did notinvade muscle and did not reach the limits ofresection.

During 1957 and 1958, abscesses were drainedand sinuses of the perianal, sacral, and axillaryregions excised. During 'May, 1961, he was againadmitted, complaining of pain in the right but-tock with fever and chills. There were mnultiplesinuses in the axillae and buttocks. Hard, tender,

FIG. 2. Case 2. The fungating, ulcerated epider-moid carcinoma of the right buttock. Wash. U.Ill. 57-4212.

Volume 159Number 3

EPIDERMAL CARCINOMA OCCURRING IN ACNE CONGLOBATA

FIG. 3. A fairly well differentiated infiltrative epidermoid carcinoma of the buttock of Case1. In other areas, the tumor was very well differentiated with abundant keratin formation.W. U. 11. 62-3409. 85 X.

friable masses in the skin about the anus con-tained epidermoid carcinoma. There were a recto-perineal fistula and absence of tone of the analsphincter. On Mlay 31, a wide perineal resectionwas performed removing the anus, ischio-rectal fatand perianal skin. Split-thickness skin grafts wereapplied to the defect. There was epidermoid car-cinoma in the perianal skin, invading the ischio-rectal fat. There was no tumor in 20 regionallymph nodes, or at the margins of resection. InSeptember, 1962, the patient returned with a 3cm. diameter perineal ulcer at the edge of theprevious graft. On September 20, a wide resectionwas performed including portions of the mem-branous urethra, right corpus cavernosum andseminal vesicle. Split-thickness grafts were againapplied to the region. Epidermoid cancer wasagain found projecting deep into the dermis.

PathogenesisFrom 1952 through 1960, multiple bi-

opsies and resections of skin were madefrom varied body sites of Case 1 (F. B.).

453

FIG. 4. A biopsy of axillary skin of Case 1.Epidermal hyperplasia, scarring, and dilated apo-crine glands are noted. Exudate is not seen in theglands; in this way the lesion differs from by-dradenitis suppurativa. W. U. Ill. 62-3404. 40 X.

454 DILLON AN

FIG. 5. The typical alterations of acne conglo-bata are seen in this specimen from the btuttockof Case 2. Note the scarring, chronic inflamma-tion and the epithelial lined sintus tracts. NV. U. Ill.62-3408. 40 x.

kD SPJUT Annals of SturgeryMarch 1964

The sites ineluLded the sacral area, anus,axilla, neck, buttocks, and thigh. The skinin all the areas had the features of acnesetn conglobata: comedones, hyperplasia ofthe epidermis, epidermal-lined sintuses andfistulae, scarring, acute and chronic inflam-mation, and epidermal inclusion cysts. Theepithelial hyperplasia was often atypical;pseudoepitheliomatous hyperplasia existedin many places within the epidermis andsinus tracts. Scarring obliterated the cu-taneous appendages and often extended toand into underlying skeletal muscle. Themultiple biopsies of the axillae showed thesame changes; in addition, the apocrineglands were dilated but did not containpus. Consequently, hidradenitis supurativadid not exist.

In the first case, all carcinomas, the supra-sacral one (1952), the one of the buttocks(1959) and that in a perineal sinus tract(1960), were well differentiated invasiveepidermoid carcinomas associated with thescarring, inflammation and sinuses. Theneoplasm invaded muscles of the buttocksin the first two carcinomas.

FIG. 6. The well differentiated epidermoid carcinoma of the perianal region of Case 2.W. U. 111. 62-3407. 35 X.

V'olume 159 EPIDERMAL CARCINO'MA OCCURRING IN ACNE CONGLOBATA 4,55Number 3

lMultiple biopsy specimens had also beentaken from Case 2 (G. B.). These werefrom the skin of the buttocks, anterior ab-dominal wall, thigh, anus, axillae, peri-neum, and sacrum. Histologic alterationswere identical to those of the first case.All three carcinomas, that from the rightbuttock resected in 1957, that from theperianal region resected in 1961 and thatfrom the perineum in 1962, were well dif-ferentiated invasive epidermoid carcino-mas. None of the six epidermoid carcino-mas has metastasized to regional lymphnodes.

DiscussionInfections, intracutaneous sinuses and

fistulae, septicemia and, rarely, epidermoidcarcinoma complicate acne seu conglobata.The pathogenesis of the carcinomas maybe related to the chronic inflammation, asituation analogous to that of epidermoidcarcinomas arising in the sinuses of chronicosteomyelitis. These carcinomas and thosethat occur in acne seu conglobata are usu-ally well differentiated, slow growing andslow to metastasize. In fact, of the knowncarcinomas arising in acne seu conglobatanone have had demonstrable metastases.One of our patients (G. B.) and another

previously reported 2 received radiation fortreatment of acne. The intervals from ex-posure to diagnosis of cancer were sevenyears and two months respectively. Theseare shorter than usually seen in radiationinduced skin cancers.1 It is consequentlyunlikely that radiation was the primarycause of these cancers.Both of our patients have had three

carcinomas. The question arises whetherthe later lesions were primaries or persist-ences. We are inclined to believe that thelater lesions are new primaries for the fol-lowing reasons: 1) in all excisions, no tumorwas found at the lines of resection; 2) thelesions were widely separated; and 3) theinterval of time between the excision ofthe first and the appearance of the secondtumors was seven years (F. B.) and fouryears (G. B.).

The treatment of the two mie was simlli-lar: wide resection of the tumor and sur-rounding tissue first, followed some yearslater by a more extensive resection forcancers wvhich wvere probably7 new7 pri-maries.

It would seem that the most logicaltreatment of these carcinomas would bepreventive. Elimination of the drainingsinuses and involved skin in those areaswhich have shown a propensity for devel-oping carcinoma, that is, buttocks, peri-neum, and sacral regions, by wide excisionand skin grafting before carcinoma devel-ops should be effective.

SummaryTwo patients with acne conglobata, com-

plicated by epidermoid carcinomas, are re-ported. Both are believed to have threeindependent carcinomas.Treatment in both cases consisted of

wide resection followed by perineal resec-tion four and seven vears later.

All known cases of epidermoid carci-nomas arising in areas of acne conglobatahave occurred in the same region-sacrum,buttocks, or perineum. None have metas-tasized to regional lymph nodes, or distally.

References1. Ackerman, L. V. and J. A. del Regato: Cancer,

St. Louis, C. V. Mosby, p. 171, 1962.2. Hellier, F. F.: A Familial Case of Acne Vul-

garis with Lesions Suggesting a Relationshipto Acne Conglobata. Brit. J. Dermatol. &Syph., 51:109, 1939.

3. Lang, E.: Hautkrankheiten, Wiesbaden, J. F.Bergman, p. 504, 1902.

4. MIichelson, H. E. and P. K. Allen: Acne Con-globata. Arch. Dermatol. & Syph., 23:49,1931.

5. Reitmann, K.: Acne Aggregata Seu Conglobata.Arch. f. Dermatol. u. Syph., 90:249, 1908.

6. Schiff, B. and A. B. Kern: Carcinoma Devel-oping in Chronic Acne Conglobata. Arch.Dermatol., 75:879, 1957.

7. Selisky, A. B.: Zur Histologie Acne Conglo-bata. Arch. f. Dermatol. u. Syph., 158:460,1929.

8. Spitzer, L.: Dermatitis Follicularis et Perifol-licularis Conglobata. Dermatol. Zeitschr., 10:109, 1903.