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Pyoderma Gangrenosum of Penile and Scrotal Skin
0022-5347 /82/ _'_2 72-0547$02.00/0 Vol. 127. March Printed U.S ..!L
T::iE ~louRNAL OF UROLOGY
Copyright© 1982 by ?he Williams & Wilkins Co.
PYODERMA GANGRENOSUivI OF PENILE AND SCROTAL SKIN I. DANIEL SANUSI, * ENRIQUE GONZALEZ
AND
DENNIS D. VENABLE
From the Departments of Pathology and Urology, Louisiana State University Medical Center, Shreveport, Louisiana
ABSTRACT
Pyoderma gangrenosum is a unique cutaneous ulceration with characteristic clinical features. Although pyoderma gangrenosum may occur on any area of the it is most commonly found on the lower extremities. Involvement of the external genitalia is rare. We report a case of pyoderma gangrenosum involving the penile and scrotal skin associated with ulcerative colitis. The cutaneous genital lesions were the major presenting manifestations of an underlying chronic inflammatory bowel disease. Pyoderma gangrenosum is a noninfectious cutaneous ulceration that may start as a pustule, furuncle-like nodule or hemorrhagic bulla. Pyoderma gangrenosum often is associated with internal disorders. The association between pyoderrna gangrenosum and ulcerative colitis was first recognized in 1930 by Brunsting and associates. 1 Since then, the association of ulcerative colitis with various disorders, especially pyoderrna gangrenosum, has been confirmed by various authors. 2 - 4 Although pyoderma gangrenosum may occur on any area of the body it rarely involves the penile and scrotal skin. Herein we report a case of pyoderma gangrenosum involving the penis, scrotum and perineum associated with ulcerative colitis in which the cutaneous lesions were the major presenting manifestations. CASE REPORT
A 67-year-old black man was seen in the urology clinic in June 1980 with a 3-week history of penile swelling. He also complained of having chronic profuse watery diarrhea for 18 months. Physical examination revealed swelling of the scrotal and penile skin with phimosis, associated balanoposthitis and local tissue maceration. There were several ulcerative lesions, measuring up to l. 7 cm. in diameter, with slightly raised margins on the glans penis and foreskin (fig. 1). There were numerous scattered pustules on the penile and scrotal skin. Similar erosions and pustules were seen on the perineum. A complete blood count and subsequent hematologic studies revealed anemia of chronic illness. Other laboratory data were essentially unremarkable. Dark field examination of secretions obtained from the penile lesions was negative for spirochetes. Rapid plasma reagin was negative. Upper gastrointestinal series, proctoscopy, colonoscopy, barium enema studies and rectal biopsy revealed changes compatible with ulcerative colitis. The clinical diagnoses were chronic balanoposthitis, phimosis, chronic diarrhea and anemia associated with inflammatory bowel disease. A dorsal slit of the foreskin was performed to assist in the control of any associated secondary infection. The patient was treated with salicylazosulfapyridine and steroids for 4 weeks with only minimal relief In August he underwent total colectomy with abdominoperineal resection and terminal ileostomy. Histopathologic examination of the colon confirmed the diagnosis of ulcerative colitis. Characteristic crypt abscesses of the colon were found. Granulomas were not identified. A circumcision was performed on September 2 to assist in local control of the genital lesions.
FIG. l. Photograph of penile and scrotal skin shows edema, numerous scattered pustules, multiple ulcers and post-inflammatory hypopigmentation.
Examination of specimens taken from the foreskin and perineum revealed identical histologic features. In some areas there was massive infiltration of the upper dermis with polymorphonuclear leukocytes and eosinophils (fig. 2). These subepidermal abscesses occasionally drained to the skin surface (fig. 3). In other areas there was infiltration with lymphocytes accompanied by marked acanthosis of the overlying epidermis. Granulomas were not seen. The histologic findings were consistent with pyoderrna gangrenosum. Postoperatively, the patient had no complications and was discharged from the hospital on September 4 on no medication.
Accepted for publication May 1, 1981. * Requests for reprints: Department of Pathology, Louisiana State University Medical Center, P. 0. Box 33932, Shreveport, Louisiana 71130.
547
548
SANUSI, GONZALEZ AND VENABLE
The penile, scrotal and perinea! lesions resolved slowly during the subsequent 6-month period with no additional treatment. DISCUSSION
Since its first description in 1930 pyoderma gangrenosum has become well established as a clinical entity. It is a rare skin disease, usually starting as ~1 discrete pustules that may coalesce or break down to form gangrenous ulcers. 5 The characteristic ulcer is irregular in outline with raised, purple, tender undermined borders and an edematous necrotic base. When characteristic ulcers are present diagnosis is easy. However, patients with pyoderma gangrenosum often have pustules, papules or plaques that evolve or resolve without ever passing through an ulcerative phase. 6 The early pustular lesions may resemble iododerma, bromoderma, folliculitis, herpesvirus infection or dermatitis herpetiformis. The lesions may arise spontaneously or at points of trauma. Pyoderma gangrenosum is most commonly found on the lower extremities but may appear on any area of the body. 6 • 7 The histologic findings, although not diagnostic, are especially important to rule out other specific
FIG. 2. Photomicrograph of pustule of foreskin shows subepidermal abscesses and marked acanthosis of the epidermis. H & E, reduced from X40.
diseases, including herpes genitalis, dermatitis herpetiformis, syphilis, malignancy and various granulomatous diseases. Early lesions often are characterized by subepidermal abscesses. These often drain to the skin surface, resulting in epidermal necrosis and ulceration. Vasculitis usually is absent. lmmunofluorescent studies do not contribute to the diagnosis of pyoderma gangrenosum. The association of pyoderma gangrenosum with ulcerative colitis is well established. The general impression in the literature has been that 50 per cent of patients with pyoderma gangrenosum have ulcerative colitis. 5 ' 8 Although pyoderma gangrenosum is most commonly associated with ulcerative colitis it also may be associated with a number of diseases, including Crohn's disease, rheumatoid arthritis, leukemia, polycythemia vera, myeloid metaplasia, other paraproteinemias and chronic active hepatitis. 7• 9 The course of pyoderma gangrenosum commonly parallels that of associated ulcerative colitis. Successful medical or surgical treatment of the colitis usually has been followed by resolution of all cutaneous lesions. 2 • 10 The pyoderma gangrenosum in our patient resolved within 6 months following total colectomy. The etiology of pyoderma gangrenosum is not known. 7• 10 The earliest observations suggesting that pyoderma gangrenosum was bacterial in origin were supported by culture findings but were not verified by later studies. It was later proposed that pyoderma gangrenosum was an example of a Shwartzman phenomenon. This theory lost favor because vascular inflammatory reaction is not observed as a prominent histologic feature of pyoderma gangrenosum. A viral etiology was proposed by some authors but could not be confirmed by other investigators. The theory that proteolytic enzymes capable of digesting epidermal cells had a role in the pathogenesis of pyoderma gangrenosum and ulcerative colitis lacks confirmation. More recent studies favored an immunologic explanation of the pathogenesis of pyoderma gangrenosum. Although altered concentrations of immunoglobulin often are seen in pyoderma gangrenosum the pattern of alteration is not consistent enough to help in the diagnosis. Other observations favoring an immunologic mechanism include the demonstration of delayed type reactions to bacterial filtrates of staphylococci in patients with pyoderma
FIG. 3. A, photomicrograph of ulcer margin of foreskin shows intense suppuration in upper dermis, epidermal necrosis, acanthosis and parakeratosis. B, photomicrograph of pustule of foreskin reveals subepidermal abscesses that drain to skin surface. H & E, reduced from XlOO.
PYODERMA GANGRENOSUM OF PENILE AND SCROTAL SKIN
gangrenosum, the rejection of autologous skin grafts and the demonstration of delayed hypersensitivity to intradermal injection of autologous leukocytes. These and other data on the etiologic factors of pyoderma gangrenosum and its association with ulcerative colitis are still under investigation. Dr. Warren D. Grafton reviewed this manuscript.
4. 5. 6.
7. REFERENCES 8. 1. Brunsting, L.A., Goeckerman, W. H. and O'Leary, P.A.: Pyoderma
(ecthyma) gangrenosum. Clinical and experimental observations in five cases occurring in adults. Arch. Dermatol. Syph., 22: 655, 1930. 2. Samitz, M. H.: Skin complications of ulcerative colitis and Crohn's disease (with special reference to pyoderma gangrenosum). Cutis, 12: 533, 1973. '-'· Greenstein, A. J., Janowitz, H. D. and Sachar, D. B.: The extra-
9.
10.
549
intestinal complications of Crohn' s disease and ulcerative colitis: a study of 700 patients. Medicine, 55: 401, 1976. O'Loughlin, S. and Perry, H. 0.: A diffuse pustular eruption associated with ulcerative colitis. Arch. Dermatol., 114: 1061, 1978. Thornton, J. R., Teague, R.H., Low-Beer, T. S. and Read, A. E.: Pyoderma gangrenosum and ulcerative colitis. Gut, 21: 247, 1980. Shore, R. N.: New look at pyoderma gangrenosum. Cutis, 20: 209, 1977. Callen, J.P. and Taylor, W. B.: Pyoderma gangrenosum-a literature review. Cutis, 21: 61, 1978. Perry, H. 0.: Pyoderma gangrenosum. South. Med. J., 62: 899, 1969. Hickman, J. G. and Lazarus, G. S.: Pyoderma gangrenosum: new concepts in etiology and treatment. In: Dermatology Update. Reviews for Physicians. Edited by S. L. Moschella. New York: Elsevier, p. 325, 1979. Watts, J. M., De Dombal, F. T. and Goligher, J. C.: Long-term complications and prognosis following major surgery for ulcerative colitis. Brit. J. Surg., 53: 1014, 1966.
T::iE ~louRNAL OF UROLOGY
Copyright© 1982 by ?he Williams & Wilkins Co.
PYODERMA GANGRENOSUivI OF PENILE AND SCROTAL SKIN I. DANIEL SANUSI, * ENRIQUE GONZALEZ
AND
DENNIS D. VENABLE
From the Departments of Pathology and Urology, Louisiana State University Medical Center, Shreveport, Louisiana
ABSTRACT
Pyoderma gangrenosum is a unique cutaneous ulceration with characteristic clinical features. Although pyoderma gangrenosum may occur on any area of the it is most commonly found on the lower extremities. Involvement of the external genitalia is rare. We report a case of pyoderma gangrenosum involving the penile and scrotal skin associated with ulcerative colitis. The cutaneous genital lesions were the major presenting manifestations of an underlying chronic inflammatory bowel disease. Pyoderma gangrenosum is a noninfectious cutaneous ulceration that may start as a pustule, furuncle-like nodule or hemorrhagic bulla. Pyoderma gangrenosum often is associated with internal disorders. The association between pyoderrna gangrenosum and ulcerative colitis was first recognized in 1930 by Brunsting and associates. 1 Since then, the association of ulcerative colitis with various disorders, especially pyoderrna gangrenosum, has been confirmed by various authors. 2 - 4 Although pyoderma gangrenosum may occur on any area of the body it rarely involves the penile and scrotal skin. Herein we report a case of pyoderma gangrenosum involving the penis, scrotum and perineum associated with ulcerative colitis in which the cutaneous lesions were the major presenting manifestations. CASE REPORT
A 67-year-old black man was seen in the urology clinic in June 1980 with a 3-week history of penile swelling. He also complained of having chronic profuse watery diarrhea for 18 months. Physical examination revealed swelling of the scrotal and penile skin with phimosis, associated balanoposthitis and local tissue maceration. There were several ulcerative lesions, measuring up to l. 7 cm. in diameter, with slightly raised margins on the glans penis and foreskin (fig. 1). There were numerous scattered pustules on the penile and scrotal skin. Similar erosions and pustules were seen on the perineum. A complete blood count and subsequent hematologic studies revealed anemia of chronic illness. Other laboratory data were essentially unremarkable. Dark field examination of secretions obtained from the penile lesions was negative for spirochetes. Rapid plasma reagin was negative. Upper gastrointestinal series, proctoscopy, colonoscopy, barium enema studies and rectal biopsy revealed changes compatible with ulcerative colitis. The clinical diagnoses were chronic balanoposthitis, phimosis, chronic diarrhea and anemia associated with inflammatory bowel disease. A dorsal slit of the foreskin was performed to assist in the control of any associated secondary infection. The patient was treated with salicylazosulfapyridine and steroids for 4 weeks with only minimal relief In August he underwent total colectomy with abdominoperineal resection and terminal ileostomy. Histopathologic examination of the colon confirmed the diagnosis of ulcerative colitis. Characteristic crypt abscesses of the colon were found. Granulomas were not identified. A circumcision was performed on September 2 to assist in local control of the genital lesions.
FIG. l. Photograph of penile and scrotal skin shows edema, numerous scattered pustules, multiple ulcers and post-inflammatory hypopigmentation.
Examination of specimens taken from the foreskin and perineum revealed identical histologic features. In some areas there was massive infiltration of the upper dermis with polymorphonuclear leukocytes and eosinophils (fig. 2). These subepidermal abscesses occasionally drained to the skin surface (fig. 3). In other areas there was infiltration with lymphocytes accompanied by marked acanthosis of the overlying epidermis. Granulomas were not seen. The histologic findings were consistent with pyoderrna gangrenosum. Postoperatively, the patient had no complications and was discharged from the hospital on September 4 on no medication.
Accepted for publication May 1, 1981. * Requests for reprints: Department of Pathology, Louisiana State University Medical Center, P. 0. Box 33932, Shreveport, Louisiana 71130.
547
548
SANUSI, GONZALEZ AND VENABLE
The penile, scrotal and perinea! lesions resolved slowly during the subsequent 6-month period with no additional treatment. DISCUSSION
Since its first description in 1930 pyoderma gangrenosum has become well established as a clinical entity. It is a rare skin disease, usually starting as ~1 discrete pustules that may coalesce or break down to form gangrenous ulcers. 5 The characteristic ulcer is irregular in outline with raised, purple, tender undermined borders and an edematous necrotic base. When characteristic ulcers are present diagnosis is easy. However, patients with pyoderma gangrenosum often have pustules, papules or plaques that evolve or resolve without ever passing through an ulcerative phase. 6 The early pustular lesions may resemble iododerma, bromoderma, folliculitis, herpesvirus infection or dermatitis herpetiformis. The lesions may arise spontaneously or at points of trauma. Pyoderma gangrenosum is most commonly found on the lower extremities but may appear on any area of the body. 6 • 7 The histologic findings, although not diagnostic, are especially important to rule out other specific
FIG. 2. Photomicrograph of pustule of foreskin shows subepidermal abscesses and marked acanthosis of the epidermis. H & E, reduced from X40.
diseases, including herpes genitalis, dermatitis herpetiformis, syphilis, malignancy and various granulomatous diseases. Early lesions often are characterized by subepidermal abscesses. These often drain to the skin surface, resulting in epidermal necrosis and ulceration. Vasculitis usually is absent. lmmunofluorescent studies do not contribute to the diagnosis of pyoderma gangrenosum. The association of pyoderma gangrenosum with ulcerative colitis is well established. The general impression in the literature has been that 50 per cent of patients with pyoderma gangrenosum have ulcerative colitis. 5 ' 8 Although pyoderma gangrenosum is most commonly associated with ulcerative colitis it also may be associated with a number of diseases, including Crohn's disease, rheumatoid arthritis, leukemia, polycythemia vera, myeloid metaplasia, other paraproteinemias and chronic active hepatitis. 7• 9 The course of pyoderma gangrenosum commonly parallels that of associated ulcerative colitis. Successful medical or surgical treatment of the colitis usually has been followed by resolution of all cutaneous lesions. 2 • 10 The pyoderma gangrenosum in our patient resolved within 6 months following total colectomy. The etiology of pyoderma gangrenosum is not known. 7• 10 The earliest observations suggesting that pyoderma gangrenosum was bacterial in origin were supported by culture findings but were not verified by later studies. It was later proposed that pyoderma gangrenosum was an example of a Shwartzman phenomenon. This theory lost favor because vascular inflammatory reaction is not observed as a prominent histologic feature of pyoderma gangrenosum. A viral etiology was proposed by some authors but could not be confirmed by other investigators. The theory that proteolytic enzymes capable of digesting epidermal cells had a role in the pathogenesis of pyoderma gangrenosum and ulcerative colitis lacks confirmation. More recent studies favored an immunologic explanation of the pathogenesis of pyoderma gangrenosum. Although altered concentrations of immunoglobulin often are seen in pyoderma gangrenosum the pattern of alteration is not consistent enough to help in the diagnosis. Other observations favoring an immunologic mechanism include the demonstration of delayed type reactions to bacterial filtrates of staphylococci in patients with pyoderma
FIG. 3. A, photomicrograph of ulcer margin of foreskin shows intense suppuration in upper dermis, epidermal necrosis, acanthosis and parakeratosis. B, photomicrograph of pustule of foreskin reveals subepidermal abscesses that drain to skin surface. H & E, reduced from XlOO.
PYODERMA GANGRENOSUM OF PENILE AND SCROTAL SKIN
gangrenosum, the rejection of autologous skin grafts and the demonstration of delayed hypersensitivity to intradermal injection of autologous leukocytes. These and other data on the etiologic factors of pyoderma gangrenosum and its association with ulcerative colitis are still under investigation. Dr. Warren D. Grafton reviewed this manuscript.
4. 5. 6.
7. REFERENCES 8. 1. Brunsting, L.A., Goeckerman, W. H. and O'Leary, P.A.: Pyoderma
(ecthyma) gangrenosum. Clinical and experimental observations in five cases occurring in adults. Arch. Dermatol. Syph., 22: 655, 1930. 2. Samitz, M. H.: Skin complications of ulcerative colitis and Crohn's disease (with special reference to pyoderma gangrenosum). Cutis, 12: 533, 1973. '-'· Greenstein, A. J., Janowitz, H. D. and Sachar, D. B.: The extra-
9.
10.
549
intestinal complications of Crohn' s disease and ulcerative colitis: a study of 700 patients. Medicine, 55: 401, 1976. O'Loughlin, S. and Perry, H. 0.: A diffuse pustular eruption associated with ulcerative colitis. Arch. Dermatol., 114: 1061, 1978. Thornton, J. R., Teague, R.H., Low-Beer, T. S. and Read, A. E.: Pyoderma gangrenosum and ulcerative colitis. Gut, 21: 247, 1980. Shore, R. N.: New look at pyoderma gangrenosum. Cutis, 20: 209, 1977. Callen, J.P. and Taylor, W. B.: Pyoderma gangrenosum-a literature review. Cutis, 21: 61, 1978. Perry, H. 0.: Pyoderma gangrenosum. South. Med. J., 62: 899, 1969. Hickman, J. G. and Lazarus, G. S.: Pyoderma gangrenosum: new concepts in etiology and treatment. In: Dermatology Update. Reviews for Physicians. Edited by S. L. Moschella. New York: Elsevier, p. 325, 1979. Watts, J. M., De Dombal, F. T. and Goligher, J. C.: Long-term complications and prognosis following major surgery for ulcerative colitis. Brit. J. Surg., 53: 1014, 1966.