Pyoderma Gangrenosum of the Penis - A Potentially Dramatic Skin Disease
Pyoderma gangrenosum (PG) is a reactive inflammatory skin disorder that is part of the spectrum of neutrophilic dermatoses.
Neutrophilic dermatoses are characterized histopathologically by a normal polymorphonuclear leukocyte infiltrate, and they are frequently associated with other systemic diseases.
PG includes four recognized types: ulcerative (the most common), pustular,bullous, and vegetative.
2 Ulcerative PG usually presents as a painful, necrolytic ulcer with an erythematous or violaceous, undermined border.
It expands rapidly, and it can be quite destructive.
PG most commonly appears on the lower extremities, but any cutaneous or mucocutaneous site can be affected.
Penile involvement is quite rare, but because the penis is a very tissue-sensitive area, prompt diagnosis and treatment is crucial.
We report the case of a 17-year-old, previously healthy, boy who was referred to our department by his urologist for a genital ulcer that had given rise to a urethral fistula that had failed to heal after two plastic reconstructions with skin grafts.
One year before his presentation to our department,the patient had noted a painful ulcer in the posterior surface of the neck of the glans that soon expanded to involve the frenulum.
He was prescribed topical treat ment with betamethasone valerate and fucidic acid, and the lesion improved.
After 1 month, a small erosion was still present in the frenulum, and it was decided to excise the frenulum.
After the procedure, the ulcer expanded.
To facilitate healing, surgical debridement was performed, which led to deterioration of the lesion and creation of a urethral fistula.
Plastic reconstruction of the fistula with a skin graft from the prepuce did not bring improvement and, eventually, the graft was rejected.
Plastic reconstruction of the fistula with a skin graft was performed for a second time without success and the patient was referred to our department for consultation1 month after the second procedure.
Physical examination revealed a circumferential, purulent ulceration involving the glans penis, the neck of the glans, and the adjacent prepuce and shaft of the penis.
The border of the lesion was edematous and violaceous.
A suprapubic catheter was in place.
No lymphadenopathy was present.
The patient denied any sexual intercourse and any history of trauma.
His personal and family history was unremarkable.
The following investigations were normal: completeblood cell count, urinalysis, renal and liver function tests,serum immunoglobulins, antinuclear antibodies, antineutrophilic cytoplasmic antibodies, serology for syphilis,herpes simplex virus, human immunodeficiency virus,hepatitis B and C virus, aerobic and anaerobic bacterial cultures, polymerase chain reaction for Mycobacterium tuberculosis from lesion tissue, chest radiography, and colonoscopy.
A biopsy had already been performed before his presentation to us and had revealed a mild, chronic inflammation in the dermis with a predominance of polymorphonuclear leukocytes.
On the basis of the clinical and histopathologic findings, the deterioration of the lesion after surgery, and the absence of infection or neoplasia, the patient was diagnosed with PG, and therapy with 250 mg cyclosporine and 16 mg ethylprednisolone was prescribed.
Methylprednisolone was tapered gradually and finally stopped after 3.
5 months.
The improvement in the lesion was evident from the ?rst week of treatment.
After 2 months,the suprapubic catheter was removed.
Four months after the initiation of treatment and while the patient was still taking 250 mg cyclosporine, the lesion area was free of any sign of in?ammation or ulcerations, but the fistula infection or malignancy.
Taking into account the urethral fistula, metastatic Crohn's disease was also considered as a possible diagnosis but was eventually excluded,because no noncaseating granulomas were found on biopsy, and, furthermore, surgical treatment of the ulcer deteriorated the lesion.
Colonoscopy also did not produce any abnormal findings.
Neutrophilic dermatoses are characterized histopathologically by a normal polymorphonuclear leukocyte infiltrate, and they are frequently associated with other systemic diseases.
PG includes four recognized types: ulcerative (the most common), pustular,bullous, and vegetative.
2 Ulcerative PG usually presents as a painful, necrolytic ulcer with an erythematous or violaceous, undermined border.
It expands rapidly, and it can be quite destructive.
PG most commonly appears on the lower extremities, but any cutaneous or mucocutaneous site can be affected.
Penile involvement is quite rare, but because the penis is a very tissue-sensitive area, prompt diagnosis and treatment is crucial.
We report the case of a 17-year-old, previously healthy, boy who was referred to our department by his urologist for a genital ulcer that had given rise to a urethral fistula that had failed to heal after two plastic reconstructions with skin grafts.
One year before his presentation to our department,the patient had noted a painful ulcer in the posterior surface of the neck of the glans that soon expanded to involve the frenulum.
He was prescribed topical treat ment with betamethasone valerate and fucidic acid, and the lesion improved.
After 1 month, a small erosion was still present in the frenulum, and it was decided to excise the frenulum.
After the procedure, the ulcer expanded.
To facilitate healing, surgical debridement was performed, which led to deterioration of the lesion and creation of a urethral fistula.
Plastic reconstruction of the fistula with a skin graft from the prepuce did not bring improvement and, eventually, the graft was rejected.
Plastic reconstruction of the fistula with a skin graft was performed for a second time without success and the patient was referred to our department for consultation1 month after the second procedure.
Physical examination revealed a circumferential, purulent ulceration involving the glans penis, the neck of the glans, and the adjacent prepuce and shaft of the penis.
The border of the lesion was edematous and violaceous.
A suprapubic catheter was in place.
No lymphadenopathy was present.
The patient denied any sexual intercourse and any history of trauma.
His personal and family history was unremarkable.
The following investigations were normal: completeblood cell count, urinalysis, renal and liver function tests,serum immunoglobulins, antinuclear antibodies, antineutrophilic cytoplasmic antibodies, serology for syphilis,herpes simplex virus, human immunodeficiency virus,hepatitis B and C virus, aerobic and anaerobic bacterial cultures, polymerase chain reaction for Mycobacterium tuberculosis from lesion tissue, chest radiography, and colonoscopy.
A biopsy had already been performed before his presentation to us and had revealed a mild, chronic inflammation in the dermis with a predominance of polymorphonuclear leukocytes.
On the basis of the clinical and histopathologic findings, the deterioration of the lesion after surgery, and the absence of infection or neoplasia, the patient was diagnosed with PG, and therapy with 250 mg cyclosporine and 16 mg ethylprednisolone was prescribed.
Methylprednisolone was tapered gradually and finally stopped after 3.
5 months.
The improvement in the lesion was evident from the ?rst week of treatment.
After 2 months,the suprapubic catheter was removed.
Four months after the initiation of treatment and while the patient was still taking 250 mg cyclosporine, the lesion area was free of any sign of in?ammation or ulcerations, but the fistula infection or malignancy.
Taking into account the urethral fistula, metastatic Crohn's disease was also considered as a possible diagnosis but was eventually excluded,because no noncaseating granulomas were found on biopsy, and, furthermore, surgical treatment of the ulcer deteriorated the lesion.
Colonoscopy also did not produce any abnormal findings.
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