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E-IJD® - CORRESPONDENCE
Year : 2021  |  Volume : 66  |  Issue : 4  |  Page : 447
Penile Pyoderma Gangrenosum: A Mimicker to Know


Department of Dermatology, Mohammed V University, Rabat, Morocco

Date of Web Publication17-Sep-2021

Correspondence Address:
Khallaayoune Mehdi
Department of Dermatology, Mohammed V University, Rabat
Morocco
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_717_20

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How to cite this article:
Mehdi K, Hind P, Mariame M, Karima S. Penile Pyoderma Gangrenosum: A Mimicker to Know. Indian J Dermatol 2021;66:447

How to cite this URL:
Mehdi K, Hind P, Mariame M, Karima S. Penile Pyoderma Gangrenosum: A Mimicker to Know. Indian J Dermatol [serial online] 2021 [cited 2021 Dec 2];66:447. Available from: https://www.e-ijd.org/text.asp?2021/66/4/447/326140




Sir,

Although pyoderma gangrenosum has a predilection for the lower extremities, it may actually affect any area of the skin. Penile pyoderma gangrenosum (PPG) is rare and has been poorly described. It remains unknown to many dermatologists and urologists, whereas misdiagnosis or delayed diagnosis may lead to mutilating lesions. Here, we report two cases of PPG with two different atypical presentations to raise awareness of this rare entity.


   Case 1 Top


An 85-year-old gentleman with a 10-year history of diabetes mellitus presented with a painful ulceration of the penis progressing for 3 months. He reported having an initial pruritus of the glans with a papular lesion rapidly turning to progressive painful ulceration. Clinical examination revealed a single 5-cm purulent ulceration of the glans with an indurated base [Figure 1]. No superficial lymphadenopathies were found and his general status was conserved.
Figure 1: Purulent ulceration of the glans

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A first surgical biopsy was performed showing nonspecific inflammatory changes. Sexually transmitted infections (STIs) testing was negative and no infective agent could be identified from local samples or skin biopsy culture. Other laboratory findings including complete blood count, plasma C-reactive protein (CRP), cytobacteriological exam of urine, and renal and liver function tests were all normal. Computed tomography (CT) urography showed no abnormalities in the urinary tract. During the hospitalization, the patient presented a peripheral pustular lesion on the lateral side of the glans. Histopathology revealed a neutrophilic dermal infiltrate with focal neutrophilic vasculitis without cytocalsia [Figure 2]. The pustule progressed to purulent ulceration indicating a clear pathergy phenomenon [Figure 3]. Based on these data, the diagnosis of PPG was made. Assessment for an underlying disease, such as inflammatory bowel disease or malignancy, was negative. Oral prednisone was started at 0.5mg/kg/day allowing a slow healing of the main ulceration after one month with a persistent peripheral lesion due to a pathergy phenomenon on the first surgical biopsy site [Figure 4]. After two months, the patient presented complete healing [Figure 5]. Prednisone was slowly tapered down and stopped after six months. No relapse has occurred in three years.
Figure 2: Dense neutrophilic dermal infiltrate with vascular alterations (HES 40)

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Figure 3: Pathergy phenomenon on the pustule biopsy site

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Figure 4: Delayed healing on the surgical biopsy site

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Figure 5: Complete healing of the lesions after two months of prednisone

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   Case 2 Top


A 61-year-old man with a history of diabetes mellitus complicated by renal dysfunction and ischemic heart disease was referred for evaluation of a necrosis of the glans progressing for one month. He related having an initial purulent nodule on the glans, which rapidly progressed to painful ulceration with necrotic lesions. Several courses of antibiotics were attempted before the patient was referred to a urologist, where a surgical debridement procedure was performed. Owing to postoperative extension of the lesions, a partial peniectomy surgery was proposed to the patient who refused and, then, was referred to a dermatologist. Physical examination revealed total necrosis of the glans with circumferential purulent ulceration of the corona [Figure 6]. No superficial lymphadenopathy was found and general status was conserved.
Figure 6: Total necrosis of the glans with ulceration of the corona

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Laboratory findings revealed a biological inflammatory syndrome with high CRP (140 mg/L) and neutrophilia (10000/mm3). Calcemia, phosphoremia, and parathormone (PTH) levels were all normal. STI testing was negative, whereas local microbiological samples showed rare insignificant mycelial filaments. Owing to patient's vascular history, an abdominopelvic CT angiography was performed showing no arterial stenosis. Testing for cryoglobulins was also negative. Skin biopsy revealed a nonspecific inflammatory infiltrate with numerous neutrophilic cells and neutrophilic vasculitis without cytoclasia [Figure 7]. No arteriolar calcification was noticed. Based on these data, a diagnosis of PPG was made and treatment with prednisone 0.5 mg/kg/day was given. A marked improvement in pain was obtained within a few days. Unfortunately, after 3 weeks, the patient presented an acute decompensated heart failure and finally died.
Figure 7: Vasculitis lesion without cytoclasia (HES×40)

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Since its first description in 1930 by Brunsting et al.,[1] only about 30 cases of PPG have been reported. Average age is around 50 years, but all ages might be affected. Clinical manifestations are variable, but the most common presentation is characterized by solitary chronic purulent painful ulceration of the glans and/or foreskin progressively enlarging and leading to mutilating lesions. Among the wide variety of presentations, a special consideration must be given to the necrotic form that may manifest with necrotic and extensive lesions mimicking Fournier's gangrene.[2] This latest form illustrated by our second patient might be challenging to diagnose and commonly lead to penile mutilations as patients are often systematically managed with debridement surgeries. Other variants include vegetative forms,[3] vesiculopustular lesions,[4] or, even, abscess of corpus cavernosum, which is considered by some authors to be an early form of PPG.[5]

The pathergy phenomenon is very common in PPG and has a considerable diagnostic value.[6] Worsening of the lesions and/or delayed healing following a surgical act were observed in both of our patients. drawing attention to PPG diagnosis. However, in many cases, aggressive debridement surgeries or excision are performed, resulting in considerable injury with major functional repercussions. Surgical acts should therefore be avoided in any case of suspicion of PPG.

Histopathology is not specific of PPG and not necessary for the diagnosis. Histological features may vary depending on the stage of the lesion and the biopsy site. Classic findings include dermal neutrophilic infiltrate, vascular alterations, necrosis, or, even, a granulomatous infiltrate.[7] Nonspecific inflammatory changes are also common. The interest of the biopsy for both of the patients was above all to exclude the other differential diagnoses of penile ulcerations and necrosis. In fact, PPG should be considered as a diagnosis of elimination that requires to exclude other penile conditions, especially STIs (syphilis, HIV, and HSV), tuberculosis, malignancy (carcinoma, melanoma, or lymphoma), calciphylaxis, erosive lichen, bowel disease, Behcet's disease, bullous disease (pemphigus vulgaris), or pathomimia.[7] Depending on the patient's initial clinical, biological, and histological findings, specific investigations to rule out one of these conditions should be performed. Necrotic PPG lesions may raise suspicion for Fournier's gangrene, calciphylaxis, vasculitis, or ischemic gangrene. Infectious gangrene was first suspected in our second patient; however, the diagnosis seemed unlikely as bacteriological findings were negative and no improvement was obtained after surgical debridement and several courses of antibiotics. Calcyphylaxis as well as ischemic gangrene of the glans were also considered, which then excluded basing on the absence of histological arteriolar calcification, normal PTH level, and normal abdominopelvic CT angiography.

In classic pyoderma gangrenosum, an underlying disease, such as inflammatory bowel disease, rheumatoid arthritis, or hematologic malignancy, is found in 50% of cases.[8] Incidence could be much lower in PPG as no association is found in the vast majority of reported cases. As such, PPG looks as a distinct entity from female genital pyoderma ganhrenosum, which is more commonly associated with systemic diseases and often associate extragenital cutaneous involvement.[9] The main associations reported with PPG are ulcerative colitis, renal tumors, myelodysplastic syndromes, and HIV.[7] In both of the cases, no systemic disease except diabetes mellitus was found.

Pharmacological management does not differ from classic pyoderma gangrenosum. General corticosteroid therapy is the first-line treatment. Topical corticosteroids or tacrolimus may be used in the superficial or pustular forms.[4],[10] Reconstructive surgery should be only considered far from the active phase.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Brunsting LA, Goeckerman WH, O'Leary PA. Pyoderma (ecthyma) gangrenosum. Clinical and experimental observations in five cases occurring in adults. Arch Dermatol Syphil 1930;22:655-80.  Back to cited text no. 1
    
2.
Baskin LS, Dixon C, Stoller ML, Carroll PR. Pyoderma gangrenosum presenting as Fournier's gangrene. J Urol 1990;144:984-6.  Back to cited text no. 2
    
3.
Preciado MM, Almeida JG, López AP. Vegetative pyoderma gangrenosum associated with renal failure. Rev Cubana Med Trop 2001;53:212-6.  Back to cited text no. 3
    
4.
Larsen CG, Thyssen JP. Pustular penile pyoderma gangrenosum successfully treated with topical tacrolimus ointment. Acta Derm Venereol 2012;92:104-5.  Back to cited text no. 4
    
5.
Lida K, Mizuno K, Kawai N, Ito E, Shintani Y, Morita A, et al. A case of abscess of corpus cavernosum as an early symptom of penile pyodermal gangrenosum: We propose the possibility of a new pathogenic finding. Hinyokika Kiyo 2015;61:115-9.  Back to cited text no. 5
    
6.
Ng E, Lee M, Dunglison N. Pyoderma gangrenosum of the penis: An important lesson. ANZ J Surg 2015;85:91-2.  Back to cited text no. 6
    
7.
Badgwell C, Rosen T. Penile pyoderma gangrenosum. Dermatol Online J 2006;12:8.  Back to cited text no. 7
    
8.
Hadi A, Lebwohl M. Clinical features of pyoderma gangrenosum and current diagnostic trends. J Am Acad Dermatol 2011;64:950-4.  Back to cited text no. 8
    
9.
Satoh M, Yamamoto T. Genital pyoderma gangrenosum: Report of two cases and published work review of Japanese cases. J Dermatol 2013;40:840-3.  Back to cited text no. 9
    
10.
Lally A, Hollowood K, Bunker C B, Turner R. Penile pyoderma gangrenosum treated with topical tacrolimus. Arch Dermatol 2005;141:1175-6.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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