Indian Journal of Dermatology
: 2016  |  Volume : 61  |  Issue : 2  |  Page : 239-

Pyodermatitis-pyostomatitis vegetans with penile ulceration complicated by acute glomerulonephritis

Anza Khader1, Betsy Ambooken2, Kunnummal Muhammed1, Manikoth P Binitha1, Deepthi N Sureshan1, Shyni P Manakkad3,  
1 Department of Dermatology and Venereology, Government Medical College, Kozhikode, India
2 Department of Dermatology and Venereology, Government Medical College, Thrissur, Kerala, India
3 Department of Pathology, Government Medical College, Kozhikode, India

Correspondence Address:
Anza Khader
From the Department of Dermatology and Venereology, Government Medical College, Kozhikode

How to cite this article:
Khader A, Ambooken B, Muhammed K, Binitha MP, Sureshan DN, Manakkad SP. Pyodermatitis-pyostomatitis vegetans with penile ulceration complicated by acute glomerulonephritis.Indian J Dermatol 2016;61:239-239

How to cite this URL:
Khader A, Ambooken B, Muhammed K, Binitha MP, Sureshan DN, Manakkad SP. Pyodermatitis-pyostomatitis vegetans with penile ulceration complicated by acute glomerulonephritis. Indian J Dermatol [serial online] 2016 [cited 2021 Dec 6 ];61:239-239
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Full Text


Pyodermatitis-pyostomatitis vegetans (PD-PSV) is a rare inflammatory mucocutaneous dermatoses characterized by annular vegetative plaques of intertriginous areas and snail-track ulcers of oral mucosa. It is considered as a specific marker of ulcerative colitis.[1]

A 21-year-old man, who had a 5-year history of recurrent ulceration of the left groin, presented with ulcers over the glans penis of 2-month duration. For the past 1 month, he had recurrent episodes of abdominal pain and diarrhea. There was no history of trauma or sexual contact prior to the onset of the lesions. On examination, he appeared emaciated and had pallor, bilateral pitting pedal edema, bilateral inguinal lymph node enlargement, and a high blood pressure of 170/100 mmHg. There was a large erythematous annular plaque 30 cm × 15 cm in size, extending from the left inguinal area to the upper thigh with linear scars in a rippled pattern. He also had two irregular linear ulcers covered with purulent discharge, pustulation and multiple depressed scars on the glans penis [Figure 1]a and [Figure 1]b.{Figure 1}

Laboratory investigations demonstrated a low hemoglobin level of 8.8 g%, a total leucocyte count of 10,000 cells/mm 3 with 10% eosinophils and a raised erythrocyte sedimentation rate of 142 mm in the 1st h. Blood urea was 34 mg/dL and serum creatinine 1.1 mg/dL. Liver function tests were normal except for a low albumin level of 2.9 g/dL. Urine microscopy showed albumin, red blood cells, and casts. A stool examination was negative for ova, cysts, and occult blood. Screening for hepatitis and human immunodeficiency viruses, chest X-ray, pathergy, and Mantoux tests was negative. Tissue smears from the ulcers stained with Leishman and Ziehl–Neelsen stains showed no organisms. Skin biopsy showed pseudoepitheliomatous hyperplasia, acanthosis, neutrophilic abscess, and eosinophilic infiltrate in the dermis [Figure 2]a and [Figure 2]b. Direct immunofluorescence from perilesional skin was negative.{Figure 2}

Ultrasonography of abdomen and pelvis showed edematous thickening of the large bowel loops and acute bilateral Grade 1 renal parenchymal damage. Serum C3, antinuclear antibodies, and C-antineutrophil cytoplasmic antibodies were negative. Flexible sigmoidoscopy revealed multiple discrete ulcers up to the splenic flexure, which on biopsy showed ulceration, congestion, and inflammation of the lamina propria without granulomas.

The patient was treated with intravenous dexamethasone 8 mg daily, which was tapered over 3 weeks. There was complete healing of lesions within 3 weeks and no recurrence after 1 year of follow-up [Figure 3].{Figure 3}

PD-PSV is a distinct chronic pustular dermatoses presenting with cutaneous and/or oral lesions. The oral cavity is not always affected though it may be the sole manifestation. The skin lesions usually involve the intertriginous areas such as axillae and groin in the form of annular vegetating plaques fringed with pustules. The “snail track” appearance of oral lesions occurs due to coalescence of eroded lesions to form folded linear ulcers. The disease is common among whites between 15 and 31 years of age, with a male to female ratio of 3:1.[1] There is a strong association as high as 70% with inflammatory bowel disease (IBD), especially ulcerative colitis, although the exact etiology remains unexplained.

Peripheral eosinophilia is seen in 90% cases and is a valuable aid to the diagnosis. Associated liver dysfunction such as sclerosing cholangitis, chronic hepatitis, and pericholangitis may be seen.[1] Zinc deficiency has been reported to occur with the disease.[2]

Histological changes include pseudoepitheliomatous hyperplasia, hyperkeratosis, acanthosis, intra or subepithelial abscesses containing eosinophils and neutrophils. Older lesions are less likely to demonstrate abundant eosinophils. Direct immunofluorescence is usually negative.[1]

PD-PSV has been variably considered under pyoderma gangrenosum and pemphigus vegetans.[3] A literature search for PD-PSV very often led to “pyodermite vegetante” coined by Hallopeau. Hallopeau classified his cases as a benign form of Neumann's pemphigus vegetans, a finding which was later proved correct by positive results, on direct immunofluorescence specimens.[4]

Vegetative pyoderma gangrenosum or superficial granulomatous pyoderma presents as relatively nonpainful exophytic lesions not associated with systemic disease and histologically shows a three-layered granuloma.[5]

Pyoderma vegetans or blastomycosis-like pyoderma generally presents with verrucous exudative plaques in immunocompromised patients.[6]

We suggest that PD-PSV should be considered as a discrete entity occurring in patients with IBD, characterized by annular plaques in the intertriginous areas, with or without mucosal involvement, either oral or genital, with peripheral eosinophilia, eosinophilic, and neutrophilic infiltrate in the epidermis or dermis without granulomas, and with negative immunofluorescence findings. To the best of our knowledge, there have been no previous reports of PD-PSV complicated by glomerulonephritis.

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