Verrucous lesions over external genitalia as a presenting feature of pemphigus vegetans.

Pemhigus vegetans is a rare variant of pemphigus vulgaris which primarily occurs in the flexures of the body. We report a case of pemphigus vegetans in an 85-year-old female presenting with hypertrophic verrucous lesions over external genitalia and perianal region. There was no history of preceding oral lesions. The diagnosis of pemphigus vegetans was considered on the clinical ground and confirmed by histopathological examination. Copyright:

INTRODUCTION

Pemphigus vulgaris is a chronic vesiculobullous disorder with various phases or relapses and remittances.[1] One of the variant of pemphigus vulgaris is pemphigus vegetans, occurring in l%–5% of the patients.[2] It is most commonly caused by the production of autoantibodies against intercellular adhesion proteins desmoglein 1 and desmoglein 3.[34] Due to its rarity, it is often misdiagnosed and poses a diagnostic challenge if it occurs on locations seemingly uncommon. We report a case of pemphigus vegetans in an 85-year-old woman presenting to the dermatology outpatient department (OPD) with verrucous hypertrophic growth over the genitalia and perianal region.

CASE REPORT

An 85-year-old female patient presented to the dermatology OPD with the complaints of itchy growth over the external genitalia, perianal region, and left axilla for the past 4 months. It was also associated with painful defecation. She also developed thickening and crusting of the lips and fissuring of the tongue leading to intolerance to spicy food for the past 2 months. On detailed history taking, initially, fluid-filled lesions were present over the same sites which easily ruptured, leaving behind erosions. Subsequently, hypertrophic and verrucous lesions appeared over the external genitalia and perianal region. There was a past history of the appearance of similar lesions 20 years back at similar sites. She was diagnosed as a case of condyloma lata and treated with injection benzathine penicillin 3 months back. As there was no response, she was referred to our OPD. She was a chronic tobacco chewer.

On cutaneous examination, she had hyperpigmented, dry, verrucous, nontender plaques over the genitalia extending to the groin [Figure 1], perianal region [Figure 2], and the left axilla [Figure 3]. There were palpable bilateral discrete inguinal lymph nodes, firm in consistency. The lips were thickened along with fissuring and dry, adherent crustations. The tongue was enlarged with deep dorsal fissuring, suggestive of the cerebriform tongue [Figure 4]. The oral mucosa was pale. Systemic examination was within the normal limits. Complete blood count revealed hemoglobin level of 9.8 g/dL. Serum HIV and (RPR) Rapid Plasma Reagin were nonreactive. Local inguinal ultrasonography confirmed the presence of enlarged lymph nodes bilaterally. Punch biopsies were taken from lesions over the right inguinal region and axilla. Differentials considered included pyoderma vegetans, condyloma acuminata, pemphigus vegetans, and malignancy. Pus culture and sensitivity taken from the lesion through a sterile swab stick showed no growth at the end of 7 days.

Figure 1

Verrucous vegetating lesions on the vulva and groin at the time of presentation

Figure 2

Lesions around the anus and perineum at the time of presentation

Figure 3

Verrucous hypertrophic lesion in left underarm at the time of presentation

Figure 4

Thickening and fissuring of the lips and tongue at the time of presentation

Histopathological examination showed superficial and mid-perivascular and interstitial infiltrate of lymphocytes, neutrophils, and eosinophils with pseudocarcinomatous hyperplasia of the epidermis. Hyperplastic epidermis showed focal spongiosis with neutrophils and eosinophils, with suprabasal clefts at places with scant acantholysis [Figures 5 and 6]. The diagnosis of pemphigus vegetans was thus confirmed.

Figure 5

Hematoxylin and eosin stained cut section at ×4 magnification

Figure 6

Histopathological examination showing superficial and mid-perivascular and interstitial infiltrate of lymphocytes, neutrophils, and eosinophils with pseudocarcinomatous hyperplasia of the epidermis, along with hyperplastic epidermis showing focal spongiosis with neutrophils and eosinophils, with supra-basal clefts at places with scant acantholysis. (×10 magnification)

She was initially treated with oral prednisolone (30 mg) once a day, injectable cefotaxime (1 g) intravenous (IV) 8 hourly, and injectable metronidazole (400 mg) IV 8 hourly. Subsequently, prednisolone was tapered 5 mg every 15 days. Hematinics were given to treat the anemia. Topically, she was treated with clobetasol propionate (0.05%) and framycetin sulfate creams. On follow-up after 6 weeks, she improved markedly in the form of a reduction in the thickness of the lesions [Figure 7] and decrease in the intensity of pruritus. Significant improvement was present in the lesions over the lips and tongue [Figure 8].

Figure 7

View of the groin and vulva at 6-week follow-up, showing significant improvement

Figure 8

View of the lips and tongue at 6-week follow-up, showing significant improvement

DISCUSSION

Pemphigus vegetans is a relatively rare variant of pemphigus vulgaris, commonly with verrucous, cauliflower-like growth in the flexures of the body, and the periorificial regions, although cases with isolated involvement of the arms, feet, fingers, breasts, and oral cavity have been reported.[256] In this case, verrucous lesion over the external genitalia was presenting a feature of pemphigus vegetans and then involved the perianal region, axilla, and lips. In pemphigus vegetans, there is the development of antibodies against desmoglein 3 and/or desmoglein 1 along with antibodies against desmocollin 1, 2, and 3.[78]

Clinically, it is classified into two types – the Neumann type and the Hallopeau type.[9] In the Neumann type, the lesions begin as the classical lesions of pemphigus vulgaris and then form heaped-up vegetative plaques. In the Hallopeau type, the lesions begin as pustular lesions in groups, later leading to the formation of the heaped-up plaques. The Hallopeau type has a relatively benign course and responds to a lower dose of steroids and has prolonged remission, while the Neumann type needs higher doses of systemic corticosteroids and is resistant to treatment.[39] In our patient, benign course and good response to steroids were in favor of the diagnosis of Hallopeau type. Differential diagnoses of the lesions include vegetating herpes simplex infection, acrodermatitis continua suppurativa, pyoderma gangrenosum, pyoderma vegetans, Bowen's disease, squamous cell carcinoma, paraneoplastic pemphigus, epidermolysis bullosa acquisita, and pyodermatitis-pyostomatitis vegetans.[510] Positive serum anti-desmoglein 3 and immunofluorescence staining of the biopsy should be done to have a gold standard diagnosis, although it was not done in our case due to the nonavailability of antibody detection and staining method in the institute. In the present case, the diagnosis was confirmed by histopathological examination and response to systemic and topical corticosteroids. This case was unique, as the presenting feature of pemphigus vegetans was verrucous lesions over the external genitalia and then extending to the perianal region, groin, axilla, and lips.

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Conflicts of interest

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