Indian Journal of Dermatology
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CASE REPORTS
Year : 2005  |  Volume : 50  |  Issue : 3  |  Page : 166-167
Pemphigus vegetans of hallopeau


Department of Dermatology and STD, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi-110 002, India

Correspondence Address:
Soni Nanda
Department of Dermatology and STD, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi-110 002
India
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   Abstract 

A 42 year old male had pemphigus vegetans of Hallopeau of groin and perianal area for the last 4 months. He also had lesions on scalp and mouth. Histology showed intraepidermal eosinophilic abscess.


Keywords: Pemphigus vegetans, Hallopeau type


How to cite this article:
Nanda S, Grover C, Garg VK, Reddy B S. Pemphigus vegetans of hallopeau. Indian J Dermatol 2005;50:166-7

How to cite this URL:
Nanda S, Grover C, Garg VK, Reddy B S. Pemphigus vegetans of hallopeau. Indian J Dermatol [serial online] 2005 [cited 2019 Aug 21];50:166-7. Available from: http://www.e-ijd.org/text.asp?2005/50/3/166/18935



   Introduction Top


Pemphigus vegetans is an uncommon variant of pemphigus vulgaris (PV), comprising only 1% to 20% of cases.[1] It is characterized by blisters and erosions associated with verrucous vegetations. It has been divided into Hallopeau and Neumann types. The cutaneous lesions in the Hallopeau type begin as pustules and heal as vegetating plaques while those in Neumann type, with a worse prognosis, are characterized by vegetations developing during the course of PV.[1] Vegetating lesions seem to be a reactive pattern of the skin to the auto-immune insult of the PV, with certain areas of the skin showing more of a tendency to form vegetations. Local moisture, heat and friction are important factors in their development. The sites involved are the intertriginous areas (the axillae, groins and inframammary folds), scalp and the face. Oral involvement is reported in 60-80% of PV, but has been stated to be present in pemphigus vegetans with verifying frequencies.[1]-[3] Cerebriform tongue, characterized by a pattern of sulci and gyri on the dorsum of tongue has been reported in up to 50% cases of Neumann type.[4]


   Case report Top


A 42 year old male had raised, mildly itchy, skin colored lesions in the groins and perianal area for the past 4 months. He also gave history of recurrent pus filled lesions over the scalp and oral ulceraions for the past 5 years. There was no history of any fluid filled lesion or any genital ulcer in the past. Patient denied any history of extra-marital sex contact. There was no history of recurrent abortions in wife. Family history of similar lesions was not present.

Muco-cutaneous examination revealed multiple, skin colored, macerated papules over the peri-anal region and moist, vegetating, oozing plaques present in the left groin [Figure - 1].

Patient had inguinal lymphadenopathy 1x1 cm, discrete, non-tender, mobile and firm with normal overlying skin. Examination of the genitalia revealed no abnormality. Scalp showed violaceous plaques studded with pustules but there was no evidence of cicatricial alopecia. Oral mucosa examination revealed violaceous plaques over the buccal mucosa and hard palate. Rest of the muco-cutaneous and systemic examinations were within normal limits.

Dark ground illumination, both from the lesion and the lymph nodes, was found to be negative on three consecutive days. VDRL in serial dilutions was non-reactive and TPHA was negative. Tzanck smear showed eosinophils but no acantholytic cell. A skin biopsy was done from peri-anal papule and sent for histopathological examination. Histopathology revealed intraepidermal eosinophilic abscesses, few acantholytic cells and a supra-basal cleft. Dermis showed intense inflammatory infiltrate comprising of plasma cells and eosinophils [Figure - 2]. Immuno-fluorescence studies could not be performed due to lack of facility.

A diagnosis of pemphigus vegetans of Hallopeau was established on the basis of classical clinical and histopathological picture. The patient was treated with systemic steroids (1 mg/kg body wt/day ) and showed significant improvement with drying up of lesions in 6 weeks.


   Discussion Top


Diagnosis in the present case was established on the basis of characteristic clinical morphology in the form of vegetating papules to plaques in the groins and perianal region, involvement of the oral mucosa and scalp, negative DGI and VDRL, eosinophilic micro-abscesses with acantholytic cells in the epidermis and dense inflammatory infiltrate in the dermis as seen in pemphigus vegetans.[5] The diagnosis was further confirmed by the excellent response to steroids.

Condyloma lata form the most important differential diagnosis. These are the extremely infectious lesions of secondary syphilis which present as flesh colored moist oozing papules in the intertriginous areas. These lesions are often pruritic and might persist for months. These are often confused with condyloma acuminata and hemorrhoids.[6] The presence of numerous plasma cells with perivascular cuffing in histopathology, further complicated the picture. But plasma cells could be a histopathological feature of most of the cases with chronic dermatitis. The diagnosis of condyloma lata can be established on the basis of a positive DGI, a reactive VDRL and a eharacteristic histopathology in the form an intense plasma cell infiltrate with obliterative endarteritis and capillary proliferation in the dermis.

The Hallopeau type has a relatively benign course and responds to topical therapy, low dose steroids and occasionally to dapsone while the Neumann type has a poor prognosis and is resistant to treatment.

We would like to emphasize that, though rare, a differential diagnosis of pemphigus vegetans should be kept in mind in cases of condyloma lata, especially in those not responding to therapy.



 
   References Top

1.Ahmed AR, Blose DA. Pemphigus vegetans. Neumann type and Hallopeau type. Int J Dermatol 1984;23:135-41.  Back to cited text no. 1    
2.Downie JB, Dicostanzo DP, Cohen SR. Pemphigus vegetans - Neumann variant associated with intranasal heroin abuse. J Am Acad Dermatol 1998 ;39: 872-5.  Back to cited text no. 2    
3.Nelson CG. Pemphigus vegetans of Hallopeau. Arch Dermatol 1978; 114:627-8.  Back to cited text no. 3    
4.Premlatha S, Jayakumar S, Yesudan P, Thambiah AS. Cerebriform tongue: a clinical sign in pemphigus vegetans. Br J Dermatol 1981; 104: 587-91.  Back to cited text no. 4    
5.Lever WF, Schaunburg-Lever G. Histopathology of skin. 7th ed. Philadelphia: Lippincott,1990:121-2.  Back to cited text no. 5    
6.Rook A, Wilkinson DS, Ebling FGH, et al. Textbook of Dermatology, 5th ed. Oxford: Blackwell Science, 1992:1638-45.  Back to cited text no. 6    


Figures

[Figure - 1], [Figure - 2]



 

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    Abstract
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    Case report
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    References
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