Vegetating cutaneous plaques: a case report

¹Department of Dermatology, CHU Hassan II, Fez, Morocco, ²Pathology Department, CHU Hassan II, Fez, Morocco

^&Corresponding author
Selma El Kadiri, Department of Dermatology, CHU Hassan II, Fez, Morocco

Introduction    

Pemphigus vegetans represent only 1-2% of all cases of pemphigus [1]. It has two subtypes: Neumann and Hallopeau. The Neumann form initially begins with vesicular, flaccid bullae and erosive lesions resembling to a typical pemphigus vulgaris. Contrary to the less common Hallopeau type that starts with pustules and remains localized with a relatively benign course [2]. The disease affects the axilla, inframammary, intergluteal and inguinal folds inciting formation of vegetations maintained by maceration, friction and infection [3]. We report here a case of pemphigus vegetans with exclusive cutaneous vegetations.

Patient and observation     

A 44-year-old Moroccan female hospitalized for itchy lesions over inframammary fold, groin, perianal area and limbs. The skin lesions started with flaccid blisters that become crusted erosions for the past 6 months. They finally evolved into vegetating plaques. She denied systemic symptoms or the ingestion of suspicious drugs. The patient was previously treated by antibiotics without improvement. Laboratory tests were carried out and revealed diabetes put on metformin and glimepiride. Clinical examination revealed well-defined, multiple dark brown, indurated, superelevated plaques with verrucous and seborrheic-keratosis like surface over the infra-mammary area (Figure 1), back, both legs, genital and perianal area (Figure 2, Figure 3).

Nikolsky's sign was negative. Buccal, genital, nasal and ocular mucosae were spared. General physical and systemic examination revealed nothing abnormal. Complete blood count revealed peripheral eosinophilia. Histological examination showed acanthosis, papillomatosis, acantholysis and the presence of microabscesses containing eosinophils (Figure 4). Direct immunofluorescence showed intercellular immunoglobulin G (IgG) deposits in the epidermis. Indirect immunofluorescence revealed IgG autoantibodies directed against desmosomes. The diagnosis of pemphigus vegetans Neumann type was confirmed. She was treated with oral corticosteroid (0.5mg/kg per day) and rituximab, an anti-CD20 monoclonal antibody 1g 15 days apart with significant regression of the lesions (Figure 5, Figure 6, Figure 7).

Discussion     

Pemphigus vegetans is a rare disease that may begin with flaccid blisters that become erosions and form papillomatous proliferations localized especially in folds or on the scalp [1]. The tongue often shows cerebriform morphologic features early in the course of the disease [2]. Verrucous plaques on the scalp, fingers, perianal and genital areas have been reported as the mode of onset of the disease [3]. The lesions evolve to coalesce forming large patches into groups or figurate patterns [3]. In our case, the patient didn´t have mucosal or scalp involvement.

Our patient had lesions over the flexures and extensors with no involvement of the mucosa. She had a pattern of sulci, gyri over her lesions corresponding to a cerebriform pattern which may probably correspond to the papillary hyperplasia as reported once before by Rebello et al. [4]. Cases induced by the ingestion of the drugs such as captopril and enalapril and by the intranasal use of heroin and cocaine have been reported. The association with HIV infection and gastric cancer may have been fortuitous [3].

Pemphigus vegetans must be differentiated from other causes of vegetations such as halogenoderma which includes ioderma, bromoderma and fluoroderma, blastomycosis-like pyoderma, chromoblastomycosis, pyodermatitis-pyostomatitis vegetans, granuloma inguinal, condyloma lata and amebic granulomas specially when there is no involvement of the mucosa [3]. The laboratory findings, epidemiology, pathogenesis and treatment of pemphigus vegetans are the same as those for pemphigus vulgaris [4]. Desmocollin-3 antibodies have been reported in some cases and hypothesized to explain the different clinical features in vegetans versus vulgaris [5].

Conclusion     

We report a cerebriform pattern of cutaneous pemphigus vegetans with sulci and gyri sparing mucosa. So in routine pratices, extensive cerebriform lesions over the flexures and extensors too and also pemphigus vegetans should be kept as good differential.

Competing interests     

The authors declare no competing interests.

Authors' contributions     

All the authors have read and agreed to the final manuscript.

Figures     

Figure 1: verrucous and vegetating plaques over inframammary area

Figure 2: vegetative hyperkeratotic plaques in limbs and perianal area

Figure 3: perineal involvement with vegetative hyperkeratotic plaques in limbs

Figure 4: histological examination showed acanthosis, papillomatosis, acantholysis and the presence of microabscesses containing eosinophils

Figure 5: re-examination 3 months later showing clinical remission in the inframammary area

Figure 6: re-examination 3 months later showing clinical remission in the perianal area and the limbs

Figure 7: re-examination 3 months later showing clinical remission in the genital area and the limbs

References