Pemphigoid Vegetans in Childhood: A Case Report and Short Review of Literature.

Pemphigoid vegetans is a very rare type of bullous pemphigoid which usually affects the elderly and has not been reported in children. It shows a clinical resemblance to pemphigus vegetans but has distinct histological and immunopathological features of bullous pemphigoid. A 9-year-old girl presented with recurrent purulent and verrucous vegetating lesions on her forehead, groin and vulva along with scaling, crusted, bullous and purulent lesions on the eyelids, periorbital, periauricular, perioral region and lips. She had oral lesions and a cerebriform tongue. Though she showed clinical features of pemphigus vegetans, histology revealed a subepidermal blister with the absence of acantholysis. Direct immunofluorescence studies were suggestive of bullous pemphigoid. On clinicopathological correlation, a diagnosis of pemphigoid vegetans was made. She responded well to oral corticosteroids and dapsone therapy with complete resolution of the lesions.

What was known?

Pemphigoid vegetans is a rare variant of bullous pemphigoid characterized by vegetative and purulent lesions.

Clinically it resembles pemphigus vegetans but histology and immunofluorescence findings are suggestive of bullous pemphigoid.

Introduction

Pemphigoid vegetans is a very rare variant of bullous pemphigoid characterized by vegetative and purulent lesions located in groin, face, axillae, thigh, hands, eyelids and perioral areas. Winkelman and Su[1] first described this entity in 1979 and till now 11 cases of this entity have been described in the world literature.[1234567891011] All cases have been described in adults with age ranging between 23 and 89 years.[1234567891011] We report a case of pemphigoid vegetans in a 9-year-old girl and to our knowledge this is the first case of childhood pemphigoid vegetans. The diagnosis was based on clinical, histopathological and immunopathological features.

Case Report

A 9-year-old girl presented with complaints of recurrent multiple pruritic vesiculopustular and hypertrophic raised lesions involving forehead, eyelids, periorbital region, perioral areas, lips, vulva and groin since 1 year. She also complained of ulcers in the oral cavity. The lesions first started on the forehead, periorbital region, vulva and groin. They gradually enlarged into purulent vegetating plaques [Figure 1a]. She was treated with topical steroids and antibiotics which led to partial resolution of the lesions. After 2 months, similar lesions appeared along with vesicles and pustules involving periorbital areas, eyelids, perioral region, periauricular area and vulva. She was treated with antibiotics, antiviral agents and topical steroids and showed partial resolution of lesions. Six months later, she presented with another recurrence of lesions to our outpatient department. On examination, multiple nontender, vesicopustules coalescing over a brown to black hypertrophic plaque with adherent yellow crust and erosions were seen involving both eyebrows, eyelids, lips and perioral areas [Figure 2a]. Vegetating lesions were seen on the vulva [Figure 2b]. Oral mucosa showed multiple ulcers with fissured cerebriform tongue [Figure 1b]. There was no positive family history. There was no history of fever, diarrhoea or drug intake. A differential diagnosis of pemphigus vegetans and chronic bullous disease of childhood was considered. All hematological and biochemical investigations were normal except for an eosinophilia of 16%. Pus for culture was negative. Serum IgG, IgM, IgA and complement levels were normal. Serum IgE levels were raised [588 IU/ml]. Antidesmoglein antibody 1 (anti-Dsg-1) was 4.6 U/ml (Normal value < 14) and antidesmoglein antibody 3 (anti-Dsg-3) was 6.62 U/ml (normal value < 7) detected by enzyme-linked immunosorbent assay (ELISA). A skin biopsy showed the presence of a subepidermal bulla with the presence of eosinophils and neutrophils. Surrounding epidermis showed epidermal hyperplasia with acanthosis and elongation of rete ridges. No acantholysis was seen [Figure 3a]. Direct immunofluorescence of perilesional skin showed linear deposition of IgG at the basement membrane zone (BMZ). Specimen was further treated with 1M NaCl and section of salt split skin were treated with IgG. The BMZ band was seen on the epidermal side [roof pattern] of the split confirming the diagnosis of bullous pemphigoid [Figure 3b].

Figure 1

(a) Purulent vegetating lesions on the face atfirst presentation. (b) Cerebriform tongue showing fissuring

Figure 2

(a) Crusted bullous and purulent lesions on eyelids, lips and perioral regions. (b) vegetating lesions on vulva

Figure 3

(a) Subepidermal bulla formation with epidermal hyperplasia of the adjacent epidermis. (b) Direct immunofluorescence showing linear deposition of IgG on the epidermal side salt split skin

Hence, a diagnosis of pemphigoid vegetans was made based on the clinical, histological and immunofluorescent features. The patient was put on dapsone 100 mg once daily along with tablet prednisolone 30 mg for 2 weeks followed by tapering of dose by 5 mg every fortnight. There was complete resolution of lesions with hyperpigmentation, scarring and milia formation [Figure 4].

Figure 4

(a) Complete resolution of the lesions on the face with hyperpigmentation and milia formation (b) Complete resolution of the lesions on vulva showing hyperpigmentation

Discussion

Bullous pemphigoid is a blistering autoimmune disease of the elderly with very few cases reported in childhood. Pemphigoid vegetans has not been reported in children till now, ours being the first case. Our patient developed vegetating and vesiculopustular lesions along with oral lesions. The common causes of these type of lesions are pemphigus vegetans, pyoderma vegetans, pyodermatitis, pyostomatitis vegetans and rarely cicatricial and pemphigoid vegetans. Though these lesions can have overlapping clinical features, histology and immunofluorescence studies will distinguish these conditions from each other. On review of the 11 cases of pemphigoid vegetans reported earlier, the age ranged from 23 to 89 years [Table 1].[1234567891011] All patients presented with intertriginous vegetating plaques but vesicles and pustules were seen in 7 out of 11 cases.[711] Mucosal involvement has been seen in 6 out of 11 cases[7] and serum eosinophilia has been documented in four cases.[14711] Our case has shown both these features. Two cases have shown inflammatory bowel disease.[12] Site of involvement has been localized in some cases and has been more extensive in others, as in the present case. They include groin, vulva, axillae, face, eyelids, periorbital areas, perioral areas, lips and umbilicus.[7] Subepidermal blister formation with epidermal hyperplasia has been a consistent feature in all cases. Direct and indirect immunofluorescence was typical of bullous pemphigoid in all cases. Target antigens have been reported in only four cases,[591011] One patient reacted with BP230 and the 160 kDa, 130 kDa and 95 kDa proteins,[5] while two other patients have shown the presence of both BP230 and BP180 antigens by immunoblot and ELISA technique.[911] One case has shown features of both pemphigus and pemphigoid vegetans with dual reactivity to both BP230 and Dsg1 protein.[10] Direct immunoelectron microscopy was helpful in diagnosis of one case.[8] Our case was similar to other cases regarding site, type of lesions, histological features and direct immunofluorescence (DIF) pattern. The presence of cerebriform tongue in our case has not been reported in earlier cases. Various therapies have been used for the treatment of this entity. Topical antibiotic has been used in one case,[1] topical corticosteroids in four cases,[34910] topical steroid with antibiotic in three cases,[2711] dapsone in one case[5] and oral corticosteroids in one case.[6] Four cases have shown complete resolution,[571011] two cases have shown relapse after treatment[46] and three cases have not completely resolved.[239] To conclude, pemphigoid vegetans is a very rare entity marked by vegetative and purulent lesions clinically resembling other vegetative lesions. Immunofluorescence studies are critical for diagnosis of this lesion. Our case is the first case of childhood pemphigoid vegetans to be reported.

Table 1

Summary of previously reported cases of pemphigoid vegetans

What is new?

Only 11 cases of pemphigoid vegetans have been reported in the world literature. We report the first case of pemphigoid vegetans in a child.