E-IJD CASE REPORT

Pemphigoid Vegetans in Childhood: A Case Report and Short Review of Literature Yasmeen Khatib, Meena Makhija1, Richa D Patel, Gayatri Karad1

Abstract From the Departments of vegetans is a very rare type of which usually affects the Pathology and 1Dermatology, elderly and has not been reported in children. It shows a clinical resemblance to R. N. Cooper Hospital, Mumbai, vegetans but has distinct histological and immunopathological features of bullous pemphigoid. Maharashtra, India 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 Address for correspondence: the eyelids, periorbital, periauricular, perioral region and lips. She had oral lesions and a Dr. Yasmeen Khatib, cerebriform tongue. Though she showed clinical features of , histology Department of Pathology, revealed a subepidermal blister with the absence of . Direct immunofluorescence R. N. Cooper Hospital, studies were suggestive of bullous pemphigoid. On clinicopathological correlation, a diagnosis Mumbai – 400 056, Maharashtra, India. of pemphigoid vegetans was made. She responded well to oral corticosteroids and dapsone E‑mail: [email protected] therapy with complete resolution of the lesions.

Key Words: Childhood, dapsone, immunofluorescence, pemphigoid vegetans

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 plaques [Figure 1a]. She was treated with topical steroids Pemphigoid vegetans is a very rare variant of bullous and antibiotics which led to partial resolution of the pemphigoid characterized by vegetative and purulent lesions. After 2 months, similar lesions appeared along lesions located in groin, face, axillae, thigh, hands, eyelids with vesicles and pustules involving periorbital areas, and perioral areas. Winkelman and Su[1] first described eyelids, perioral region, periauricular area and vulva. She this entity in 1979 and till now 11 cases of this entity was treated with antibiotics, antiviral agents and topical have been described in the world literature.[1‑11] All cases steroids and showed partial resolution of lesions. Six have been described in adults with age ranging between months later, she presented with another recurrence of 23 and 89 years.[1‑11] We report a case of pemphigoid lesions to our outpatient department. On examination, vegetans in a 9‑year‑old girl and to our knowledge this multiple nontender, vesicopustules coalescing over a brown is the first case of childhood pemphigoid vegetans. The to black hypertrophic plaque with adherent yellow crust diagnosis was based on clinical, histopathological and and erosions were seen involving both eyebrows, eyelids, immunopathological features. lips and perioral areas [Figure 2a]. Vegetating lesions were seen on the vulva [Figure 2b]. Oral mucosa showed multiple Case Report ulcers with fissured cerebriform tongue [Figure 1b]. A 9‑year‑old girl presented with complaints of recurrent There was no positive family history. There was no multiple pruritic vesiculopustular and hypertrophic raised history of fever, diarrhoea or drug intake. A differential lesions involving forehead, eyelids, periorbital region, diagnosis of pemphigus vegetans and chronic bullous perioral areas, lips, vulva and groin since 1 year. She also disease of childhood was considered. All hematological complained of ulcers in the oral cavity. The lesions first and biochemical investigations were normal except for started on the forehead, periorbital region, vulva and an eosinophilia of 16%. Pus for culture was negative. groin. They gradually enlarged into purulent vegetating Serum IgG, IgM, IgA and complement levels were normal. Serum IgE levels were raised [588 IU/ml]. Antidesmoglein Access this article online antibody 1 (anti‑Dsg‑1) was 4.6 U/ml (Normal value < 14) Quick Response Code: and antidesmoglein antibody 3 (anti‑Dsg‑3) was Website: www.e‑ijd.org 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 DOI: 10.4103/0019-5154.160534 of eosinophils and neutrophils. Surrounding epidermis showed epidermal hyperplasia with acanthosis and

Indian Journal of 2015; 60(4) 86 Khatib, et al.: Childhood pemphigoid vegetans elongation of rete ridges. No acantholysis was seen childhood. Pemphigoid vegetans has not been reported [Figure 3a]. Direct immunofluorescence of perilesional in children till now, ours being the first case. Our skin showed linear deposition of IgG at the basement patient developed vegetating and vesiculopustular membrane zone (BMZ). Specimen was further treated with lesions along with oral lesions. The common causes of 1M NaCl and section of salt split skin were treated with these type of lesions are pemphigus vegetans, pyoderma IgG. The BMZ band was seen on the epidermal side [roof vegetans, pyodermatitis, pyostomatitis vegetans and pattern] of the split confirming the diagnosis of bullous rarely cicatricial and pemphigoid vegetans. Though these pemphigoid [Figure 3b]. lesions can have overlapping clinical features, histology Hence, a diagnosis of pemphigoid vegetans was made and immunofluorescence studies will distinguish these based on the clinical, histological and immunofluorescent conditions from each other. On review of the 11 cases features. The patient was put on dapsone 100 mg once daily of pemphigoid vegetans reported earlier, the age ranged [1‑11] along with tablet prednisolone 30 mg for 2 weeks followed from 23 to 89 years [Table 1]. All patients presented by tapering of dose by 5 mg every fortnight. There was with intertriginous vegetating plaques but vesicles and complete resolution of lesions with hyperpigmentation, pustules were seen in 7 out of 11 cases.[7,11] Mucosal scarring and milia formation [Figure 4]. involvement has been seen in 6 out of 11 cases[7] and serum eosinophilia has been documented in four Discussion cases.[1,4,7,11] Our case has shown both these features. Two [1,2] Bullous pemphigoid is a blistering autoimmune cases have shown inflammatory bowel disease. Site of disease of the elderly with very few cases reported in involvement has been localized in some cases and has been more extensive in others, as in the present case.

a b a Figure 1: (a) Purulent vegetating lesions on the face at first presentation. (b) b Cerebriform tongue showing fissuring Figure 2: (a) Crusted bullous and purulent lesions on eyelids, lips and perioral regions. (b) vegetating lesions on vulva

a b a Figure 3: (a) Subepidermal bulla formation with epidermal hyperplasia of the adjacent b epidermis. (b) Direct immunofluorescence showing linear deposition of IgG on the Figure 4: (a) Complete resolution of the lesions on the face with hyperpigmentation and epidermal side salt split skin milia formation (b) Complete resolution of the lesions on vulva showing hyperpigmentation

87 Indian Journal of Dermatology 2015; 60(4) Khatib, et al.: Childhood pemphigoid vegetans

They include groin, vulva, axillae, face, eyelids, periorbital areas, perioral areas, lips and umbilicus.[7] Subepidermal blister formation with epidermal hyperplasia has been 12 F 9 + + + 12% Ig G − Not done Not done Present case 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,[5,9,10,11] One patient reacted with BP230 and the [5]

11 F 89 + + − 17% C3 and IgG + + + Nagamoto et al. 160 kDa, 130 kDa and 95 kDa proteins, while two other patients have shown the presence of both BP230 and BP180 antigens by immunoblot and ELISA technique.[9,11] One case has shown features of both pemphigus and pemphigoid vegetans with dual reactivity to both BP230

10 F 85 + − − N C3 and IgG + + − Hatano et al. 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,

9 F 83 + − − N C3 and IgG + + + T Suda histological features and direct immunofluorescence (DIF) et al. 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

8 M 57 + − − N/A Ig G − Not done Not done Delpuget et al. corticosteroids in four cases,[3,4,9,10] topical steroid with antibiotic in three cases,[2,7,11] dapsone in one case[5] and oral corticosteroids in one case.[6] Four cases have shown complete resolution,[5,7,10,11] two cases have shown relapse 7 M 79 + + − 12% IgG + Not done Not done Kim J et al. after treatment[4,6] and three cases have not completely resolved.[2,3,9] 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 6 F 76 + + + N/A and IgA IgG, C3 _ Not done Not done Ogasawara et al. of this lesion. Our case is the first case of childhood pemphigoid vegetans to be reported.

5 F 77 + + + N/A IgG + + − Chan et al. 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.

4 M 75 + − + 23% and IgG C3 + Not done Not done Ueda et al. References 1. Winkelmann R, Su W. Pemphigoid vegetans. Arch Dermatol 1979;115:446‑8.

3 M 82 + − + N/A and IgG C3 + Not done Not done Al‑Najjar et al. 2. Kuokkanen K, Helin H. Pemphigoid vegetans. Report of a case. Arch Dermatol 1981;117:56‑7.

1: Summary of previously reported cases of pemphigoid vegetans reported 1: Summary of previously Table 3. Al‑Najjar A, Reilly GD, Bleehen SS. Pemphigoid vegetans: A case report. Acta Derm Venereol 1984;64:450‑2.

2 F 28 + + + 2% C3 and IgG − Not done Not done Kuokkanen Helin and 4. Ueda Y, Nashiro K, Seki Y, Otsuka F, Tamaki K, Ishibashi Y. Pemphigoid vegetans. Br J Dermatol 1989;120:449‑53. 5. Chan LS, Dorman MA, Agha A, Suzuki T, Cooper KD, Hashimoto K. Pemphigoid vegetans represents a bullous pemphigoid variant. Patient’s IgG autoantibodies identify

1 F 23 + + + 20% M Ig IgG, C3 and + Not done Not done Winkelmann Su and the major bullous pemphigoid antigen. J Am Acad Dermatol 1993;28:331‑5. 6. Ogasawara M, Matsuda S, Nishioka K, Asagami C. Pemphigoid vegetans. J Am Acad Dermatol 1994;30:649‑50. 7. Kim J, Chavel S, Girardi M, McNiff J. Pemphigoid vegetans: A case report and review of the literature. J Cutan Pathol 2008;35:1144‑7. 8. Delpuget B, Bernad P, Bedane C, Boulinguez S, Garnier A, Bonnetblanc JM. Pemphigoid vegetans. An immunoelectron

Case number Sex (years) Age lesions Vegetative lesions Purulent involvement Mucosal count Eosinophil DIF* IIF** BP230***autoantibodies BP180****autoantibodies Reference antibody 180 pemphigoid ****BP 180: Bullous antibody, 230 pemphigoid ***BP 230: Bullous immunofluorescence, **IIF: Indirect immunofluorescence, *DIF: Direct microscopic study. Ann Dermatol Venereol 1997;124:467‑9.

Indian Journal of Dermatology 2015; 60(4) 88 Khatib, et al.: Childhood pemphigoid vegetans

9. Suda‑Takayanagi T, Hara H, Ohyama B, Hashimoto T, Terui T. Hashimoto T, et al. Case of pemphigoid vegetans positive A case of pemphigoid vegetans with autoantibodies against with both BP180 and BP230 in enzyme‑linked immunosorbent both BP180 and BP 230 antigens. J Am Acad Dermatol assays J Dermatol 2014;41:667‑8. 2011;64:206‑8. 10. Hatano Y, Ishikawa K, Koga H, Ishii N, Hashimoto T, Takeo N, How to cite this article: Khatib Y, Makhija M, Patel RD, Karad G. et al. A case of concurrent pemphigoid vegetans and Pemphigoid vegetans in childhood: A case report and short review of pemphigus vegetans remitted without oral corticosteroid. Br J literature. Indian J Dermatol 2015;60:422. Dermatol 2014;170:1192‑4. Received: August, 2014. Accepted: November, 2014. 11. Nagamoto E, Fujisawa A, Jinnin M, Koga H, Ishii N, Source of support: Nil, Conflict of Interest: Nil.

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