Indian J Dermatol. 2015 Jul-Aug; 60(4): 422.

PMCID: PMC4533577
doi: 10.4103/0019-5154.160534: 10.4103/0019-5154.160534 PMID: 26288447

Pemphigoid Vegetans in Childhood: A Case Report and Short

Review of Literature
Yasmeen Khatib, Meena Makhija,1 Richa D Patel, and Gayatri Karad1

From the Department of Pathology, R. N. Cooper Hospital, Mumbai, Maharashtra, India

1
Department of Dermatology, R. N. Cooper Hospital, Mumbai, Maharashtra, India
Address for correspondence: Dr. Yasmeen Khatib, Department of Pathology, R. N.
Cooper Hospital, Mumbai - 400 056, Maharashtra, India. E-mail: [email protected]

Received 2014 Aug; Accepted 2014 Nov.

Copyright : © Indian Journal of Dermatology

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Abstract

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.

Keywords: 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

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.
[1,2,3,4,5,6,7,8,9,10,11] All cases have been described in adults with
age ranging between 23 and 89 years.[1,2,3,4,5,6,7,8,9,10,11] 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].
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].

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].[1,2,3,4,5,6,7,8,9,10,11] All patients presented with
intertriginous vegetating plaques but vesicles and pustules were
seen in 7 out of 11 cases.[7,11] Mucosal involvement has been seen
in 6 out of 11 cases[7] and serum eosinophilia has been documented
in four cases.[1,4,7,11] Our case has shown both these features. Two
cases have shown inflammatory bowel disease.[1,2] 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,
[5,9,10,11] 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.[9,11] 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,[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 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 of this lesion. Our case is the first case of childhood
pemphigoid vegetans to be reported.

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.

Footnotes
Source of support: Nil
Conflict of Interest: Nil.

References

1. Winkelmann R, Su W. Pemphigoid vegetans. Arch Dermatol.

1979;115:446–8. [PubMed: 373640]
2. Kuokkanen K, Helin H. Pemphigoid vegetans. Report of a case.
Arch Dermatol. 1981;117:56–7. [PubMed: 7006518]

3. Al-Najjar A, Reilly GD, Bleehen SS. Pemphigoid vegetans: A case

report. Acta Derm Venereol. 1984;64:450–2. [PubMed: 6208733]

4. Ueda Y, Nashiro K, Seki Y, Otsuka F, Tamaki K, Ishibashi Y.

Pemphigoid vegetans. Br J Dermatol. 1989;120:449–53. [PubMed:
2653403]

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 the major bullous pemphigoid
antigen. J Am Acad Dermatol. 1993;28:331–5. [PubMed: 8436652]

6. Ogasawara M, Matsuda S, Nishioka K, Asagami C. Pemphigoid

vegetans. J Am Acad Dermatol. 1994;30:649–50. [PubMed: 8157794]

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.
[PubMed: 18988318]

8. Delpuget B, Bernad P, Bedane C, Boulinguez S, Garnier A,

Bonnetblanc JM. Pemphigoid vegetans. An immunoelectron
microscopic study. Ann Dermatol Venereol. 1997;124:467–9.
[PubMed: 9739912]

9. Suda-Takayanagi T, Hara H, Ohyama B, Hashimoto T, Terui T. A

case of pemphigoid vegetans with autoantibodies against both
BP180 and BP 230 antigens. J Am Acad Dermatol. 2011;64:206–8.
[PubMed: 21167421]

10. Hatano Y, Ishikawa K, Koga H, Ishii N, Hashimoto T, Takeo N, et

al. A case of concurrent pemphigoid vegetans and pemphigus
vegetans remitted without oral corticosteroid. Br J Dermatol.
2014;170:1192–4. [PubMed: 24359309]
11. Nagamoto E, Fujisawa A, Jinnin M, Koga H, Ishii N, Hashimoto T,
et al. Case of pemphigoid vegetans positive with both BP180 and
BP230 in enzyme-linked immunosorbent assays. J Dermatol.
2014;41:667–8. [PubMed: 24985550]

Figures and Tables

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
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
Table 1

Summary of previously reported cases of pemphigoid vegetans

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