Table of Contents    
Clinical Image
 
Childhood bullous pemphigoid
Amarouch Hajar1, Benzekri Leila1, Ismaili Nadia1, Senouci Karima1, Badredine Hassam1
1Dermatology & Venerology Department, University Hospital Of IBN SINA Rabat Morocco.

Article ID: 100010Z09AH2016
doi:10.5348/Z09-2016-10-CL-3

Address correspondence to:
Amarouch Hajar
Rue Belmfedel
service de dermatologie Chu Ibn Sina Université Mohamed
V Souissi Rabat Maroc

Access full text article on other devices

  Access PDF of article on other devices

[HTML Abstract]   [PDF Full Text] [Print This Article]
[Similar article in Pumed] [Similar article in Google Scholar]

How to cite this article
Hajar A, Leila B, Nadia I, Karima S, Hassam B. Childhood bullous pemphigoid. J Case Rep Images Med 2016;2:9–11.



Case Report

A two-year-old girl was presented with vesiculobullous eruptions initially on the palmoplantar region then extend to the whole body (Figure 1). On examination multiple tense vesicles, bullae and crusted erosions touching the whole tegument were seen. Nikolsky's sign was negative. Mucous membrane examination revealed erosions over buccal mucosa and hard palate. There was no regional lymphadenopathy. Histopathological examination revealed subepidermal blister and a mild inflammatory infiltrate on the dermis. Direct immunofluorescence (DIF) showed linear deposition of IgG and C3 at the dermoepidermal junction. The specific blood test searching for cutaneous basement membrane antibodies was positive. Based on clinical, histological and immunopathological criteria, the diagnosis of childhood bullous pemphigoid was made. The patient was treated with prednisone 1 mg/kg/day associated with methotrexate 5 mg/week which led to a rapid resolution of the skin lesions within two months. Topical steroids and dapsone were ineffective in this case.

Cursor on image to zoom/Click text to open image
Figure 1: Presence of multiple tight, widespread vesicles bullae and crusted erosions.


Discussion

Bullous pemphigoid(BP) is an acquired autoimmune blistering disorder affecting mostly elderly people and rarely children [1] . There have been fewer than 100 cases of childhood bullous pemphigoid reported. Clinically, it is similar to adult bullous pemphigoid, with tense vesicles and bullae of variable size and localization. Urticarial papules with pruritus are usual in bullous pemphigoid [1]. However, mucous membrane involvement, thus hand and feet lesions are more frequent in children [2]. Histology and direct immunofluorescence are fundamental for the diagnosis. It presents as a subepidermal bullae with variable amount of eosinophils; associated to a linear deposition of IgG and/or C3 at the basement membrane zone. The presence of IgG antibodies reacting with the basement membrane zone is considered to be diagnosis criterion. Clinical differential diagnoses include linear IgA dermatosis, bullous impetigo, dermatitis herpetiformis, mastocytosis, epidermolysis bullosa, and porphyria. In our case clinical and immunopathological criteria made the diagnosis. Topical or systemic corticosteroids represent the first line treatment at a dose of 0.5–1 mg/kg/day. Other immunosuppressive drugs frequently used are methotrexate, azathioprine, cyclosporine, cyclophosphamide, and mycophenolate mofetil [3].


Conclusion

In conclusion, a diagnosis of childhood bullous pemphigoid should be considered in any infant or child presenting with vesiculobullous eruptions, especially on the palms and soles. Early onset of appropriate treatment must be done to avoid infections, fluid and electrolyte loss.

Keywords: Childhood bullous pemphigoid, Corticosteroids, Dapsone, Methotrexate


References
  1. Waisbourd-Zinman O, Ben-Amitai D, Cohen AD, et al. Bullous pemphigoid in infancy: Clinical and epidemiologic characteristics. J Am Acad Dermatol 2008 Jan;58(1):41–8.   [CrossRef]   [Pubmed]    Back to citation no. 1
  2. Mael-ainin M, El Morabite K, Hassam B, Senouci K. Infantile bullous pemphigoid. Report of a new case. [Article in French]. Presse Med 2014 Dec;43(12 Pt 1):1403–4.   [CrossRef]   [Pubmed]    Back to citation no. 2
  3. Roujeau JC, Ingen-Housz-Oro S, Leroux C, Joly P. Treatment of bullous pemphigoid and pemphigus. The French experience, 2009 update. G Ital Dermatol Venereol 2009 Aug;144(4):333–8.   [Pubmed]    Back to citation no. 3
[HTML Abstract]   [PDF Full Text]

Author Contributions
Amarouch Hajar – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Benzekri Leila – Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Ismaili Nadia – Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Senouci Karima – Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Badredine Hassam – Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2016 Amarouch Hajar et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.