Introduction
Bullous Pemphigoid in infants is a rare entity. Although after linear IgA dermatosis it is considered the second most common acquired immunobullous disease in infants.(1) It was first described in 1977 with increasing reporting since then.(2,3) We report 3 cases of bullous pemphigoid in infants admitted to our hospital between 2003 and 2019.
Case Series
| Case | 2003 | 2015 | 2018 |
| Sex | Boy | Girl | Girl |
| Age at referral | 3,5 months | 8 months | 3,5 months |
| Age of onset | 3 months | 4 months | 2,5 months |
| First localization | Hands & Feet | Hands & Feet | Hands & Feet |
| Clinical course | Erythematous papules Urticaria like lesions Vesicles & Bullae | Generalized blistering Pruritus++ | Urticaria like lesions Erythematous plaques Bullae Pruritus |
| Mucosal involvement | No | No | No |
| Scarring | N/A | No | N/A |
| Investigations | Infections (herpes, varicella, bacteria): negative General blood test: normal BP 180: 164 AU BP 230: not tested Biopsy: Linear C3 (and IgG) depositions at the basement membrane. No subepidermal bullae. Spongiotic superficial dermatitis with eosinophils. | Infections (herpes, varicella, bacteria): negative General blood test: increased transaminases BP 180: >200 AU BP 230: negative Biopsy: IgG and C3 depositions at the basement membrane | Infections (varicella, bacteria): negative General blood test: normal BP 180: >200 AU BP 230: negative Biopsy: IgG and C3 depositions at the basement membrane |
| Therapy | Medrol (1mg/kg/day) Topical potent corticosteroids Flammazine | Prednisone Dapsone | Topical potent corticosteroids |
| Outcome | No new blisters since the start of medrol (almost 4 months old) | No new blisters since the start of dapsone (9 months old) Stop treatment without recurrence 9 months after starting dapsone Gradual decrease of BP 180 values | No new blisters since the end of January 2019 (5 months old) Gradual decrease of BP 180 values |
| Vaccination/ Infection | First symptoms during bronchitis. Vaccination was administered after the onset of the first symptoms. | N/A | First symptoms 8 days after first vaccination |
Discussion
If we compare our cases with the current literature, we can conclude that the mean age of our patients was just above 3 months, which is slightly less than the 4.5 months suggested in the literature.(2) Some authors suggest a female predominance while others cannot confirm this.(2–4) Acral involvement, present in all our cases, is very common in infants. Mucosal involvement on the other hand is very common in childhood bullous pemphigoid.(4) Other clinical findings include itchy urticarial wheals and plaques with tense bullae. The golden standard for diagnosing Bullous Pemphigoid is clinical presentation and skin biopsy with direct immunofluorescence, which reveals a linear IgG and C3 deposition at the basement membrane. Additional to that, determining autoantibody titers can be useful: anti-BP180 antibodies are more frequent in infants than anti-BP230 antibodies. The impact of these titers on the clinical course is under discussion.(1) Prognosis of infantile Bullous Pemphigoid is very favourable. The majority of patients achieve complete remission in weeks to months, which is an important difference with the adult patient population.(4) The likelihood of a relapse is virtually non-existent. In conclusion we would like to consider the possible role of prior vaccinations/infections in this disease, although it is very difficult to prove and little evidence can be found about it in the literature.(3,5,6)
References
- Ferreira BR, Vaz AS, Ramos L, Reis JP, Gonçalo M. Bullous pemphigoid of infancy – report and review of infantile and pediatric bullous pemphigoid. Dermatol Online J. 2017 Feb 16;23(2).
- Schwieger-Briel A, Moellmann C, Mattulat B, Schauer F, Kiritsi D, Schmidt E, et al. Bullous pemphigoid in infants: characteristics, diagnosis and treatment. Orphanet J Rare Dis. 2014 Dec 10;9(1):185.
- Waisbourd-Zinman O, Ben-Amitai D, Cohen AD, Feinmesser M, Mimouni D, Adir-Shani A, et al. Bullous pemphigoid in infancy: Clinical and epidemiologic characteristics. J Am Acad Dermatol. 2008 Jan;58(1):41–8.
- Marathe K, Lu J, Morel KD. Bullous diseases: Kids are not just little people. Clin Dermatol. 2015 Nov;33(6):644–56.
- Neri I, Greco A, Bassi A, Orgaz-Molina J, Balestri R, Oranges T, et al. Bullous pemphigoid in infant post vaccination: Myth or reality? Int J Immunopathol Pharmacol. 2016 Jun 18;29(2):295–9.
- Baroero L, Coppo P, Bertolino L, Maccario S, Savino F. Three case reports of post immunization and post viral Bullous Pemphigoid: looking for the right trigger. BMC Pediatr. 2017 Dec 23;17(1):60.
Noë E.1, Morren M.2, Spanoudi-Kitrimi I.1
(1): Department of Dermatology, University Hospitals Leuven, Leuven, Belgium
(2): Unité de dermatologie pediatrique , hôpital des enfants CHUV Lausanne, Switzerland