Bullous Pemphigoid in A Previously Healthy Adolescent A Case Report and Literature Review
Bullous Pemphigoid in A Previously Healthy Adolescent A Case Report and Literature Review
Bullous Pemphigoid in A Previously Healthy Adolescent A Case Report and Literature Review
Narmeen Giacaman, MDa,*, Rawan Sami N. Abusaada, MDa, Salem M. Tos, MDa, Mohammad G. Ibdah, MDa,
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Adam M. Reid Mahagney, MDe, Asmaa Rjoob, MDa, Musallam Abukhalil, MDc, Hamza Salimd, Basel Musmard,
Sufyan Zuwahreh, MDb
Introduction: Bullous pemphigoid (BP) is considered the most common bullous autoimmune disorder, characterized by
autoantibodies directed against hemidesmosomes in the skin and mucous membranes. It usually affects elderly individuals in the
sixth through eighth decades of life, with an average age at onset of 65 years. Only a few cases have been reported in children and
teenagers.
Case presentation: Herein, we report a 17-year-old boy who presented with a pruritic vesicular rash on his arms and legs
accompanied by erythema. He was treated at the beginning with topical lotion and acyclovir, but the rash kept deteriorating and
eventually bullae appeared, involving also his mouth. A dermatologist was consulted and diagnosed him with BP, and he was treated
accordingly.
Discussion: BP is the most prevalent autoimmune bullous illness, caused by autoantibodies against hemidesmosomes in the
basement membrane of skin and mucosal surfaces, which in turn attract immune cells, including T-cells and neutrophils, and activate
them, which causes damage to and separation of keratinocytes, resulting in the bullous formation. Diagnosis can be accomplished
by recognizing clinical symptoms supported by histopathological and immunofluorescence testing. Steroids, whether topical or
systemic, are the cornerstone treatment; depending on the extent of the disease, other immunosuppressant drugs can be used as a
second line.
Conclusion: BP manifestations are polymorphic; physicians should keep in mind that they may present with non-bullous, pruritic
lesions, which may persist for some days to several months before bullae appear. Although this disease is rare in the young
population, it should be considered in the differential diagnosis of bullous lesions.
Keywords: bullae, bullous pemphigoid, case report, clinical dermatology
Introduction HIGHLIGHTS
• Bullous pemphigoid is rarely seen in adolescence.
In 1953, Lever introduced the term pemphigoid to describe a • The cutaneous manifestations of bullous pemphigoid are
disease characterized by bullous formation due to subepidermal polymorphic.
detachment to distinguish it from pemphigus, an intraepidermal • It can be misdiagnosed on initial presentation before bullae
blistering disorder brought on by acantholysis. appear.
Only few cases of bullous pemphigoid (BP) were reported in
children and teenagers in the literature, as this disease mainly
affects the elderly in their eighth decade of life and has no preference for either gender[1]. In this case, we describe a patient
with free past medical and surgical history that first presented
a
with pruritic vesicular rash surrounded by erythema on his arms,
College of Medicine, Al-Quds University, Abu Dis, bInternal Medicine Department,
Beit-Jala Governmental Hospital, Bethlehem, cFaculty of Medicine, Islamic University
legs, and trunk for a few days, followed by diffuse skin involve-
of Gaza, Gaza, dAn-Najah National University, Nablus, Palestine and eThe Hebrew ment of bullous lesions. He was treated mainly with topical and
University, Hadassah Medical School, Jerusalem, Israel systemic corticosteroids, which led to significant improvement.
Sponsorships or competing interests that may be relevant to content are disclosed at On follow-up, his lesions healed, and he was back to his
the end of this article. normal life.
*Corresponding author. Address: Al-Quds University, Main Campus, P.O. Box 89,
Abu Dis, Palestine.Te.:+ 970 227 906 06. E-mail: [email protected]
(N. Giacaman). Case presentation
Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. This is an
open access article distributed under the Creative Commons Attribution License 4.0 A 17-year-old boy complained of pruritic vesicular rash surrounded
(CCBY), which permits unrestricted use, distribution, and reproduction in any by erythema on his arms, legs, and trunk for few days. He was seen
medium, provided the original work is properly cited. by his family doctor and was diagnosed with chickenpox caused by
Annals of Medicine & Surgery (2023) 85:5039–5042 varicella-zoster virus. The doctor gave him symptomatic treatment
Received 4 October 2022; Accepted 10 June 2023 (calamine lotion) for the pruritis and acyclovir. Two days later, the
Published online 22 July 2023 rash kept getting worse and bullae started to appear; he also
http://dx.doi.org/10.1097/MS9.0000000000000995 developed painful mouth lesions (Fig. 1). The patient was seen by a
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Figure 1. Painful mouth lesions involving the oral mucosa and the soft palate.
dermatologist in his clinic who suspected bullous pemphigoid and for his age, including complete blood count, Thyroid function
sent him to be admitted to our hospital for treatment as the patient tests, basic metabolic panel, liver function tests, and kidney
could not have anything by mouth. function tests. So, he was started on intravenous (i.v.) fluids to
On admission, the patient had stable vital signs but looked prevent dehydration, pantoprazole 40 mg i.v., amoxicillin/cla-
dehydrated with cracked lips and dry skin. Diffuse skin involve- vulanic acid to prevent secondary skin infection, triamcinolone
ment was seen (Figs 2, 3) including axillae, arms, legs, trunk, oral cream for his oral lesions, clobetasol lotion (corticosteroid) for his
mucosal membrane, and the groin area. Nikolsky sign was skin lesions for 2 weeks and methylprednisolone 80 mg IV*1 to
negative on examination. Lab tests were all in the normal range be converted to prednisolone 40 mg once oral intake is tolerated.
Figure 2. Diffuse skin involvement by bullous pemphigoid. Pictures taken after 4 days in treatment.
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Giacaman et al. Annals of Medicine & Surgery (2023) Annals of Medicine & Surgery
and systemic glucocorticoids was used as more than 30% of the Research registration unique identifying number
skin surface area was affected. (UIN)
The recommendations prescribe or may explore the following
additional medications: cyclosporine, cyclophosphamide, plas- Not applicable.
mapheresis/immunoapheresis, sulfonamides, topical tacrolimus,
TNF (tumor necrosis factor) inhibitors, and other biologic Guarantor
agents[12-14]. According to the updated S2K guidelines for BP
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