Pemphigus Foliaceus in an Adolescent

Adah, Ameze; Hill, Kelley; Wyatt, Julie P.; Didion, Lisa

doi:10.1016/j.jpeds.2014.07.023

Cited by 5 publications

(2 citation statements)

References 4 publications

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“…Annular (polycyclic) [3], follicular [27], and erythrodermic [19,24] variants have been reported in pediatric-onset disease. Mucous membranes are rarely involved in PF, except for mild conjunctivitis in one case [25]. Interestingly, photoinduced PF has also been described in the literature, with four out of 29 patients reporting exacerbation of skin lesions after exposure to sunlight [2,4,8,27].…”

Section: Discussionmentioning

confidence: 91%

Sporadic Pemphigus Foliaceus in a 3-Year-Old Vietnamese Girl: A Case Report and Literature Review

Nguyen,

Chau,

Doan Vo

et al. 2024

Case Reports in Dermatological Medicine

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Pemphigus foliaceus is an uncommon autoimmune intraepidermal blistering disease characterized by immunoglobulin (Ig) G autoantibodies that attack desmoglein-1 in the epidermis. There are two predominant forms of pemphigus foliaceus, sporadic and endemic. Sporadic pemphigus foliaceus is known to be more prevalent in middle-aged and elderly people and to be extremely rare in children. Less than 40 nonendemic pediatric pemphigus foliaceus cases have been documented in the literature. This report documents a case of sporadic pemphigus foliaceus in a 3-year-old Vietnamese girl who presented with generalized scaling and crusted erosions over the body.

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Section: Discussionmentioning

confidence: 91%

Sporadic Pemphigus Foliaceus in a 3-Year-Old Vietnamese Girl: A Case Report and Literature Review

Nguyen,

Chau,

Doan Vo

et al. 2024

Case Reports in Dermatological Medicine

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“…One patient received oral betamethasone 4.5 mg daily [ 19 ]. Two patients were treated with intravenous corticosteroid pulse doses with either methylprednisolone or betamethasone of up to 500 mg [ 11 , 25 ]. Topical corticosteroids were reported in some cases without improvement.…”

Section: Reviewmentioning

confidence: 99%

Treatment of Pediatric Pemphigus Foliaceus

Carver,

Kalesinskas,

Dheden

et al. 2023

Cureus

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Pemphigus foliaceus (PF) is an autoimmune blistering disease limited to the superficial skin without mucosal involvement. It is clinically, histologically, and immunopathologically distinct from pemphigus vulgaris (PV). As data on pediatric PF is often merged with data on both pediatric and adult PV patients, isolating clinical outcomes in pediatric PF is not always possible. Therefore, the authors of this review analyzed clinical outcomes following therapy in pediatric PF patients only. A search of databases resulted in 33 pediatric patients with PF. In total, 19 (57.6%) patients were treated with conventional immunosuppressive therapies (CISTs), which consisted of systemic corticosteroids and multiple immunosuppressive agents (ISAs). Further, 14 (42.4%) patients were treated with biologic agents, predominantly rituximab (RTX). The mean age of those treated with biologics was 12.8 years (range = 0.88-18 years) compared to 8.9 years (range = 0.92-15 years) of those treated with CIST (p = 0.01). Treatment with biologics was initiated significantly longer after the diagnosis of PF when compared to patients treated with CIST (p = 0.003). RTX was used in all patients who received biologic therapy. Two (6%) patients also received intravenous immunoglobulin. When clinical outcomes were compared between CIST and biologic therapy, rates of clinical remission, partial remission, and relapse, were not statistically significantly different between groups. When RTX was used, rates of relapse and adverse events were higher in those treated with the lymphoma protocol (375 mg/m 2 once weekly for four weeks) compared to those treated with the rheumatoid arthritis protocol (two doses of 1,000 mg two weeks apart) (p < 0.0001). The incidence of adverse events was statistically significantly higher in patients treated with CIST when compared to RTX (p = 0.003). These included both physical and psychological changes. The infection rate after treatment with RTX was 7.1%. These outcomes occurred during a follow-up of 12.5 months (range = 1-36 months) in the CIST group and 20.5 months (range = 6-67 months) in the biologic therapy group. The difference in the follow-up period was not statistically significant. The literature suggests that biologics are superior to CIST in treating pemphigus patients. The results of this review suggest similar responses to therapy in pediatric PF patients treated with biologics compared to CIST. This may have been due to a limited duration of follow-up and a lack of detailed treatment outcomes in pediatric PF patients. The data in this review strongly suggests that specific treatment protocols need to be developed and implemented for pediatric PF patients. These patients are at a critical phase in life where PF therapy can influence or affect physical growth, hormonal changes, psychosocial development, and essential education.

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Bullous Diseases in Children: A Review of Clinical Features and Treatment Options

Schultz

Hook

2019

Pediatr Drugs

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Pemphigus Foliaceus in an Adolescent

Cited by 5 publications

References 4 publications

Sporadic Pemphigus Foliaceus in a 3-Year-Old Vietnamese Girl: A Case Report and Literature Review

Sporadic Pemphigus Foliaceus in a 3-Year-Old Vietnamese Girl: A Case Report and Literature Review

Treatment of Pediatric Pemphigus Foliaceus

Bullous Diseases in Children: A Review of Clinical Features and Treatment Options

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