Pemphigus vulgaris in a 12-year-old boy

Lehman, R M; Landau, Joseph W.; Newcomer, Victor D.

doi:10.1016/s0022-3476(65)80250-5

Cited by 18 publications

(9 citation statements)

References 13 publications

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“…' Only five patients have been reported with biopsy proven childhood pem¬ phigus vulgaris. [2][3][4][5][6] In every case the correct diagnosis was delayed due to the low index of suspicion for this dis¬ ease in a young patient. In each in¬ stance the process presented in¬ sidiously with erosions confined to the oral mucous membrane.…”

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confidence: 99%

Pemphigus Vulgaris in Childhood

Murphy¹,

Harrell²

1972

Arch Pediatr Adolesc Med

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confidence: 99%

Pemphigus Vulgaris in Childhood

Murphy¹,

Harrell²

1972

Arch Pediatr Adolesc Med

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“…There were 17 boys and 16 girls. The age at the onset of the disease varied from 1.5 to 12 years (mean 8.3 yrs) (8–36). Thirty patients were treated with systemic corticosteroids alone or in combination with ISA for periods that ranged from 0.3 to 11.2 years (mean 4.5 yrs).…”

Section: Resultsmentioning

confidence: 99%

“…Thirty patients were treated with systemic corticosteroids alone or in combination with ISA for periods that ranged from 0.3 to 11.2 years (mean 4.5 yrs). They were followed up for periods ranging from 0.3 to 16 years (mean 5.2 yrs) (8,12–15,17,19,22–36). There were no data provided on the duration of treatment or follow‐up on four patients, but information on the clinical outcome was provided (9–11,16).…”

Section: Resultsmentioning

confidence: 99%

“…Of the 33 patients, five had mucosal involvement only (17,21,25,29,32), one had cutaneous involvement only (14), and 27 had cutaneous and mucosal involvement (8–13,15,16,18–20,22–24,26–31,33–36); 31 of the 32 patients with mucosal involvement had oral involvement (8–13,15–17,19–36), and of these 31 patients, one, four, and patients had nasal, ocular and genital involvement respectively (8,18,20,22,25,26,29–31).…”

Section: Resultsmentioning

confidence: 99%

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Analysis of Current Therapy and Clinical Outcome in Childhood Pemphigus Vulgaris

Mabrouk

Ahmed

2011

Pediatric Dermatology

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Pemphigus vulgaris (PV) is a rare, potentially fatal chronic autoimmune disease of the skin and mucous membrane. The objective of this study was to analyze the clinical outcomes and side effects associated with treatment of childhood PV (CPV). A retrospective review of the English language literature was conducted through PUBMED using the words childhood pemphigus vulgaris, and treatment or clinical outcome. Only patients under 12 years of age were included. Thirty-three cases were found in 29 reports. Mean age at onset was 8.3 years (range 1.5-12 yrs). Mucosal involvement (97.0%) was more common than cutaneous involvement (84.8%). Oral mucosa was the most common site of mucosal involvement (93.9%), followed by genital (20.6%), ocular (11.8%), and nasal mucosa (2.9%). Mean duration of therapy was 4.5 years (range 0.6-14.5 yrs), and mean duration of follow-up was 5.2 years (range 0.6-16 yrs). Complete recovery with no further therapy was achieved in 18.2% and partial recovery with minor relapses while on maintenance therapy in 78.8%. One patient died due to infection (3.0%). Serious side effects were present in 60.6%. The most common were cushingoid features (65.0%), growth retardation (50.0%), and infection (50.0%). Two patients who were refractory to systemic corticosteroids and immunosuppressive agents (ISA) had a favorable clinical response to Rituximab. Current therapy for CPV involving the use of long-term systemic corticosteroids in conjunction with ISA results in prolonged immunosuppression, causing systemic infections and growth retardation. Safer and more effective therapies need to be explored.

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“…Sixteen cases of histologically proven pemphigus vulgaris in patients aged 16 and under have been reported since 1955 (Berger et al, 1973;Canizares & Dorsey, 1956;Elias et al, 1972;Hadida et al, 1966;Jordan, Ihrig & Perry, 1969;Khamaganova, Vedrova & Lukiyanova, 1977;Lehman, Landau & Newcomer, 1965;Menter, Bell & Wells, 1973;Mincer, Turner & Sibelius, 1975;Mintzer & Rubin, 1955;Murphy & Harrell, 1972;Paltzik & Laude, 1978;Petkov & Georgiva, 1966;Stiegleder, 1955;Walker, 1978;Wranicz, Sysa Jedrzejowska & Broniarezyk-Dyla, 1975). Ten were female and six male.…”

Section: Discussionmentioning

confidence: 99%