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An infant with bullous pemphigoid. 2007

António Luís Santos, and Alberto Vieira Mota, and José Ramon, and José Manuel Lopes, and Filomena Azevedo
Dermatology Department, Hospital de São João and Faculty of Medicine of Oporto, Portugal.

Bullous pemphigoid is the most common subepidermal autoimmune blistering disease. However, it is very rare in the pediatric population. A 5-month-old male infant presented with multiple bullae located on palms and soles. Complete blood count revealed relative eosinophilia . A biopsy revealed a subepidermal blister present along with a dermal mononuclear infiltrate rich in eosinophils. In focal areas collagen flame figures, were also observed. Direct immunofluorescence showed linear reactivity for C3c and IgG at the dermal-epidermal junction. IgA was not found. Complete resolution was achieved after oral deflazacort (1.5mg/kg/day) with progressive dose reduction over 2 weeks. Bullous pemphigoid is extremely rare in the infant. In our case the disease presented with an unusual localized (acral) clinical distribution and a favorable outcome.

UI MeSH Term Description Entries
D007074 Immunoglobulin G The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B. Gamma Globulin, 7S,IgG,IgG Antibody,Allerglobuline,IgG(T),IgG1,IgG2,IgG2A,IgG2B,IgG3,IgG4,Immunoglobulin GT,Polyglobin,7S Gamma Globulin,Antibody, IgG,GT, Immunoglobulin
D007166 Immunosuppressive Agents Agents that suppress immune function by one of several mechanisms of action. Classical cytotoxic immunosuppressants act by inhibiting DNA synthesis. Others may act through activation of T-CELLS or by inhibiting the activation of HELPER CELLS. While immunosuppression has been brought about in the past primarily to prevent rejection of transplanted organs, new applications involving mediation of the effects of INTERLEUKINS and other CYTOKINES are emerging. Immunosuppressant,Immunosuppressive Agent,Immunosuppressants,Agent, Immunosuppressive,Agents, Immunosuppressive
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D010391 Pemphigoid, Bullous A chronic and relatively benign subepidermal blistering disease usually of the elderly and without histopathologic acantholysis. Pemphigoid,Bullous Pemphigoid,Pemphigoids
D011282 Pregnenediones Unsaturated pregnane derivatives containing two keto groups on side chains or ring structures. Diketopregnenes,Dioxopregnenes
D012008 Recurrence The return of a sign, symptom, or disease after a remission. Recrudescence,Relapse,Recrudescences,Recurrences,Relapses
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D004305 Dose-Response Relationship, Drug The relationship between the dose of an administered drug and the response of the organism to the drug. Dose Response Relationship, Drug,Dose-Response Relationships, Drug,Drug Dose-Response Relationship,Drug Dose-Response Relationships,Relationship, Drug Dose-Response,Relationships, Drug Dose-Response
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup

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