Childhood bullous pemphigoid--report of a case with dermal fluorescence on salt-split skin. 1995

S H Wakelin, and J Allen, and F Wojnarowska
Department of Dermatology, Churchill Hospital, Oxford, U.K.

Bullous pemphigoid (BP) is an acquired bullous disorder which predominantly affects the elderly. It is rare in children, and may be clinically indistinguishable from other immunobullous disorders. As routine histology may be non-specific, a definitive diagnosis of childhood BP usually depends on the results of direct and indirect immunofluorescence investigations. We report a 5-year-old girl who developed bullous pemphigoid, associated with atypical immunofluorescence findings. Indirect immunofluorescence on split-skin showed a pure dermal pattern of IgG binding. This is usually suggestive of epidermolysis bullosa acquisita, but Western immunoblotting was positive with epidermal extracts, confirming a diagnosis of BP. Dermal binding on split-skin occurs in about 5% of adult cases of BP, and has not been reported previously in childhood BP.

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
D010391 Pemphigoid, Bullous A chronic and relatively benign subepidermal blistering disease usually of the elderly and without histopathologic acantholysis. Pemphigoid,Bullous Pemphigoid,Pemphigoids
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005148 Facial Dermatoses Skin diseases involving the FACE. Favre-Racouchot Syndrome,Nodular Elastoidosis,Facial Dermatosis,Nodular Elastosis,Dermatoses, Facial,Dermatosis, Facial,Elastoidoses, Nodular,Elastoidosis, Nodular,Elastoses, Nodular,Elastosis, Nodular,Favre Racouchot Syndrome,Nodular Elastoidoses,Nodular Elastoses,Syndrome, Favre-Racouchot
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012867 Skin The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
D012965 Sodium Chloride A ubiquitous sodium salt that is commonly used to season food. Sodium Chloride, (22)Na,Sodium Chloride, (24)NaCl
D015153 Blotting, Western Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes. Immunoblotting, Western,Western Blotting,Western Immunoblotting,Blot, Western,Immunoblot, Western,Western Blot,Western Immunoblot,Blots, Western,Blottings, Western,Immunoblots, Western,Immunoblottings, Western,Western Blots,Western Blottings,Western Immunoblots,Western Immunoblottings
D019084 Fluorescent Antibody Technique, Indirect A form of fluorescent antibody technique commonly used to detect serum antibodies and immune complexes in tissues and microorganisms in specimens from patients with infectious diseases. The technique involves formation of an antigen-antibody complex which is labeled with fluorescein-conjugated anti-immunoglobulin antibody. (From Bennington, Saunders Dictionary & Encyclopedia of Laboratory Medicine and Technology, 1984) Immunofluorescence Antibody Test, Indirect,Immunofluorescence Technique, Indirect,Fluorescent Antibody Technic, Indirect,Immunofluorescence Technic, Indirect,Indirect Fluorescent Antibody Technic,Indirect Fluorescent Antibody Technique,Indirect Immunofluorescence,Indirect Immunofluorescence Assay,Assay, Indirect Immunofluorescence,Assays, Indirect Immunofluorescence,Immunofluorescence Assay, Indirect,Immunofluorescence Assays, Indirect,Immunofluorescence Technics, Indirect,Immunofluorescence Techniques, Indirect,Immunofluorescence, Indirect,Immunofluorescences, Indirect,Indirect Immunofluorescence Assays,Indirect Immunofluorescence Technic,Indirect Immunofluorescence Technics,Indirect Immunofluorescence Technique,Indirect Immunofluorescence Techniques,Indirect Immunofluorescences

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