title: Neonatal onset diffuse cutaneous mastocytosis: a case report and review of the literature.
abstract: Diffuse cutaneous mastocytosis is a rare variant of mast cell disease with widespread erythroderma, which is normally clinically apparent in early infancy. We report the case of a neonate who presented with diffuse erythrodermic rash and bullous lesions. Biopsy specimens showed a dense dermal infiltrate of mast cells. Serum histamine and tryptase levels were elevated. No somatic mutation of the c-kit gene was found. Blistering ceased at 5 months of age, but atopic dermatitis appeared at 6 months and allergic workup revealed a high level of food-specific IgE. Herein, we describe the case and provide the first review of the literature on neonatal onset diffuse cutaneous mastocytosis to clarify the prognosis of this condition.
authors.affiliation: Department of Neonatology, Center for Perinatal Medicine, Oita Prefectural Hospital, Beppu-city, Oita, Japan. sakuraliberty@beppu2.hosp.go.jp
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Koga
authors.forename: Takayuki
authors.initials: T
authors.lastname: Kokubo
authors.forename: Mutsumi
authors.initials: M
authors.lastname: Akaishi
authors.forename: Koichi
authors.initials: K
authors.lastname: Iida
authors.forename: Seigo
authors.initials: S
authors.lastname: Korematsu
mesh_terms: D001768:Blister / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D004205:Cromolyn Sodium / Q000627:therapeutic use; D003876:Dermatitis, Atopic / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D003879:Dermatologic Agents / Q000627:therapeutic use; D005076:Exanthema / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D005512:Food Hypersensitivity / Q000097:blood / Q000178:diet therapy; D006632:Histamine / Q000097:blood; D006634:Histamine H1 Antagonists / Q000627:therapeutic use; D006801:Humans; D007073:Immunoglobulin E / Q000097:blood; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008407:Mast Cells / Q000187:drug effects / Q000473:pathology; D034701:Mastocytosis, Cutaneous / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics; D013256:Steroids / Q000627:therapeutic use; D053802:Tryptases / Q000097:blood
chemical_list: D003879:Dermatologic Agents; D006634:Histamine H1 Antagonists; D013256:Steroids; D007073:Immunoglobulin E; D006632:Histamine; D019009:Proto-Oncogene Proteins c-kit; D053802:Tryptases; D004205:Cromolyn Sodium