title: Atypical form of transient reactive papulotranslucent acrokeratoderma in a cystic fibrosis carrier.
abstract: We report the first documented case of an atypical form of transient reactive papulotranslucent acrokeratoderma (TRPA) in a patient heterozygous for the ΔF508 CFTR(cystic fibrosis transmembrane conductance regulator) mutation. TRPA represents a condition that classically presents with translucent to white plaques that become evident after water exposure. An atypical form with persistent lesions has also been described. Our patient is a 16-year-old girl with small, white papules coalescing into pebbly plaques on the palms. This condition is exacerbated after 5-10 min of water exposure and is associated with discomfort. The skin biopsy showed expanded stratum corneum, orthohyperkeratosis and dilation of eccrine ducts consisting with TRPA. A cystic fibrosis carrier state, barrier function defect, hyperhidrosis and the intake of cyclooxygenase inhibitors may have been pathogenic factors in our patient.
authors: Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, FL 33136, USA.
authors: Katherine
authors: K
authors: Baquerizo
authors: Sadegh
authors: S
authors: Amini
authors: Jonette E
authors: JE
authors: Keri
authors: Evangelos V
authors: EV
authors: Badiavas
authors: George W
authors: GW
authors: Elgart
mesh_terms: D000293:Adolescent; D003550:Cystic Fibrosis / Q000150:complications* / Q000235:genetics; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D004817:Epidermis / Q000473:pathology*; D005260:Female; D006225:Hand; D006579:Heterozygote; D006801:Humans; D007645:Keratoderma, Palmoplantar / Q000209:etiology* / Q000235:genetics / Q000473:pathology; D009154:Mutation; D012867:Skin / Q000473:pathology
chemical_list: C093349:cystic fibrosis transmembrane conductance regulator delta F508; D019005:Cystic Fibrosis Transmembrane Conductance Regulator