We report a 6-day-old Japanese girl showing generalized erythroderma accompanied by yellowish,exfoliative scaling that was accentuated on the face and scalp.Histological analysis showed psoriasiform dermatitis with ac...We report a 6-day-old Japanese girl showing generalized erythroderma accompanied by yellowish,exfoliative scaling that was accentuated on the face and scalp.Histological analysis showed psoriasiform dermatitis with acanthotic epidermis and premature shedding of the stratum corneum.Measurement of trypsin-like hydrolytic activity in SC showed six-fold greater activity compared with age-matched controls.DNA analysis revealed two mutations,375delAT and 966insC,in exons5 and 11,respectively,of the SPINK5 gene.Although at 4 weeks the child was still too young to display characteristic hair abnormalities or atopic diathesis,we diagnosed Netherton syndrome based on enzyme assay and DNA analysis.展开更多
Pityriasis rubra pilaris (PRP) is a rare papulosquamous disease with typical onset during the first and fifth decades. The skin disorder normally starts on t he scalp and spreads caudally Within a few weeks. It often ...Pityriasis rubra pilaris (PRP) is a rare papulosquamous disease with typical onset during the first and fifth decades. The skin disorder normally starts on t he scalp and spreads caudally Within a few weeks. It often results in a generali zed erythroderma with sharply demarcated islands of sparing (“ nappes claires” ). A 65-year-old patient with severe PRP showed good clinical improvement a fter 8 months of treatment when treated with acitretin in combination with photo therapy and systemic glucocorticosteroids.展开更多
文摘We report a 6-day-old Japanese girl showing generalized erythroderma accompanied by yellowish,exfoliative scaling that was accentuated on the face and scalp.Histological analysis showed psoriasiform dermatitis with acanthotic epidermis and premature shedding of the stratum corneum.Measurement of trypsin-like hydrolytic activity in SC showed six-fold greater activity compared with age-matched controls.DNA analysis revealed two mutations,375delAT and 966insC,in exons5 and 11,respectively,of the SPINK5 gene.Although at 4 weeks the child was still too young to display characteristic hair abnormalities or atopic diathesis,we diagnosed Netherton syndrome based on enzyme assay and DNA analysis.
文摘Pityriasis rubra pilaris (PRP) is a rare papulosquamous disease with typical onset during the first and fifth decades. The skin disorder normally starts on t he scalp and spreads caudally Within a few weeks. It often results in a generali zed erythroderma with sharply demarcated islands of sparing (“ nappes claires” ). A 65-year-old patient with severe PRP showed good clinical improvement a fter 8 months of treatment when treated with acitretin in combination with photo therapy and systemic glucocorticosteroids.
