Trichorrhexis Invaginata Pathognomonic For Netherton Syndrom
A 6-month-old male infant, child of first-cousin marriage, presented with an inflamed, erythematous and scaly skin. Parents emphasized the uneasiness, irritability, intensive scratching, and red scaly patches all over his body and scalp. Symptoms were initiated during the neonatal period and did not respond to multiple medications for six months. The symptoms developed progressively. On physical examination, diffuse erythematous and scaly patches were seen on the face, neck, shoulders, back, trunk, and upper chest thighs. He had moderate dehydration signs. His height and weight growth charts were appropriate for his age. His hair was brittle and short with fine shafts. Microscopic findings on the hair shaft were compatible with trichorrhexis invaginata, also known as bamboo hair. His parents had no history of skin lesions on their body. Histopathological examination from the child’s skin revealed psoriasiform dermatitis capable with innate lymphoid cells. Genetic analysis confirmed the Netherton syndrome diagnosis.

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