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536 SCANNING ELECTRON MICROSCOPIC AND XRAY MICROANALYTIC STUDIES OF HAIR. NAIL AND FOLLICULAR KERATOTlC PAPULE IN PACHYONYCHIA CONGENITA. Naoto Ohtake. Hldeaki Suniyama. Katsuhiko Tsukamoto, Masutaka Furue and Kunihiko Tamaki. Department of Dermatology, Yamanashi Medical Universitv. Yamanashi. Ia”a” A i-‘year-old bay was diagnosed with Iadassohn~Lewandsky type of pachyonychla congenita (PC) because of “ail hypertrophy. plantar blister and hyperkeratosis. follicular keratosis. oral leukokeratosis. and hoarseness. However. he had no sign of hair changes. neonatal teeth and steatocystoma multiplex. Purpose Surface structures and elements of the hair and nail in this patient were compared with those in normal boys. Morover. we investigated the structure and element of the follicular keratotic papuIe in comparison with those of the hair and nail in this patient. Method : The hair, nail and follicular keratatic papule from this patient. and the hair and nails from 3 normal boys were studied with HITACHI S~2250N Natural SEM and HORIBA EMAX-S770 Energy Dispersive X-ray Micro Analwar. Results : The nail from this patient showed rougher surface structure and lower sulfur level than the “ails from 3 normal boys. However. the hax from this patient showed the same surface structure and analytic patter” as the hair from 3 normal boys. The surface structure and analytic pattern of follicular keratotic papule with PC were much similar to those of his nail. but not those of his hair. Conclusion The hair of this patient was normal in scanning electron microscopic and X-ray microanalytic studies 8s well as clinical examination. As keratotic materials in “ail hypertr”phy and follicular keratosis showed similar low sulfur levels in X-ray microanalysis, we suggest that the decrease of the sulfur~containing amino acid is associated with hyperkeratosis in PC.