parents also noticed small hyperkeratotic growth over both soles, which over a period of years increased in thickness and area. It was associated with pain during walking. There was no history of natal teeth. On examination, the nails showed thickened, lusterless nail plate of all the nails with upward growth of its distal portion. The nails also showed the presence of a great amount of subungual hyperkeratosis and adhesion of the nail plate to the underlying nail bed [ Figure 1]. There was hypercurvature of the transverse axis of all nail plates giving a "pinched shape" or "door wedge shape" to the free edge of the nail plate [ Figure 2]. On cutaneous examination multiple hyperpigmented macules and hyperkeratotic follicular papules were present over neck, trunk and lower limbs [ Figure 3]. Few large, thick, hyperkeratotic growths were also seen over pressure areas of both the feet [ Figures 4 and 5]. The oral mucosa showed erythema and ulcer over the tongue. He also had hyperhydrosis of palms and soles. Systemic examination revealed no abnormality. The routine investigations were within the normal limits. Skin scraping for potassium hydroxide mount was negative for fungal elements. Patient's hair, teeth, throat, and ophthalmological examination were normal.Patient's younger sister also had similar nail and skin lesions since early childhood and died due to pulmonary tuberculosis at the age of 10 years. No other ABSTRACT Pachyonychia congenita (PC) is a rare genodermatosis characterized by hypertrophic nail changes and nail dystrophy. A 17-year-old male presented with thickened, yellowish brown discoloration of all the nails and multiple hyperkeratotic papules over his chest. Patient's younger sister also had similar nail and skin lesions since childhood. Presence of characteristic clinical features led to a diagnosis of a rare condition of PC type-1.
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