Pachyonychia Congenita Associated with Oral Leukoplakia: A Rare Case Report with Review of Literature
نویسنده
چکیده
Pachyonychia congenita comprises a heterogeneous group of autosomal dominantly inherited conditions manifesting with characteristic nail thickening, palmoplantar keratoderma, follicular keratosis and oral manifestations like mucosal leukokeratosis. Less frequently epidermal cysts, hair-shaft abnormalities, natal teeth and laryngeal involvement may be seen. It is distributed almost evenly throughout the world affecting 1 in 100,000 populations. Here, we report a case of pachyonychia congenita type-1 with an added emphasis on dental findings in 45-year-old South Indian male patient.
منابع مشابه
Pachyonychia congenita type II: report of a case
Pachyonychia congenital (PC) is a rare genodermatosis affecting the nails, skin, oral mucosae, larynx, hair, and teeth. General clinical presentation includes thickening of finger and toenails, painful plantar keratoderma, hyperhidrosis, oral leukokeratosis, follicular keratosis, palmar keratoderma, cutaneous cysts, hoarseness, twisted hair and prenatal or natal teeth. Clinically, PC is d...
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BACKGROUND Pachyonychia congenita (PC) is a rare inherited disorder of keratinization characterised by hypertrophic nail dystrophy, painful palmoplantar blisters, cysts, follicular hyperkeratosis and oral leukokeratosis. It is associated with mutations in five differentiation-specific keratin genes, KRT6A, KRT6B, KRT6C, KRT16, or KRT17. OBJECTIVES Living with Pachyonychia Congenita can be iso...
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Pachyonychia congenita is a rare type of ectodermal dysplasia further classified into 4 types. Cutaneous manifestations seen in most of the cases of Pachyonychia congenita include palmoplantar keratoderma, follicular hyperkeratosis, wedge shaped nails, oral leukokeratosis and woolly hair. A 25-year-old male presented to us with thickened nails and scanty scalp hair. On examination, we noticed h...
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