Pachyonychia Congenita Type I with Severe Oral Leukokeratosis
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چکیده
Pachyonychia Congenita (PC) is a rare autosomal dominant keratin disorder that affects a number of ectodermal structures including the nails and palmoplantar skin, and often involves the oral mucosa, tongue, larynx, teeth and hair. Clinical features are usually present at birth or early infancy. There are two main subtypes of PC. Fingernail thickening and oral keratosis are more common and severe in PC-1 and cystic lesions, hair abnormalities, natal teeth and pili torti are more common in PC-2. We report the case of a 6-year-old boy with PC-1 presenting with severe and painful oral leukokeratosis and extensive caries. Review (J Int Dent Med Res 2012; 5: (2), pp. 88-93)
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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A 30-year-old man presented with lesions on his oral mucosa and soles. There were no similar complaints in his family members. The dermatological examination revealed follicular hyperkeratosis on his trunk and upper extremities and flesh-colored, firm cystic lesions on his axillae. He had focal, painful, hyperkeratotic areas sited particularly on both his soles and palms. In addition to these, ...
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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 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 throug...
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Pachyonychia congenital (PC) is a rare autosomal dominant genodermatosis characterized hyperkeratosis affecting the nails and palmoplantar areas, oral leukokeratosis, and cystic lesions. A 39-year-old woman with PC type 1 (Jadassohn-Lewandowsky syndrome) and B-cell lymphoma is described. No similar disorders or parental consanguinity were found in her family. Typical features of PC developed si...
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