Mal de Meleda: A Case report in Eastern india

Mal de Meleda is a rare ( Prevalence 1:1,00,000 ) autosomal recessive, diffuse, transgradient, nonsyndromic, non-epidermolytic, palmoplantar keratoderma (PPK) with associated scleroatrophy, nail changes, pseudoainhum around digits & perioral erythema without a tendency of spontaneous resolution .We present here a case of Mal de Meleda. A 10 year old male student from rural area presented with keratoderma involving palms, soles, dorsum of foot & hand along with involvement of knee & elbow. Diagnosis was confirmed by histopathological examination which showed hyperkeratosis without epidermolysis. This type of PPK may be confused with others types ( Greither’s disease,Huriez syndrome,Olmsted’s syndrome,Vohwinkal’s syndrome,Papillon Lefevre syndrome ) of PPKs & disorders of cornification.