ALKAPTONURIA AND KERATOELASTOIDOSIS MARGINAL AN UNUSUAL ASSOCIATION

Abstract

disorder with an autosomal recessive inheritance. The disease is typically characterized by a triad of dark urine, cutaneous hyperpigmentation, and arthropathy [1]. The diagnosis may easily be made during infancy when an observant mother notes dark staining of diapers; however clinical signs and symptoms usually develop following the deposition of pigment in connective tissues in the fourth decade of life. Cutaneous pigmentation, an important signs of disease, is more pronounced in sun-exposed sites and in areas with high sweat gland density. Bluish discolouration of skin overlying cartilages, especially on the ear and nose, may be one of the earliest signs of the disease [1]. We report a patient who presented with hyperpigmentation and acrokeratoelastoides marginalis like lesions on palms alongwith cutaneous and systemic manifestations of disease.