Case Report DOI: 10.18231/2455-8478.2017.0087 IP International Journal of Ocular Oncology and Oculoplasty, October-December,2017;3(4):326-328 326 Dyschromatosis universalis hereditaria with bilateral keratopathy- A rare co-existence Vinay Gupta 1 , Kalpana Sharma 2,* , K P Chaudhary 3 1 Assistant Professor, 2 Senior Resident, 3 Professor, Indira Gandhi Medical College *Corresponding Author: Email: doctorkalpana.84@gmail.com Abstract Dyschromatosis universalis hereditaria (DUH) is a pigmentary disorder of rare occurence comprising both hyperpigmented and hypopigmented macules forming an irregular pattern. Although ocular albinism, iris coloboma, aniridia, chorioretinal coloboma have been associated with it, we report a case of DUH with bilateral keratopathy which is a rare co-existence. This case represents the rarest association of DUH with decompensated cornea which was managed successfully with penetrating keratoplasty. Keywords: Dyschromatosis, Keratopathy, Keratoplasty. Introduction Dyschromatosis, a group of pigmentary disorders, is characterized by the both hyperpigmented and hypopigmented pleomorphic macules. This condition was first described by Ichikawa and Hiraga in 1933. (1,2) It forms the part of wide spectrum of dyschromatosis disorders namely dyschromatosis universalis hereditaria (DUH), dyschromatosis symmetrica hereditaria (DSH), acropigmentation of Dohi, and a segmental form called unilateral dermatomal pigmentary dermatosis. DUH is commonly found in Japan some isolated cases have also been reported in India, Europe, Saudi Arabia and South America. We report a case of DUH with corneal decompensation managed successfully with penetrating keratoplasty. Case Report A 42 years old male presented in eye OPD with diminution of vision in his right eye for the last 20 years which was gradual, painless and progressive. His past history revealed development of brownish lesions all over the body from 6 years of age which were accompanied by itching and burning sensation on photoexposed areas and intermittent sloughing of skin which was more during summer season. He underwent penetrating keratoplasty in his left eye in 1997 for similar complaint with no postoperative complications. His family history revealed similar complaints of diminution of vision both eyes in his elder sister for which she underwent penetrating keratoplasty both eyes. On cutaneous examination multiple generalized hyperpigmented macules of the size of 0.5mm to 5mm approximately interspersed with hypopigmented macules.(Fig. 1,2) The lesions were more distributed over the trunk (Fig. 3). There was involvement of oral mucosa, palms and soles. (Fig. 4,5,6) There were no lesions on hair, nails and teeth. His systemic examination showed no detected abnormality. Laboratory examination of blood profile i.e. complete hemogram, renal and liver function tests, within normal limits. Ultrasonogram of the kidney, urinary bladder and upper abdomen revealed no detectable abnormality. Skin biopsy of the hyperpimented macule showed increase in epidermal melanin in basal layers. (Fig. 7) Based on the clinical findings, a diagnosis of DUH was made. Fig. 1, 2: Hyperpigmented macules over face and head. Fig. 3: numerous generalized hyperpigmented macules interspersed with spotty hypopigmented macules over trunk Fig. 4: hyperpigmented macules over hard and soft palate