Oculodermal melanocytosis, also known as Nevus of Ota, is a hamartomatous melanocytic nevus of dermal melanocytes. It presents as a bluish grey patch on the face, which may be congenital or acquired involving the area of distribution of the ophthalmic and maxillary branches of the trigeminal nerve. The nevus can be unilateral or bilateral, and, in addition to skin, it may involve ocular and ora...

Noteworthy heterogeneity exists in the rare diseases associated with childhood glaucoma. Primary congenital glaucoma is mostly sporadic; however, 10% to 40% of cases are familial. CYP1B1 gene mutations seem to account for 87% of familial cases and 27% of sporadic cases. Childhood glaucoma is classified in primary and secondary congenital glaucoma, further divided as glaucoma arising in dysgenes...

been reported in the world literature.4 The cutaneous pigmentation commonly involves the ophthalmic, maxillary, and occasionally the mandibular division of the trigeminal nerve. Oculodermal melanocytosis occurs most often in orientals and blacks and is rarely seen in the white population.2 5-7 In contrast, malignant melanoma transformation associated with ODM and OM is known to occur more often...

THE naevus of Ota is characterized by benign mesodermal melanosis of the skin of the face with pigmentation of the eye and its adnexa. It is probably more familiar to the dermatologists. Pusey (1916) was the first to describe the association of scleral and facial pigmentation in a young Chinese student. Later, in Japan, Ota and Tanino (1939) reported, under the name of naevus fusco-caeruleus ma...

Esophageal melanocytosis is a rare benign condition characterized by melanocytic proliferation in esophageal squamous epithelium and melanin deposition in the mucosa. Because of its uncommon nature, pathologists and gastroenterologists lack experience with this entity. We present a case of esophageal melanocytosis in a 66 years old male patient who presented with atypical chest pain and dysphag...

BACKGROUND The potential association of dermal melanocytosis with lysosomal storage disease in infancy is an uncommonly known and poorly understood entity. OBSERVATIONS We describe 2 infants with extensive dermal melanocytosis in association with GM1 gangliosidosis type 1 and Hurler syndrome, respectively. A literature analysis revealed 37 additional cases. Clinically, dermal melanocytosis as...

Dermal melanocytosis is characterized by the presence of ectopic melanocytes in the dermis. The most common forms include the Mongolian spot, blue nevus, nevus of Ota, and nevus of Ito. Some types of dermal melanocytosis do not fit into any of these morphologic categories, however. Our case demonstrated an extensive amount of uniform deep blue patches of nevi with unilateral distribution on the...