Ophthalmic fuscocoeruleus nevus D22.30

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 12.10.2022

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Synonym(s)

mesodermal melanosis of the face and sclera; nevus of Ota; Nevus Ota; oculodermal melanocytosis; Oculodermal melanocytosis; Ota-Naevus

History
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Ota, 1939

Definition
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Mostly congenital dermal melanocytic nevus occurring almost exclusively in people of Mongolian descent, especially in women. An acquired variant has been described. See also naevus fuscocoeruleus deltoideoacromialis.

Occurrence/Epidemiology
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Mostly performed by Asians. Common in Japan.

Manifestation
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Not always visible at birth. First manifestations are described up to the 10th LJ. Girls are 3-5 times more frequently affected than boys.

Localization
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Service area of the 1st and 2nd trigeminal branch.

Clinical features
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Integument: Irregular, light reddish-brown to blackish-bluish pigmentation corresponding to the Mongolian spot, half-sided in the cheek area along the trigeminal nerve innervation.

Optional development of papular or small-nodular, raised efflorescences. Involvement of the buccal mucosa is possible.

Extracutaneous manifestation: Involvement of the eye and the buccal mucosa is possible. Up to 26% of patients show bilateral manifestation. Involvement of the eardrum (55%), eyes (49%), nasal mucosa (28%), pharynx (24%) and palate (18%). Associated symptoms: glaucoma, cataract, deafness, occiput deformity, hemiatrophia facei.

Histology
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In the middle and upper corium partly grouped, preferably periadnexial, spindle-shaped melanocytes filled with pigment granules, no melanophages.

Therapy
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If necessary, cosmetic cover (e.g. Dermacolor). Laser treatment sometimes leads to a brightening of the pigmentation. Clarification and monitoring of any accompanying symptoms.

Progression/forecast
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Rare development of a malignant melanoma. Intracerebral melanin-forming tumours possible.

Literature
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  1. Alvarez-Cuesta CC (2002) Nevus of Ota associated with ipsilateral deafness. J Am Acad Dermatol 47: S257-259
  2. Hidano A et al (1991) Acquired dermal melanocytosis of the face and extremities. Br J Dermatol 124: 96-99
  3. Kono T et al (2003) Use of Q-switched ruby laser in the treatment of nevus of ota in different age groups. Lasers Surg Med 32: 391-395
  4. Ota M, Tanino H (1939) Naevus fuscocaeruleus ophthalmo-maxillaris and melanosis bulbi. Tokyo Iji Shinshi 63: 1243-1245
  5. Terheyden P et al (2001) Nevus of Ota and choroid melanoma. dermatologist 52: 803-806
  6. Ueki H et al (1991) Acquired bilateral Naevus Ota. dermatologist 42: 584-586

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Last updated on: 12.10.2022