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Mongolian Spot

  • Presentation (Fig. 15-1)

    • Blue-gray patches present at birth or early infancy

    • Usually buttocks, lumbosacral back

    • Common in black, Hispanic, and Asian races

    • Color due to Tyndall effect (scattering of light as it strikes dermal melanin)

  • Course

    • Usually resolves by early to late childhood

    • Extensive Mongolian spots (dermal melanocytosis) with dorsal/ventral distribution, indistinct borders, and persistent and/or “progressive” behavior may be a sign of underlying lysosomal storage disease (most commonly GM1 gangliosidosis type 1 and Hurler disease)


Dermal melanocytosis (Mongolian spot). (Used with permission from Dr. Agim.)

Nevus of Ota

  • Also known as oculodermal melanocytosis, nevus fuscoceruleus ophthalmomaxillaris (Fig. 15-2)

  • Presentation

    • Unilateral bluish gray discoloration of facial skin and adjacent cornea; in darker skin blue component is less obvious

    • Affects region supplied by trigeminal nerve V1 and V2 ± ipsilateral sclera

    • Congenital or acquired by second decade

    • More common in black or Asian races, females

  • Treatment: pigmented lesion lasers

  • Course

    • Persists; both this and nevus of Ito may increase in size/intensity over time

    • Ocular involvement: risk of glaucoma, small melanoma risk


Nevus of Ota. (Used with permission from Dr. Agim.)

Nevus of Ito

  • Also known as nevus fusoceruleus acromiodeltoides (Fig. 15-3)

  • Presentation: similar to nevus of Ota but localized to unilateral shoulder, lateral neck, scapula, and/or deltoid

  • Course: persists

  • Treatment: pigmented lesion lasers


Nevus of Ito. (Used with permission from Dr. Agim.)

Mosaic Hypopigmentation

  • Includes hypomelanosis of Ito (incontinentia pigmenti acromians), nevus depigmentosus (achromic nevus) (Fig. 15-4)

  • Presentation

    • Benign, hypopigmented oval or round patches, bands, or swirls

    • May be localized or extensive

    • Arranged along one or more Blaschko lines

    • No preceding vesicular or inflammatory stages

    • Incidence of systemic manifestations highest with most extensive lesions

    • Most commonly central nervous system (CNS), musculoskeletal, and eyes depending on particular chromosome defect and level of mosaicism

  • Course: persists


Cutis tricolor (mosaic hyper and hypopigmentation). (Used with permission from Dr. Agim.)


Blueberry Muffin Baby

  • Presentation (Fig. 15-5)

    • Multiple, dark blue to magenta, small, nonblanching papules and macules

    • Present at birth or by first day of life

  • Etiology

    • Extramedullary hematopoiesis

    • Associated with congenital infections (TORCH viruses, most commonly cytomegalovirus), hemolytic disease of the newborn, hereditary spherocytosis, twin-twin transfusion syndrome

  • Differential

    • Includes neoplastic-infiltrative disease (lesions typically ...

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