Congenital dermal melanocytosis - Anogenital in
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Synopsis

Congenital dermal melanocytosis (previously referred to as Mongolian spot) is a congenital, benign, blue to gray patch caused by an increased number of dermal melanocytes. These patches are commonly seen in the sacrococcygeal area in infants of Asian or African descent but may be found on any cutaneous surface in infants of all ethnicities. The pigmentation becomes most intense at 1 year of age, reaches its peak diameter by 2 years of age, and usually fades completely by adulthood.
Related topic: acquired dermal melanocytosis
Related topic: acquired dermal melanocytosis
Codes
ICD10CM:
Q82.8 – Other specified congenital malformations of skin
SNOMEDCT:
40467008 – Mongolian spot
Q82.8 – Other specified congenital malformations of skin
SNOMEDCT:
40467008 – Mongolian spot
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Child physical abuse – Childhood abuse affects children of all ages and economic and cultural backgrounds. It can often be challenging to differentiate findings attributable to child abuse from benign anogenital skin conditions. Abuse can present with purpura and ecchymoses: changes color from blue to green to brown over days to weeks, uneven pigmentation, tender to palpation. Documentation of congenital dermal melanocytosis may be helpful in avoiding any confusion.
- Congenital melanocytic nevus – Brown, well-defined, surface change (eg, papular lesions).
- Blue nevus – Usually much smaller, typically no more than 5 mm in diameter, well-defined.
- Nevus of Ito – Often speckled, progresses with age, located over the shoulder, clavicle, and scapular region.
- Nevus of Ota – Often speckled, progresses with age, located in the periorbital region.
- Venous malformation – Usually palpable, may swell or change in coloration when dependent or crying.
- Infantile hemangioma – Overlying telangiectasias, may swell or deepen in coloration when dependent or crying, rapidly elevates over days to weeks.
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Therapy
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References
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Last Updated:02/08/2021

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Congenital dermal melanocytosis - Anogenital in
See also in: Overview