Mongolian spot D22.5

Authors: Prof. Dr. med. Peter Altmeyer, Dr. med. Nikolas Bounas-Pyrros

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Last updated on: 29.10.2020

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Definition
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Harmless, in Asians frequent, in Caucasians rare, congenital, mostly extensive, in the sacral region localized, symptomless, dermal melanocytosis with gray-blue discoloration of the skin which in the majority of the affected persons regresses in the course of life.

S.a. further congenítal melanocytoses like:

nevus yamamoto

deltoideoacromial nevus

Ophthalmic fuscocoeruleus nevus

Nevus Ito

Occurrence/Epidemiology
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  • Especially for Asians (70-90% for Mongolians, Japanese and Chinese)
  • about 90% with Bolivian Indians
  • also common among black Africans
  • less frequently among members of the Caucasian race (1.0-2.0%).

Manifestation
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  • Congenital, more rarely occurring only in the first weeks of life;
  • m>w

Localization
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Distal back region, above the sacrum, buttocks and lumbar region. 5% of Mongolian spots can be found on the lower extremity.

Clinical features
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Not always visible at first sight, grey or blue-grey, 5.0-10.0 cm in size, moderately sharp or even only running out, indistinctly limited, completely non-irritating stain (no subjective symptoms) with an unchanged surface (no hypertrichosis), which recedes in the first years of life.

Histology
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Spindly or star-shaped pigment-loaded melanocytes, especially in the middle dermis.

Therapy
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Not necessary, cover cosmetically if necessary (camouflage).

Progression/forecast
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In larger collectives (n=2,100 infants) after one year, 15% of those affected showed a clear fading; 45% had completely disappeared. Lifelong persistence possible (evidence in about 4% of Japanese men between 18 and 22 years). Malignant degeneration was not observed.

Note(s)
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In rare cases extensive mongol spots have been described in association with congenital storage diseases: mucopolysaccharidosis type I (Hurler syndrome), GM1-gangliosidosis type I, mucopolysaccharidosis type II (Hunter syndrome), mucolipidosis, mannosidosis, Niemann-Pick disease.

Literature
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  1. Gupta D et al (2013) Mongolian spots. Indian J Dermatol Venereol Leprol 79:469-478
  2. Reza AM et al (2010) Incidence of Mongolian spots and its common sites at two university hospitals in Tehran, Iran. Pediatrist Dermatol 27:397-398
  3. Ricci F (2016) Extensive, irregular Mongolian patches indicating GM1 gangliosidosis type I. JDDG 14: 301-302
  4. Shih IH et al (2007) A birthmark survey in 500 newborns: clinical observation in two northern Taiwan medical center nurseries. Chang Gung Med J 30:220-225

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020