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Reticulate pigmented anomaly of flexures
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Reticulate pigmented anomaly of flexures

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Contributors: Chris G. Adigun MD, Lowell A. Goldsmith MD, MPH
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Synopsis

Reticulate pigmented anomaly of the flexures, also known as Dowling-Degos disease (DDD) or Dowling-Degos syndrome, is a rare autosomal dominant pigmentary disorder. The condition is characterized by progressive brown-black hyperpigmentation of the flexures that first appears in adolescence or adulthood. Pigmentation typically arises in the axillae, neck, and inframammary areas. Affected individuals may also have pitted acneiform scars in a perioral distribution. Other associations that are commonly seen include soft fibromas, follicular hyperkeratoses, and comedonal and cystic lesions in the flexural areas. Squamous cell carcinoma of the buttocks and perianal areas has been reported. Rare cases of DDD are associated with hidradenitis suppurativa and heterozygous mutations of PSENEN (gamma-secretase protein presenilin precursor). Mutations in POGLUT11, a gene associated with posttranslational modification of notch proteins, have been associated.

For more information, see OMIM.

Codes

ICD10CM:
L81.8 – Other specified disorders of pigmentation

SNOMEDCT:
239054009 – Reticulate pigmented anomaly of flexures

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Last Updated: 05/03/2019
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Reticulate pigmented anomaly of flexures
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Reticulate pigmented anomaly of flexures : Axilla, Brown color, Flexural, Hyperpigmented patch, Inframammary fold of chest, Lateral neck, Reticular - netlike, Superior chest, Hyperpigmented macules
Clinical image of Reticulate pigmented anomaly of flexures
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