Inherited patterned lentiginosis
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Synopsis
Inherited patterned lentiginosis (IPL) is an extremely rare, autosomal dominant condition reported in patients of African descent, especially those with mixed American Indian heritage. It is characterized by lentigines occurring primarily on the face, including the lips; they may also be found on the buttocks, elbows, hands, and feet. They initially appear during infancy or childhood and increase in number with age. There is no predilection for sun-exposed skin, and the color of macules does not vary with change in season. The mucous membranes are typically not affected.
Codes
ICD10CM:
L81.4 – Other melanin hyperpigmentation
SNOMEDCT:
765195000 – Familial generalized lentiginosis
L81.4 – Other melanin hyperpigmentation
SNOMEDCT:
765195000 – Familial generalized lentiginosis
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Peutz-Jeghers syndrome – Prominent lip, intraoral, and periorificial pigmentation is associated with hamartomatous gastrointestinal (GI) polyps and increased malignancy risk
- LEOPARD syndrome – Electrocardiographic conduction abnormalities, ocular hypertelorism, pulmonary stenosis, abnormal genitalia, growth retardation, sensorineural deafness
- Carney complex – Cardiac and cutaneous myxomas, blue nevi, endocrine abnormalities
- Cronkhite-Canada syndrome – Nonfamilial GI polyposis syndrome with associated cutaneous hyperpigmented macules, onychodystrophy, and alopecia
- Generalized lentiginosis – Diffuse lentigines without systemic or mucosal involvement
- Laugier-Hunziker syndrome – May have oral and lip involvement; nails can be affected; no systemic involvement
- Centrofacial lentiginosis – Lentigines are found on the cheeks and nose in a butterfly distribution; association with bone abnormalities; dysraphia; endocrine, neurologic, and psychiatric disease
- Solar lentigines
- Dowling-Degos disease
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Management Pearls
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Therapy
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References
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Last Reviewed:08/25/2021
Last Updated:01/17/2022
Last Updated:01/17/2022