Inherited patterned lentiginosis
Alerts and Notices
Synopsis

Codes
ICD10CM:L81.4 – Other melanin hyperpigmentation
SNOMEDCT:
765195000 – Familial generalized lentiginosis
Look For
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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
Best Tests
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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