ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences
Inherited patterned lentiginosis in Child
Other Resources UpToDate PubMed

Inherited patterned lentiginosis in Child

Contributors: Andrina Mamo BS, Susan Burgin MD
Other Resources UpToDate PubMed

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

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

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

      Subscription Required

      Management Pearls

      Subscription Required

      Therapy

      Subscription Required

      References

      Subscription Required

      Last Reviewed:08/25/2021
      Last Updated:01/17/2022
      Copyright © 2022 VisualDx®. All rights reserved.
      Inherited patterned lentiginosis in Child
      Print  
      Inherited patterned lentiginosis
      Copyright © 2022 VisualDx®. All rights reserved.