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Familial white lentiginosis
Other Resources UpToDate PubMed

Familial white lentiginosis

Contributors: Gabriel E. Molina, Susan Burgin MD
Other Resources UpToDate PubMed


Familial white lentiginosis (FWL) is a rare acquired disorder of macular hypopigmentation. The characteristic lesions (guttate white macules typically distributed on the trunk, neck, and extensor surfaces of the upper arms) present at birth or during early childhood and predominantly affect females with skin phototypes IV-V. The lesions are asymptomatic but may rapidly spread from the time they first appear and do not regress.

With only a few cases reported since its initial description, little is known regarding the pathophysiologic basis for FWL. Nevertheless, the familial nature of the disorder, with a strong predilection for females in consecutive generations, is evident in the described cases. This pattern suggests autosomal dominant or X-linked inheritance and points to a genetic component of the disorder's origin.

FWL is a benign chronic condition and does not appear to be associated with other underlying disorders.


L81.8 – Other specified disorders of pigmentation

765195000 – Familial generalized lentiginosis

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Differential Diagnosis & Pitfalls

  • Idiopathic guttate hypomelanosis – This disorder of round white macules typically affects older individuals with lighter skin phototypes and does not exhibit a sex predilection.
  • Vitiligo ponctué (see vitiligo) – Lesions are depigmented, not hypopigmented, and may arise on a hyperpigmented macule.
  • Progressive macular hypomelanosis – Lesions are ill-defined and typically spare the upper extremities and neck. Usually occurs in young adults.
  • Pityriasis alba – Ill-defined white macules with fleeting scale often affecting the cheeks of atopic patients.
  • Leukoderma punctata – Patients will have a long history of topical or systemic PUVA phototherapy.
  • Tinea versicolor – Lesions may be hyperpigmented or erythematous with scaling and are potassium hydroxide (KOH) positive.
  • Leukodermic macules of Darier disease – Onset is in later childhood.
  • Confetti-type macules of tuberous sclerosis – A rarer variant of hypopigmentation that accompanies tuberous sclerosis.

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Last Reviewed:11/19/2017
Last Updated:06/26/2022
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Familial white lentiginosis
A medical illustration showing key findings of Familial white lentiginosis : Neck, Trunk, Widespread distribution, Upper arms
Copyright © 2022 VisualDx®. All rights reserved.