Familial white lentiginosis
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
- 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.