Familial progressive hyper- and hypopigmentation

Orpha code: 280628OMIM code: 145250

Definition

Familial progressive hyper- and hypopigmentation is a rare, genetic, skin pigmentation anomaly disorder characterized by progressive, diffuse, partly blotchy, hyperpigmented lesions that are intermixed with multiple café-au-lait spots, hypopigmented maculae and lentigines and are located on the face, neck, trunk and limbs, as well as, frequently, the palms, soles and oral mucosa. Dispigmentation pattern can range from well isolated café-au-lait/hypopigmented patches on a background of normal-appearing skin to confetti-like or mottled appearance.

Disease data
Classification

Disease

Synonyms
FPHH
FPHH
ORPHA code
280628
OMIM code
145250
ICD10 code
L81.8
ICD11 code
-

No additional description.

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