Condition

Age of Onset of Dyspigmentation

Nature of Dyspigmentation

Ethnic Predisposition

Inheritance Pattern

Associated Features

Dyskeratosis Congenita

1^st decade of life

Hyperpigmentation

None

X-linked (males)

Autosomal (Both sexes)

Nail dystrophy, oral leukoplakia, bone marrow failure

Reticulate Acropigmentation of Kitamura

1^st or 2^nd decade of life

Hyperpigmentation over dorsal aspects of hands and feet

Japanese

Autosomal Dominant

Linear palmar pits

Dyschromatosis Universalis Hereditaria

Infancy and early childhood

Hypo- and hyperpigmenation over trunk, abdomen, and limbs

Asian

Autosomal Dominant

None

Xeroderma Pigmentosum

First 2 years of life

Hyperpigmentation over sun exposed areas

None

Autosomal Recessive

Sun sensitivity, freckling, cutaneous maliganacies

Naegeli-Franceschetti-Jadassohn Syndrome

Infancy and early childhood

Non-inflammatory reticulate pigmentation of neck and flexural skin

None

Autosomal

Dominant

Ectodermal dysplasia with defects in sweat glands, teeth, and nails

Epidermolysis Bullosa Simplex with Mottled Pigmentation

Infancy

Mottled pigmentation of limbs and trunk

None

Autosomal Dominant

Intraepidermal blistering, nail dystrophy, punctuate palmoplantar hyperkeratosis

Dowling-Degos’ Disease

Adolescence or adulthood

Hyperpigmentation of flexural skin

None

Autosomal Dominant

Comedonal/cystic lesions over the back or neck, pitted perioral scars, speckled macules over genitalia