Dyschromatosis universalis hereditaria in a young Nigerian female

Abstract

Dyschromatosis universalis hereditaria (DUH) is a clinically heterogeneous disorder that shows generalized mottled pigmentation. It occurs most commonly in Japanese persons, with sporadic reports from South Africa, India, and Iraq. Histopathology reveals a variable degree of pigmentary incontinence. Although the precise etiology of this disorder is not yet known, the clinicopathological findings implicate an inherent abnormality of melanosomes or melanin processing. We describe a case in a young Nigerian girl.

Hyperkeratosis lenticularis perstans (Flegel’s disease) with unusual clinical presentation. Response to isotretinoin therapy.

“…2 Clinical reports have been described in Japan, China, Taiwan, Tunicia, India, Iraq, Saudi Arabia and Niger, South America and South Africa. In Europe is a rare disease and it has been described as isolated clinical cases with an indian background.…”

“…Dyschromatosis symmetrica hereditaria (DHS) presents an acral distribution and unilateral dermatomal pigmentary disorder (UDPD) segmental lesions. 1,2 DUH has a difficult differential diagnosis. Early diagnose is very important in order to detect DUH associated comorbidities.…”

“…The keratinocytes and melanocytes of the hyperpigmentated lesions contained numerous, fully melanized melanosomes and, in contrast, the melanosomes were absent from both keratinocytes and melanocytes of hypopigmentated lesions. 2,4 b) Interference with the neural-melanocytic interaction in early embriogenic life could explain deffects in melanocytes maduration. 1 DHU has a heterogeneous phenotype.…”

“…In Europe is a rare disease and it has been described as isolated clinical cases with an indian background. 1,2,3,4,5 Autosomical dominant (AD), recessive (ar) and sporadic inheritance have been described. However AD inheritance is most common.…”

“…The face is affected only in 50% of cases and soles-palms and mucoses are usually unaffected. 1,2 Several comorbidities have been associated with DHU as short stature, photosensitivity, theeth abnormalities, deafness and cataracts. diagnosis with other entities as other dyschromatiosis, generalized reticulate pigmentary dermatosis (Naegeli-Franceschetti-Jadassohn, dermatophatia pigmentosa reticularis or dyskeratosis congenita), xeroderma pigmentosum, residual leukoderma, amyloidosis cutis discromia and vitiligo should be stablished.…”