Review Article

Clinical and Genetic Review of Hereditary Acral Reticulate Pigmentary Disorders

Table 4

Histopathological features, electron microscopy.

DisorderElectron Microscopy

DSHHyperpigmented macules
(i) Many melanosomes in keratinocytes and few in melanocytes
Hypopigmented macules
(i) Small immature melanosomes in the melanocytes with apoptotic changes

DUHHyperpigmented macules
(i) Melanosome complexes

RAPKHyperpigmented macules
(i) Increased number of melanocytes in the basal layer
(ii) Increased melanosomes in keratinocyte cytoplasm

DPRHyperpigmented macules
(i) Increased number and size of melanosomes in the basal keratinocytes

NFJS(i) Colloid-amyloid bodies in the papillary dermis and around sweat glands in the reticular dermis

EBS-MPHyperpigmented macules
(i) Many melanosomes within the basal keratinocytes
(ii) Disorganization of keratin filaments
(iii) Keratinocyte vacuolization

ACD(i) Amyloid fibrils

DSH: dyschromatosis symmetrica hereditaria; DUH: dyschromatosis universalis hereditaria; RAPK: reticulate acropigmentation of Kitamura;DPR: dermatopathia pigmentosa reticularis; NFJS: Naegeli-Franceschetti-Jadassohn Syndrome; EBS-MP: epidermolysis bullosa simplex with mottled pigmentation; ACD: amyloidosis cutis dyschromica.