Review Article

Clinical and Genetic Review of Hereditary Acral Reticulate Pigmentary Disorders

Table 3

Histopathological features, light microscopy.

DisorderLight Microscopy

DSHHyperpigmented macules
(i) Increase in melanin with pigmentary incontinence in the basal layer
(ii) Increased melanocytes sizes with elongated dendrites
Hypopigmented macules
(i) Decrease in melanin pigments and number of melanocytes

DUHHyperpigmented macules
(i) Increase in melanin with pigmentary incontinence in the basal layer
Hypopigmented macules
(i) Decreased melanin deposition in the basal layer

RAPKHyperpigmented macules
(i) Increase in melanin in the basal layer with no pigmentary incontinence
(ii) Hyperkeratosis with no parakeratosis

DPR(i) Mild orthokeratosis, papillomatosis
(ii) Heavily pigmented epidermis with pigmentary incontinence
(iii) Interface dermatitis
(iv) Superficial perivascular inflammations

NFJSHyperpigmented lesions
(i) Increase in melanin in the basal layer with pigmentary incontinence

EBS-MPHyperpigmented macules
(i) Epidermal atrophy
(ii) Increased pigmentation in the basal cells with pigmentary incontinence

ACDHyper- and hypopigmented macules
(i) Eosinophilic material (amyloid) in papillary dermis
(ii) Melanin pigment incontinence

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.