Dermatopathia Pigmentosa Reticularis Differential Diagnoses

Updated: Jun 22, 2023
  • Author: Melba Estrella, MD; Chief Editor: Dirk M Elston, MD  more...
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Diagnostic Considerations

The diagnosis of dermatopathia pigmentosa reticularis (DPR) is based on the presence of the clinical triad and a typical histopathologic picture; however, the following hyperpigmentation disorders should be considered and excluded:

  • Acromelanosis progressiva

  • Hereditary symmetric dyschromatosis of Dohi

  • Reticular acropigmentation of Kitamura

  • Heterochromia extremitarium

  • Reticulate pigmented dermatosis of the flexures (Dowling-Degos disease)

  • Congenital diffuse mottling of the skin

  • Hereditary universal dyschromatosis

  • Dyskeratosis congenita syndrome

  • Franceschetti-Jadassohn or Naegeli syndrome

Points of differentiation

Each of the following demonstrate a distinctive distribution of pigmentation with diffuse frecklelike or reticulate hyperpigmentation on the dorsa of the hands and feet, the extremities, or the flexural areas (axillae, groin): acromelanosis progressiva, hereditary symmetric dyschromatosis of Dohi, reticular acropigmentation of Kitamura, heterochromia extremitarium, and reticulate pigmented dermatosis of the flexures.

Differentiation from congenital diffuse mottling of the skin can be made histopathologically by demonstrating clubbing of the rete ridges and hyperpigmentation of the basal layer without pigmentary incontinence.

Hereditary universal dyschromatosis is associated with hyperpigmented and hypopigmented macules of irregular shape and size, as well as small stature and high-tone deafness.

Dyskeratosis congenita syndrome is an X-linked condition and occurs most commonly in males, usually in males older than 5 years. In addition to reticulate hyperpigmentation, nail dystrophy, and hyperkeratosis and atrophy of the palms and soles, patients with dyskeratosis congenita also have leukokeratosis of mucosal surfaces and blood dyscrasias.

Franceschetti-Jadassohn (Naegeli) syndrome is an autosomal dominant condition with gray-brown reticulate pigmentation of the trunk and neck that fades after adolescence. Dental anomalies and a bleeding tendency are frequently observed in these patients.

Franceschetti-Jadassohn (Naegeli) syndrome and DPR share complete absence of dermatoglyphics; a reticulate pattern of hyperpigmentation; palmoplantar keratoderma; abnormal sweating; and anomalies of the teeth, hair, and skin. [24]

Differential Diagnoses