Dermatopathia Pigmentosa Reticularis Clinical Presentation

Updated: Nov 22, 2019
  • Author: Melba Estrella, MD; Chief Editor: Dirk M Elston, MD  more...
  • Print
Presentation

History

The primary clinical feature of dermatopathia pigmentosa reticularis (DPR) is the occurrence of reticulate hyperpigmented macules at birth or in early childhood, usually by age 2 years.

The hyperpigmentation persists throughout life, showing no tendency of spontaneous fading. The reticulate network of hyperpigmented macules occurs particularly on the trunk, neck, and proximal areas of the limbs.

Next:

Physical Examination

Most reported cases of dermatopathia pigmentosa reticularis (DPR) have demonstrated a clinical triad of reticulate hyperpigmentation, mild nonscarring alopecia, and mild onychodystrophy. Al-Hamdi et al [21] reported a case of DPR with late onset of both alopecia and onychodystrophy, proposing that the reticulate hyperpigmentation may be the only feature present before puberty. Other associated features may include the following:

  • Punctate or diffuse palmoplantar hyperkeratosis

  • Darkly pigmented nipples

  • Nonscarring blisters on the dorsa of hands and feet following minor trauma or sun exposure

  • Hyperhidrosis or hypohidrosis

  • Adermatoglyphia (loss of dermal ridges on fingers and toes)

  • Thin eyebrows and sparse pubic and axillary hair

It is possible that Naegeli-Franceschetti-Jadassohn syndrome (NFJS), poikiloderma Clericuzio type, and dyskeratosis congenita can overlap, with a presentation of prenatal and postnatal growth restriction, developmental delay, microcephaly, and pigmentation pathology. [22]

Previous
Next:

Complications

No serious complications are known for dermatopathia pigmentosa reticularis (DPR). A few cases of DPR showing extracutaneous manifestations have been addressed in the literature as probable coincidences rather than complications.

Early-onset gastric carcinoma in a male with DPR was noted by Tunca et al in 2008. [23]

Goh et al [19] reported a case of DPR caused by a recurrent KRT14 mutation in which the patient had digital fibromatosis and wiry scalp hair. [19]

Very few cases of DPR have been described to present with corneal opacity and superficial punctate spots, but only one case has been reported presenting bilateral Salzmann’s nodular degeneration of the cornea. [2, 20]

Previous