DOI: 10.1097/jd9.0000000000000362 ISSN: 2096-5540

ADAR1 gene mutations in two Chinese pedigrees with dyschromatosis symmetrica hereditaria

Ya-Qi Cao, Chun-Yu Han, Jian-Wen Han
  • Infectious Diseases
  • Dermatology

Introduction:

Dyschromatosis symmetrica hereditaria (DSH) is a rare autosomal dominantly inherited disease with high penetrance. Patients with DSH display a mixture of hyperpigmented and hypopigmented macules on the dorsal aspects of the extremities and freckle-like macules on the face.

Case presentation:

We identified mutations in two Chinese pedigrees with DSH using whole-exome sequencing (WES) and verified these mutations with PCR-Sanger sequencing. We found a previously reported mutation, c.2433_2434del (p.A813Qfs*29), in exon 7 of ADAR1 in the patient of pedigree one and a novel mutation, c.3301G>T(p.V1101F), in exon 13 of ADAR1 in the patients of pedigree two.

Discussion:

We have identified two gene mutations, one of which may be a novel mutation. At present, the main focus is to improve the color of skin lesions. Laser therapy and sun protection can to some extent alleviate skin lesions.

Conclusion:

The findings of the current case have expanded the range of ADAR1 mutations associated with DSH and may be of great help for future clinical genetic counseling.

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