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J Dermatol Case Rep. 2014 Jun 30;8(2):58-9. doi: 10.3315/jdcr.2014.1174. eCollection 2014 Jun 30.

Photoletter to the editor: A neurocutaneous rarity: phacomatosis pigmentokeratotica.

Journal of dermatological case reports

Thiago Cardoso Vale, David Márcio Barbosa Santos, Ricardo Oliveira Maciel, Francisco Cardoso, Rudolf Happle

Affiliations

  1. Neurology Division, University Hospital, Faculty of Medicine, Federal University of Juiz de Fora (UFJF), Juiz de Fora, Minas Gerais, Brazil;
  2. Neurology Division, University Hospital, Faculty of Medicine, Federal University of Minas Gerais (UFMG), Belo Horizonte, Minas Gerais, Brazil;
  3. Neurology Division, Department of Internal Medicine, Faculty of Medicine, Federal University of Minas Gerais (UFMG), Belo Horizonte, Minas Gerais, Brazil;
  4. Department of Dermatology, Freiburg University Medical Center, Freiburg, Germany.

PMID: 25024780 PMCID: PMC4094740 DOI: 10.3315/jdcr.2014.1174

Abstract

Phacomatosis pigmentokeratotica is characterized by the coexistence of nevus sebaceus, papular nevus spilus and associated neurologic abnormalities. We report a case of phacomatosis pigmentokeratotica in a 28-year-old male who presented with palmar-plantar dysesthesia and ipsilateral brain hemiatrophy. As a characteristic neuroimaging finding of the disorder, we found multiple hypointense lesions involving the ipsilateral hemisphere.

Keywords: atrophy; bones; congenital abnormalities; dysesthesia; neuropathy; nevus sebaceous; nevus spilus; unilateral

References

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