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http://dx.doi.org/10.1097/DAD.0000000000002891DOI Listing

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Article Synopsis
  • Segmental Darier disease (DD) is a rare skin condition involving mutations in the calcium ATPase pump, with two types: type 1, which follows Blaschko's lines, and type 2, which features increased severity in generalized cases.
  • Type 1 segmental DD is challenging to diagnose due to its late onset, lack of family history, and similar features to other skin conditions like lichen planus and psoriasis.
  • The report documents two cases, highlighting symptoms, dermoscopic findings, histopathological correlations, and effective treatment with tretinoin gel for the first patient.
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A case of phakomatosis pigmentovascularis (PPV) type III a with a zosteriform distribution of nevus spilus as the main manifestation was reported. A 41-year-old man was born with a zosteriform distribution of pigmented rash on the left half-body, namely upper limb, shoulder and back. Physical examination revealed light brown pigments in a giant zosteriform distribution on the extensor side of the left upper limb and the left shoulder and back, with scattered brown spots and patches of variant sizes on the surface, which are consistent with the appearance of nevus spilus (NS).

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We report a rare and interesting case of a combined linear, Blaschkoid and zosteriform pattern of lichen planus pigmentosus. Dermoscopy showed discrete bluish-grey dots, globules, blotches and rods against a brownish background. A skin biopsy confirmed the diagnosis from the presence of civatte bodies, melanin incontinence and band-like inflammation.

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