A case of segmental Darier's disease
SONG Ao-lin
CUI Bing-nan
XU Chen-chen
Abstract：A 25-year-old female patient has prutitic lesionson the right side of her axilla and antecubital fossa more than 7 years.Dermatological examination was notable for the presence of brownish to redbrownish-red,flat-topped papules,which were in a segmentally distribution on the right side of her axilla and antecubital fossa.Dermoscopic examination,on the right side of axilla,showed a central polygonal irregular yellowish area with crusts surrounded by a whitish halo on a purplish background,while on the right side of axilla,showed several irregular to star-shaped brownish areas.Histopathologic examination taken from her axilla showed focal suprabasilar acantholysis accompanied by dyskeratotic keratinocytes both in the stratum spinosum and stratum corneum.Dyskeratotic cells in the stratum spinosum showed pyknotic nuclei and enlarged homogeneous eosinophilic cytoplasm,whereas basophilic elongated nuclei were observed in dyskeratotic cells of the stratum corneum,associated to corps ronds.Superficial inflammatory perivascular infiltration of lymphocytes were also noticed.Genetic testing of blood samples revealed a mutation in the ATP2A2 gene(-),and the gene detection of local skin lesions showed that ATP2A2 gene mutation(+).She was diagnosed as segmental keratosis follicularis,and was treated with 0.1%retinoic acid cream for topical application.The skin lesions were stable and gradually subsided.This patient is still being followed up.
Keywords：Darier's diseasesegmentalGenetic test
Publication Date：2023-10-10
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：3( 315-317 )