Vesiculo-bullous Darier disease late onset in association with polycythemia vera

نویسندگان

چکیده

Vesiculo-bullous lesions as well the late onset are extremely rare features of Darier disease (DD) making positive diagnosis more difficult. Its association with polycythemia vera prompts to look for signs suggestive malignant hematological transformation. We report a case 62-year-old patient, followed in internal medicine since age 58 vera. She presented pruriginous vesiculo-bullous dermatosis evolving 4 years. On clinical examination, papulo-vesicular plaques were found involving all folds: inguinal, axillary, inter-gluteal, neck and body palmo-plantar blisters pustules. Examination toe nails showed alternating red white longitudinal bands. Abdominal examination moderate splenomegaly. Skin biopsy revealed focal acantholysis supra-basal clefts, dyskeratosis presence round bodies grains compatible Darier's disease. A paraneoplastic assessment was made including thoraco-abdomino-pelvic CT scan, breast ultrasound mammography cervico-vaginal smear, scan cavum pancreatic MRI, which did not show any progressive process. The patient put on oral retinoids at rate 0.5 mg/kg per day topical calcipotriol associated bloodletting reduce hematocrit level. evolution marked by improvement. hematology department no acute leukemia or myelofibrosis other neoplasms after 2-year follow-up. DD woman vera, characterized presentation whose causal link remains be determined.

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ژورنال

عنوان ژورنال: International Journal of Research in Dermatology

سال: 2023

ISSN: ['2455-4529']

DOI: https://doi.org/10.18203/issn.2455-4529.intjresdermatol20231171