Poikiloderma Vasculare Atrophicans Showing Features of Ashy Dermatosis in the Beginning.
- Author:
Jiehyun JEON
1
;
Joo Ha KIM
;
Jae Woo AHN
;
Hae Jun SONG
Author Information
- Publication Type:Case Report
- Keywords: CD4; CD8; CD4-CD8 ratio; Mycosis fungoides; Poikiloderma; T-lymphocytes
- MeSH: Adolescent; Atrophy; Biopsy; CD4-CD8 Ratio; Diagnosis; Follow-Up Studies; Genes, T-Cell Receptor; Humans; Lymphocytes; Male; Mycosis Fungoides; Skin; Skin Diseases*; T-Lymphocytes; Telangiectasis
- From:Annals of Dermatology 2015;27(2):197-200
- CountryRepublic of Korea
- Language:English
- Abstract: Poikiloderma vasculare atrophicans (PVA) is a rare poikilodermatous variant of early-stage mycosis fungoides characterized by generalized poikiloderma, atrophy, mottled dyspigmentation, and telangiectasia. In 2001, a 14-year-old male presented with asymptomatic brownish-gray polymorphic macules throughout the body with flexural accentuation. A skin biopsy showed increased melanophages with focal hydropic changes. Ashy dermatosis was considered a possible diagnosis. In 2005, the lesions began to show darkening and lichenification in the lower part of the trunk. In 2011, his skin showed definite poikilodermatous changes, and a biopsy showed band-like inflammatory infiltrations of atypical lymphocytes, epidermal atrophy, and epidermotropism of predominantly CD4-CD8+ atypical T cells. In addition, results of T-cell receptor gene rearrangement analysis were positive. Based on the aforementioned findings, he was diagnosed with PVA. If a patient shows long-standing and progressive hyperpigmentary skin changes, periodic follow-up and repeated skin biopsies are recommended to determine the underlying condition.
