Poikiloderma Vasculare Atrophicans Showing Features of Ashy Dermatosis in the Beginning
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Poikiloderma vasculare atrophicans (PVA) is a rare poikilodermatousvariant of early-stage mycosis fungoides characterizedby generalized poikiloderma, atrophy, mottled dyspigmentation,and telangiectasia. In 2001, a 14-year-oldmale presented with asymptomatic brownish-gray polymorphicmacules throughout the body with flexural accentuation. A skin biopsy showed increased melanophages withfocal hydropic changes. Ashy dermatosis was considered apossible diagnosis. In 2005, the lesions began to show darkeningand lichenification in the lower part of the trunk. In2011, his skin showed definite poikilodermatous changes,and a biopsy showed band-like inflammatory infiltrations ofatypical lymphocytes, epidermal atrophy, and epidermotropismof predominantly CD4−CD8＋ atypical T cells. In addition,results of T-cell receptor gene rearrangement analysiswere positive. Based on the aforementioned findings, he wasdiagnosed with PVA. If a patient shows long-standing andprogressive hyperpigmentary skin changes, periodic follow-up and repeated skin biopsies are recommended to determinethe underlying condition.
Abstract
Poikiloderma vasculare atrophicans (PVA) is a rare poikilodermatousvariant of early-stage mycosis fungoides characterizedby generalized poikiloderma, atrophy, mottled dyspigmentation,and telangiectasia. In 2001, a 14-year-oldmale presented with asymptomatic brownish-gray polymorphicmacules throughout the body with flexural accentuation. A skin biopsy showed increased melanophages withfocal hydropic changes. Ashy dermatosis was considered apossible diagnosis. In 2005, the lesions began to show darkeningand lichenification in the lower part of the trunk. In2011, his skin showed definite poikilodermatous changes,and a biopsy showed band-like inflammatory infiltrations ofatypical lymphocytes, epidermal atrophy, and epidermotropismof predominantly CD4−CD8＋ atypical T cells. In addition,results of T-cell receptor gene rearrangement analysiswere positive. Based on the aforementioned findings, he wasdiagnosed with PVA. If a patient shows long-standing andprogressive hyperpigmentary skin changes, periodic follow-up and repeated skin biopsies are recommended to determinethe underlying condition.
참고문헌 (12)
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