Ann Dermatol.  2015 Apr;27(2):197-200. 10.5021/ad.2015.27.2.197.

Poikiloderma Vasculare Atrophicans Showing Features of Ashy Dermatosis in the Beginning

• Jeon J
• Kim JH
• Ahn JW
• Song HJ

• KMID: 2264811
• DOI: http://doi.org/10.5021/ad.2015.27.2.197

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.

Keyword

CD4; CD8; CD4-CD8 ratio; Mycosis fungoides; Poikiloderma; T-lymphocytes

MeSH Terms

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

Figure

• 

  Fig. 1 (A) Dusky, dark brownish pigmented reticulated patches over the whole body. (B) Scaly increased darkening and lichenification on the previous lesions. (C) Additional prominent poikilodermatous change in the previous skin lesions. (D) The poikilodermatous lesions have disappeared after narrow-band ultraviolet B phototherapy.

• 

  Fig. 2 (A) Increased number of melanophages seen in the papillary dermis with focal hydropic changes. (B) Interface dermatitis with exocytosis and necrotic keratinocytes. (C) Epidermal atrophy and lymphocytic infiltration in the upper dermis with epidermotropism of atypical lymphocytes, which are mainly CD4(red)-CD8(blue)+(arrow). (D) After phototherapy, the epidermal atypical lymphocytes are not observed. (A~D: H&E, ×100; A'~D': CD4~CD8 double stain, ×100; arrows: epidermotrphism of lymphocytes).

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