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Poikiloderma vasculare atrophicans showing features of ashy dermatosis in the beginning.

Jeon J, Kim JH, Ahn JW, Song HJ - Ann Dermatol (2015)

Bottom Line: In 2005, the lesions began to show darkening and lichenification in the lower part of the trunk.In addition, results of T-cell receptor gene rearrangement analysis were positive.Based on the aforementioned findings, he was diagnosed with PVA.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Korea University Guro Hospital, Seoul, Korea.

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.

No MeSH data available.


Related in: MedlinePlus

(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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Figure 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).

Mentions: In 2001, a 14-year-old male patient presented with asymptomatic brownish black-pigmented reticulated patches over the whole body, particularly on the neck, axillary, and antecubital areas, for an unknown period (Fig. 1A). The results of laboratory studies were all within normal limits or were negative. The histologic examination showed increased number of melanophages in the papillary dermis with focal hydropic changes in the basement membrane zone (Fig. 2A). Ashy dermatosis was considered a possible diagnosis, but the patient was lost to follow-up.


Poikiloderma vasculare atrophicans showing features of ashy dermatosis in the beginning.

Jeon J, Kim JH, Ahn JW, Song HJ - Ann Dermatol (2015)

(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).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4377411&req=5

Figure 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).
Mentions: In 2001, a 14-year-old male patient presented with asymptomatic brownish black-pigmented reticulated patches over the whole body, particularly on the neck, axillary, and antecubital areas, for an unknown period (Fig. 1A). The results of laboratory studies were all within normal limits or were negative. The histologic examination showed increased number of melanophages in the papillary dermis with focal hydropic changes in the basement membrane zone (Fig. 2A). Ashy dermatosis was considered a possible diagnosis, but the patient was lost to follow-up.

Bottom Line: In 2005, the lesions began to show darkening and lichenification in the lower part of the trunk.In addition, results of T-cell receptor gene rearrangement analysis were positive.Based on the aforementioned findings, he was diagnosed with PVA.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Korea University Guro Hospital, Seoul, Korea.

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.

No MeSH data available.


Related in: MedlinePlus