Poikiloderma vasculare atrophicans (PVA) is considered by some authors to be a rare variant of early patch-stage mycosis fungoides (MF) and by others to be a precursor to MF. It has also been referred to as poikiloderma atrophicans vasculare or poikiloderma vasculare atrophicans of Jacobi-Lane, or "poikilodermatous" variant of MF.
PVA is characterized by atrophic patches with telangiectasia and mottled pigmentation, usually on the trunk and flexural areas. The condition is typically asymptomatic; however, there may be pruritus.
Onset is typically between 40 and 60 years of age, with a slight male predilection. Initial presentation may be with one to a few patches that persist for many years. Over time, the lesions may remain stable in size or gradually enlarge or thicken as well as increase in number. Wrinkled, thin plaques that are not poikilodermatous and that resemble patch-stage MF may also be present.
Progression to a more advanced stage of MF is not reliably predictable, and the disease may remain quiescent for many years or indefinitely.
Poikiloderma vasculare atrophicans
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Synopsis
Codes
ICD10CM:
L94.5 – Poikiloderma vasculare atrophicans
SNOMEDCT:
16341002 – Poikiloderma vasculare atrophicans
L94.5 – Poikiloderma vasculare atrophicans
SNOMEDCT:
16341002 – Poikiloderma vasculare atrophicans
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Differential Diagnosis & Pitfalls
The triad of atrophy, mottled pigmentation, and telangiectasia (poikiloderma) can be seen in several other conditions:
- Chronic radiodermatitis
- Burns (see thermal or electrical burn; chemical burns are covered separately, by chemical agent)
- Dermatomyositis – Poikilodermatous changes are usually in areas of chronic erythema; histologic findings are consistent with connective tissue disease, eg, dermal mucin and interface changes.
- Systemic lupus erythematosus
- Acrodermatitis chronica atrophicans – A late stage of active infection with Borrelia afzelii. Affected areas may have dark-reddish discoloration; cryoglobulins are positive in the majority of patients.
- Localized scleroderma – Presents with indurated lesions initially with progression to atrophy with a violaceous border.
- Lichen sclerosus – Presents as ivory to porcelain-white colored plaques and papules with fissuring, telangiectasia, and sometimes erosion.
- Poikiloderma of Civatte – Erythema with mottled hyperpigmentation, usually on the sun-exposed areas of the neck and upper chest.
- Genodermatoses including Rothmund-Thomson syndrome (poikiloderma congenitale), Bloom syndrome (congenital telangiectatic erythema), dyskeratosis congenita, Cockayne syndrome, and xeroderma pigmentosum – Usually will have other history and findings associated with syndrome.
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Last Reviewed:09/23/2018
Last Updated:10/07/2018
Last Updated:10/07/2018
Poikiloderma vasculare atrophicans