Poikiloderma vasculare atrophicans

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Poikiloderma vasculare atrophicans
Other namesParapsoriasis variegata[1] or Parapsoriasis lichenoides[2]
Typical skin changes and discoloration described as poikiloderma vasculare atrophicans
SpecialtyDermatology Edit this on Wikidata

Poikiloderma vasculare atrophicans (PVA), is a cutaneous condition (

plaques (larger, raised areas), to neoplasms (spreading, tumor-like growths on the skin).[3][6]

Mycosis fungoides, a type of skin

idiopathic.[1][3][5]

PVA may be considered a rare variant of

large-plaque parapsoriasis and its cohort retiform parapsoriasis; including PVA, all three conditions fit within an updated view of the once ambiguous classification scheme known as parapsoriasis.[5]

Presentation

The layers of the epidermis (left). Melanocytes (rlght), located in the bottom epidermal layer, produce melanin.

PVA can be characterized by speckled, combined hyper- and hypopigmentation in the plaques or patches of affected skin.

stratum basale (bottom cell-layer) of the epidermis.[5] Atrophy of the skin gives it a thin, dry and wrinkled appearance, which in PVA-affected individuals has been described as "cigarette paper".[7] Hyperkeratosis, a thickening of the stratum corneum (top cell-layer of the epidermis), has also been reported.[5][9]

Cause

PVA usually has an underlying cause, attributed to existing skin diseases and disorders associated with a cutaneous lymphoma or inflammation.

idiopathic (of unknown cause), as seen in a small number of cases.[5]

Diagnosis

Classification

Poikiloderma vasculare atrophicans, or PVA, indicates that extra or altered skin pigmentation ("poikiloderma")[10] is occurring, associated with heightened visibity of capillaries ("vasculare", referring to telangiectasia) under the skin, related to thinning and wasting away ("atrophicans") of the skin and its tissue. Telangiectasia is an enlargement of capillaries underneath the skin.[10]

PVA also has common names that include parapsoriasis-related terminology (i.e. parapsoriasis variagata, or "variegated" parapsoriasis).[5] Parapsoriasis is a term first used by Brocq in 1902,[11] intended to represent a group comprising a number of uncommon skin disorders, under a once used, now antiquated classification scheme for all inflammatory dermatoses (skin diseases known to be associated with or cause inflammation).[5] Brocq chose the term "parapsoriasis" to illustrate that the dermatoses placed in this group had or would have commonalities with psoriasiasis, including appearance and chronicity (lifelong or indefinite duration).[5] This poorly designated grouping has led to confusion in establishing a nosology (a method of classifying diseases and disorders) that associated or distinguished these disorders, and through the years differing opinions and uses regarding parapsoriasis by both authors and physicians has caused further confusion.[5] In more recent times, after much discussion and growing consensus, parapsoriasis and its terminology has been revisited and re-examined often. Newer thought on parapsoriasis, such as by Sutton (1956)[12] all the way to that by Sehgal, et al. (2007)[13] has cleared much of the confusion and has sparked increased understanding of parapsoriasis and its constituents.

PVA fits within this updated view of parapsoriasis as a syndrome often associated with large plaque parapsoriasis and, or including its variant form, retiform parapsoriasis.[5] Additionally, it may be considered a precursor or variant of the lymphomatous skin disorder mycosis fungoides, which is also associated with large plaque parapsoriasis.[5] Large plaque parapsoriasis consists of inflamed, oddly discolored (such as yellow or blue), web-patterned and scaling plaques on the skin, 10 cm (3.9 in) or larger in diameter.[5] When the condition of the skin encompassed by these plaques worsens and becomes atrophic, it is typically considered retiform parapsoriasis.[5] PVA can occur in either the large plaque or retiform stage, but it can only be considered PVA when its three constituents (poikiloderma, telangiectasia, atrophy) are present.[5] PVA is therefore considered an independent syndrome identified by its constituents, wherever it occurs.[5]

In modern consideration and usage, the solitary term "poikiloderma" has also come to represent all three elements of PVA.[5] When skin diseases and disorders or skin conditions described as dermatoses contain the term poikiloderma in their assessment or diagnosis (such as with Bloom syndrome), this can sometimes be an erroneous usage of the term.[5] Discretion has been advised.[5] Usage of the entire term "poikiloderma vasculare atrophicans" may also be reserved to indicate it as the primary condition affecting the skin in cases where the disorder associated with it is secondary.[5]

See also

  • List of cutaneous conditions

References

  1. ^
    PMID 10892710
    .
  2. ^ Diseases Database (DDB): 10208
  3. ^
    PMID 1103107
    .
  4. ^
    ISBN 978-1-4160-2999-1
    .
  5. ^
    PMID 7026622
    .
  6. ^
    PMID 843023
    .
  7. ^
    PMID 15793532
    .
  8. ^ "A definition of the term "hyperpigmentation" from Aocd.com". Retrieved Feb 6, 2010.
  9. ^ "A definition of the term "hyperkeratosis" from Everydayhealth.com". Retrieved Feb 6, 2010.
  10. ^ a b "A definition of the term "poikiloderma" from Medterms.com". Retrieved Dec 28, 2009.
  11. ^ Brocq L (1902). "Les parapsoriasis". Ann Dermatol Syphiligr (Paris). 3: 433–468.
  12. ^ Sutton RL (1956). Diseases of the skin. St. Louis, Mo.: The C. V. Mosby Co. pp. 936–941.
  13. PMID 17975354
    .

External links

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