Poikiloderma vasculare atrophicans

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 (skin disease) characterized by hypo- or hyperpigmentation (diminished or heightened skin pigmentation, respectively), telangiectasia and skin atrophy.[3][4][5] Other names for the condition include prereticulotic poikiloderma and atrophic parapsoriasis.[6] The condition was first described by pioneer American pediatrician Abraham Jacobi in 1906.[7] PVA causes areas of affected skin to appear speckled red and inflamed, yellowish and/or brown, gray or grayish-black, with scaling and a thinness that may be described as "cigarette paper".[3] On the surface of the skin, these areas may range in size from small patches, to plaques (larger, raised areas), to neoplasms (spreading, tumor-like growths on the skin).[3][6]

Mycosis fungoides, a type of skin lymphoma, may be a cause of PVA. The condition may also be caused by, associated with or accompany any of the following conditions or disorders: other skin lymphomas, dermatomyositis, lupus erythematosus, Rothmund–Thomson syndrome, Kindler syndrome, dyskeratosis congenita, and chronic radiodermatitis.[4] Rare causes include arsenic ingestion, and the condition can also be idiopathic.[1][3][5]

PVA may be considered a rare variant of cutaneous T-cell lymphoma, a non-Hodgkin's form of lymphoma affecting the skin.[7] It may also be included among a number of similar conditions that are considered as precursors to mycosis fungoides. PVA is believed to be a syndrome closely associated with 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]

  1. ^ a b Howard MS, Smoller BR (Jun 2000). "Mycosis fungoides: classic disease and variant presentations". Seminars in Cutaneous Medicine and Surgery. 19 (2): 91–99. doi:10.1016/S1085-5629(00)80005-X. PMID 10892710.
  2. ^ Diseases Database (DDB): 10208
  3. ^ a b c d Chapman, R. S.; Paul, C. J. (July 1975). "Poikiloderma atrophicans vasculare as a pointer to reticulosis of the skin". Postgraduate Medical Journal. 51 (597): 463–467. doi:10.1136/pgmj.51.597.463. PMC 2496068. PMID 1103107.
  4. ^ a b Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
  5. ^ a b c Lambert WC, Everett MA (Oct 1981). "The nosology of parapsoriasis". J. Am. Acad. Dermatol. 5 (4): 373–395. doi:10.1016/S0190-9622(81)70100-2. PMID 7026622.
  6. ^ a b Bonvalet, D.; Colau-Gohm, K.; Belaïch, S.; Civatte, J.; Degos, R. (Jan 1977). "The difference forms of "parapsoriasis en plaques". A report of 90 cases (author's transl)". Annales de Dermatologie et de Vénéréologie. 104 (1): 18–25. PMID 843023.
  7. ^ a b Kreuter A, Hoffmamm K, Altmeyer P (Apr 2005). "A case of poikiloderma vasculare atrophicans, a rare variant of cutaneous T-cell lymphoma, responding to extracorporeal photopheresis". Journal of the American Academy of Dermatology. 52 (4): 706–708. doi:10.1016/j.jaad.2004.10.877. PMID 15793532.