3.3.11 Parapsoriasis-Group
ICD-11
LP4
Small Plaque Parapsoriasis (SPP) LP19.
Large Plaque Parapsoriasis (LPP) LP40.
Poikilodermia vasculare atrophicans (PVA) LP41.
Synonyms
None
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Xanthoerythroderma perstans
Digitate dermatosis
Atrophic parapsoriasis
Poikilodermatous parapsoriasi
Epidemiology
Rare. Second half of life (>50 years).
Definition
The clinical picture in some cases remains of psoriasis, without any etiopathogenetic relationship. However, there is a nosologic relationship to mycosis fungoides or other CTCL.
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Parapsoriasis is a group of diseases characterized by the presence of scaling macules or patches that can resemble psoriatic lesions. Its importance comes from the fact that some cases can represent (or transform into) cutaneous T-cell lymphomas.
Aetiology & Pathogenesis
Unknown.
Signs & Symptoms
SPP: Small red-brown, indolent (no itch), slightly scaling macules (1-2 cm), following the splitskin lines.
LPP: large geographic irregular but sharply demarcated, slightly scaling macules or plaques; no itch.
PAV: Mottled slightly (pityriasiform) scaling erythematous patches.
Localisation
SPP: predominantly trunk; skin split lines.
LPP: predominantly trunk and upper extremities.
PAV: trunk or total skin.
Classification
Small Plaque Parapsoriasis: usually no evolution into CTCL.
Large Plaque Parapsoriasis: potential precursor of CTCL (mycosis fungoides).
Poikiloderma atrophicans vasculare: potential precursor of CTCL (mycosis fungoides).
Laboratory & other workups
No special, biopsy.
Dermatopathology
Subtle eczematous changes of the epidermis with a few lymphocytes in the dermis and the epidermis. No band like infiltrate in the papilary dermis. No lymphocyte microabscesses in the epidermis.
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Subtle eczematous changes of the epidermis with a few lymphocytes in the dermis and the epidermis. No band like infiltrate in the papilary dermis. No lymphocyte microabscesses in the epidermis. Atrophy of the epidermis and poikilodermia (hyper- and depigmentation) in PAV.Subtle eczematous changes of the epidermis with a few lymphocytes in the dermis and the epidermis. No band like infiltrate in the papilary dermis. No lymphocyte microabscesses in the epidermis. Atrophy of the epidermis and poikilodermia (hyper- and depigmentation) in PAV.
Course
Chronic over years or decades. Follow-up mandatory because of potential development of mycosis fungoides.
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Chronic over years or decades. Follow-up mandatory because of potential development of mycosis fungoides. Multiple biopsies should be taken during the course of the disease.
Complications
LPP and PAV may develop to CTCL, preferentially mycosis fungoides (years or decades).
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LPP and PAV may develop to CTCL, preferentially mycosis fungoides (within years or decades). Some cases represent patients that are in fact mycosis fungoides but we do not have enough clinical-pathologic-molecular criteria to confirm the diagnosis of lymphoma.
Diagnosis
Clinical picture, histopathology and course.
Differential Diagnosis
Atopic or seborrheic eczema, CTCL subtypes in particularmycosis fungoides, tinea corporis, pityriasis versicolor.
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Atopic or seborrheic eczema, CTCL subtypes in particular mycosis fungoides, tinea corporis, pityriasis versicolor, pithyriasis rosea.
Prevention & Therapy
Prevention not possible. Topical therapy: photo-(helio-) or photochemotherapy. Glucocorticosteroid-cream temporarily.
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Prevention not possible. Topical therapy: photo-(helio-) or photochemotherapy. Glucocorticosteroid- cream temporarily. Topical calcineurin inhibitors.
Review Articles
- Burg G, Dummer R, Haeffner A, Kempf W, Kadin M. (2001) From inflammation to neoplasia: mycosis fungoides evolves from reactive inflammatory conditions (lymphoid infiltrates) transforming into neoplastic plaques and tumors. Arch Dermatol. Jul;137(7):949-52. PMID: 11453817.
- Burg, G., & Dummer, R. (1995). Small plaque (digitate) parapsoriasis is an ‘abortive cutaneous T-cell lymphoma’ and is not mycosis fungoides. Arch Dermatol, 131(3), 336-338.
- Chairatchaneeboon M, Thanomkitti K, Kim EJ. (2022) Parapsoriasis - A Diagnosis with an Identity Crisis: A Narrative Review. Dermatol Ther (Heidelb). May;12(5):1091-1102. doi: 10.1007/s13555-022-00716-y. Epub 2022 Apr 15. PMID: 35426607
- Kossard S. (2023) Critical Review-A Tribute to Louis Brocq Lymphomatoid Papulosis, the Key in Exploring the Relationship of Parapsoriasis and Mycosis Fungoides. Am J Dermatopathol. Nov 1;45(11):753-761. doi: 10.1097/DAD.0000000000002546. Epub 2023 Sep 27. PMID: 37782018
- Balan R, Grigoraş A, Popovici D, Amălinei C. (2021). The histopathological landscape of the major psoriasiform dermatoses. Arch Clin Cases. Oct 27;6(3):59-68. doi: 10.22551/2019.24.0603.10155. PMID: 34754910; PMCID: PMC8565680.
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