3.3.11 Parapsoriasis-Group

Grading & Level of Importance: C

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.

References

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