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Lymphomatoid papulosis

Field	Value
name	Lymphomatoid papulosis

| Lymphomatoid papulosis (LyP) is a rare skin disorder.

Prevalence

The overall prevalence rate of lymphomatoid papulosis is estimated at at least 1.2 cases per 1,000,000 population. This rare condition has only been studied in depth since 1968.

Presentation

It can appear very similar to anaplastic large cell lymphoma. Type "A" is CD30 positive, while type "B" is CD30 negative.

It has been described as "clinically benign but histologically malignant."

A	B	C	D	E	F
Wedge-shaped clusters of large atypical lymphocytes that are CD30+, interspersed with a mixed inflammatory infiltrate of neutrophils, histiocytes, and eosinophils.
Similar to mycosis fungoides, with bandlike infiltrate and epidermotropism of smaller atypical lymphocytes that may be CD30-.
Similar to cutaneous anaplastic large-cell lymphoma, with larger clusters or sheets of large anaplastic CD30+ cells without the interspersed mixed infiltrate of Type A.
Similar to CD8+ epidermotropic cutaneous T-cell lymphoma, with large CD8+ and CD30+ lymphocytes that often stain with cytotoxic markers (TIA-1, granzyme, perforin).
Angioinvasive with small to large angiocentric CD30+ atypical lymphocytes that invade walls of small to medium vessels in dermis or subcutaneously.
Perifollicular infiltrates of CD30+ atypical cells with folliculotrophism with or without follicular mucinosis.

Treatment

It may respond to methotrexate or PUVA.

Prognosis

It can evolve into lymphoma.

References

Helen H. Wang. (December 1992). "Epidemiology of Lymphomatoid Papulosis". The Cancer Journal.
Macaulay WL. (January 1968). "Lymphomatoid papulosis. A continuing self-healing eruption, clinically benign--histologically malignant". Arch Dermatol.
(April 2004). "Lymphomatoid papulosis: reappraisal of clinicopathologic presentation and classification into subtypes A, B, and C". Arch Dermatol.
(2006). "Clinical hematology". Elsevier Health Sciences.
Maria Proytcheva. (14 March 2011). "Diagnostic Pediatric Hematopathology". Cambridge University Press.
Hughey LC. (2015). "Practical Management of CD30⁺ Lymphoproliferative Disorders.". Dermatol Clin.
(April 2006). "CD8+ lymphomatoid papulosis and its differential diagnosis". [[American Journal of Clinical Pathology]].
(2006). "Lymphomatoid papulosis localized to the face". Dermatol. Online J..

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rare-diseases lymphoid-related-cutaneous-conditions

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