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lymphomatoid papulosis

Thursday 2 September 2010

Definition: Lymphomatoid papulosis (LyP) is a recurrent, self-healing eruption belonging to the spectrum of cutaneous CD30+lymphoproliferative disorders.

Lymphomatoid papulosis is a chronic, recurrent, self-healing papulonodular skin rash with features of a CD30+ malignant T cell lymphoma.

The prognosis is good, but patients have an increased risk of developing a cutaneous or nodal lymphoma, such as primary cutaneous anaplastic large cell lymphoma, mycosis fungoides, and Hodgkin’s lymphoma.

Three main histologic subtypes of LyP are recognized:
 type A (histiocytic),
 type B (mycosis fungoides-(MF)-like),
 type C (anaplastic large cell lymphoma-like).

A cytotoxic variant of LyP may be histopathologically indistinguishable from primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, and may be the source of pitfalls in the diagnosis and classification (LyP type D).

References

 A variant of lymphomatoid papulosis simulating primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma. Description of 9 cases. Saggini A, Gulia A, Argenyi Z, Fink-Puches R, Lissia A, Magaña M, Requena L, Simonitsch I, Cerroni L. Am J Surg Pathol. 2010 Aug;34(8):1168-75. PMID: 20661014