Lymphomatoid papulosis is one of the primary cutaneous CD30+ T-cell lymphoproliferative
disorders.
Although considered a benign disease, lymphomatoid papulosis has been associated potentially
with an increased risk of secondary hematolymphoid malignancies. In up to 20% of patients, LyP
are preceded by, associated with, or followed by another type of cutaneous or systemic lymphoma,
generally mycosis fungoides (MF), primary cutaneous anaplastic large cell lymphoma (C-ALCL).
Mrs. Rashida, 46 years old lady, presented with multiple pruritic, scaly, erythematous patches
on the face, trunk and both extremities for five years. For last two months she developed
generalized exfoliation and erythema with recurrent corps of multiple nodules on different
parts of the body of variable size. Some nodules became ulcerated. According to clinical and
histopathological findings, she diagnosed as Lymphomatoid papulosis with coexisting MF.
Primary cutaneous CD30+ lymphoproliferative
disorders (PC-CD30+LPDs) are the second most
common group of cutaneous T cell lymphomas
(CTCLs), next to mycosis fungoides (MF), and
account for about 30% of CTCLs.1 The spectrum of
PC-CD30+LPDs comprises primary cutaneous
anaplastic large cell lymphoma (CALCL),
lymphomatoid papulosis (LyP), and borderline cases.
Lymphomatoid papulosis (LyP) is a lympho
proliferative disorder characterized clinically by
recurring crops of red to violaceous self-healing
papules and nodules.2
Patients suffering from one type of cutaneous
lymphoproliferative disorder sometimes develop a
second form of lymphoid disease based in either the
skin or extracutaneous tissues.
In up to 20% of patients, LyP may be preceded by,
associated with, or followed by malignant lymphomas, with MF, Hodgkin’s disease, and CD30+
large cell lymphomas comprising 90% of the
associated lymphomas.1
Histopathologically, 3 subtypes are recognized (some
reviewed 7), namely, type A (histiocytic), type B
(mycosis fungoides—(MF)-like), and type C (anaplastic
large cell lymphoma—(ALCL)-like).1,3,4 All 3 types may
be seen in 1 and the same patient. Type B LyP, the
least common and the most controversial variant, is
considered as a histopathologic simulator of MF.5,6
This type of patients requires special considerations
with regards to work up, staging, treatment and
prognostic counselling.
