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Primary Cutaneous CD30 + Lymphoproliferative Disorders in a Patient with Severe Atopic Dermatitis:
Is There a Causative Link?
Magdalena ŻYCHOWSKA 1 , Zdzisław WOŹNIAK 2 and Joanna MAJ 1
Department of Dermatology, Venereology and Allergology, and 2 Department of Pathomorphology, Wrocław Medical University, Chałubińskiego
1, PL-50-368 Wrocław, Poland. E-mail: [email protected]
1
Accepted Sep 27, 2017; Epub ahead of print Sep 27, 2017
Primary cutaneous CD30 + lymphoproliferative disorders
comprise 3 entities: lymphomatoid papulosis (LyP),
primary cutaneous anaplastic large cell lymphoma (pc-
ALCL) and borderline diseases (1). LyP is characterized
by the presence of recurrent and self-healing papulo-
necrotic lesions. This entity may be associated with
another lymphoproliferative disease, e.g. pc-ALCL,
mycosis fungoides or Hodgkin’s lymphoma, in 20% of
cases (1, 2). Pc-ALCL predominantly affects older men
and presents as a solitary rapidly growing nodule or tu-
mour in 80% of patients. The disease is characterized by
excellent prognosis with 10-year disease-related survival
rates of 90% (1, 2).
LyP and pc-ALCL are currently considered to be
opposite ends of a spectrum of the same disease. Dif-
ferentiation of these conditions should be based on
clinical presentation and on the course of the disease, as
histopathology may not be decisive (1). In particular, LyP
type C, which is characterized by nodular infiltration of
large atypical lymphoid cells, bears a strong microscopic
resemblance to pc-ALCL (1, 2).
The immunophenotypical hallmark of LyP and pc-
ALCL is the expression of CD30, a transmembrane
protein of the tumour necrosis factor (TNF)-receptor
superfamily, which was found to be associated with
the production of cytokines participating in humoral
responses (3). Caproni et al. (4) found that remarkable
numbers of CD4 + T lymphocytes in lesional skin of
patients with active atopic dermatitis (AD) expressed
CD30. It is suspected, that clonal
transformation of CD30 + T cells in
inflammatory infiltrates may lead to
the development of primary cutaneous
CD30 + lymphoproliferative disorder.
We present here a patient with long-
lasting severe AD, who developed
LyP type C, and several months later
pc-ALCL, with subsequen