ARTICLE
Histopathological plasmacytoid differentiation and immunohistochemical
stainings of the dermal infiltrate, showing a strong positivity for L26
and CD 79 and no expression of Bcl-2, and molecular analysis revealing
a clonal rearrangement of immunoglobulin genes was consistent with the
diagnosis of cutaneous marginal zone B cell lymphoma. Computed tomography
scan of the whole body and a bone marrow specimen were performed showing
no abnormalities. We diagnosed primary cutaneous marginal zone B cell
lymphoma or skin associated lymphoid tissue (SALT)-related B-cell lymphoma.
In 1983, the concept of MALT lymphoma was developed by
Isaacson and Wright who noted that gastrointestinal tract low grade B
cell lymphomas have similar morphological features to the mucosa associated
lymphoid tissue [1]. In 1991, Santucci et al. emphasized the clinical
similarities between primary cutaneous B cell lymphoma and MALT lymphoma,
such as a localized involvement, a good response to local treatment and
a low tendency to disseminate [2]. Immunophenotypical and genotypical
features of the majority of primary cutaneous B cell lymphoma show the
absence of expression of CD5 and CD10 antigens, the absence of translocation
t(11; 14)/t(14; 18) and variable expression of bcl-2 gene rearrangement
suggesting a probable origin in the marginal zone B cells comparable to
MALT lymphomas [3-5]. So far, primary cutaneous marginal zone B cell lymphoma
with these similarities to Malt lymphomas are called SALT-related B-cell
lymphomas [5, 6]. The development of SALT-related B-cell lymphomas is
unclear and regarded as being dependent on the acquisition of B-cell lymphoid
tissue [2]. Several antigenic stimuli including tattoo reactions and borrelia
burgdorferi infection which are known to result in the development
of cutaneous lymphoid hyperplasia have been incriminated with the subsequent
development of SALT-related B-cell lymphomas [6, 7].
The clinical appearance of SALT-related B-cell lymphomas
is usually red to violaceous painless nodules with a smooth or slightly
mamitted surface growing slowly, localized essentially on the head, the
trunk and the neck, with a regional distribution [8]. Ulcerative or squamous
characters are rarely seen. Histopathological findings reveal a dense
dermal polymorphous tumoral infiltrate with cells mimicking small centrocytes
and centroblasts and plasmacytoid cells. The proportion of each of these
cell types is variable depending essentially on the age, the size and
the growth rate of lesions [3]. Predominant plasma cell component and
k light chain restriction pattern are sometimes observed in SALT-related
B-cell lymphomas. These features lead some authors to think that extramedullary
plasmacytomas not associated with multiple myeloma are a form of SALT-related
B-cell lymphomas [9].
So, concerning our observation,
two particularities must be underlined.
On one hand, the clinical appearance as xanthomatous papules
is unusual and, on the other hand, histopathological features revealing
a predominant plasmacytoid component can be a source of confusion with
infectious diseases, plasmocytic haematological disorders. The concept
of follicular center-cell lymphomas and immunocytomas which can also exhibit
a plasmacytoid contingent on histopathological examination must be discussed.
In our case, the diagnosis of center-follicular cell lymphoma seems excluded
because of the presence of a dense plasmacytoid differentiation and the
absence of an evident infiltration of true centrocytes or centroblasts.
Immunocytomas are considered according to EORTC and REAL classifications
as a SALT-related B-cell lymphomas [10].
As with the majority of SALT-related B-cell lymphomas,
no other locations of the disease were found and an orthovolt radiotherapy
was decided with a partial remission confirmed by a new cutaneous biopsy
specimen performed on a papular sequellar zone. (Fig.
5). Chemotherapy with chlorambucil was administrated with complete
clinical remission without reccurrence after one year follow up.
Article accepted on 26/3/00
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