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Lymphomatoid papulosis with CD1a+ dendritic cell hyperplasia, mimicking Langerhans cell histiocytosis
Author(s) -
Jokinen Chris H.,
Wolgamot Gregory M.,
Wood Brent L.,
Olerud John,
Argenyi Zsolt B.
Publication year - 2007
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/j.1600-0560.2006.00659.x
Subject(s) - lymphomatoid papulosis , pathology , langerhans cell histiocytosis , histiocyte , cd30 , histiocytosis , population , medicine , langerhans cell , anaplastic large cell lymphoma , biology , lymphoma , immunology , antigen , disease , environmental health
Although CD1a+ dendritic cells (DC) in cutaneous T‐cell lymphomas (CTCL) have been well documented, the presence of large numbers of DC within lymphoid infiltrates can pose a diagnostic difficulty. We present a case of a 70‐year‐old man with a 3‐year history of recurrent red papules and plaques on the extremities and trunk that was referred to our institution, with the diagnosis of Langerhans cell histiocytosis. Skin biopsies showed a wedge‐shaped cellular infiltrate in the superficial and deep dermis consisting of two cell populations. Most prominent were clusters of epithelioid cells with grooved nuclei and abundant eosinophilic cytoplasm, which stained with antibodies to CD1a and S‐100. A second, less prominent population of atypical lymphocytes, some with enlarged, hyperchromatic and convoluted nuclei, were intermixed. The latter were positive for CD30, CD3 and CD5 and negative for CD20, CD34, CD68, ALK‐1 and TdT. T‐cell receptor gene rearrangement studies confirmed a clonal T‐cell population, which with the clinical history was consistent with the diagnosis of lymphomatoid papulosis. While previous studies have shown an increased density of dermal DC in CTCL, we believe that this represents the first report of an unusually florid DC proliferation mimicking Langerhans cell histiocytosis and masking a lymphoproliferative disorder.