Primary cutaneous γδ T-cell lymphomas (PCGD-TCLs) are considered a subgroup of aggressive cytotoxic T-cell lymphomas (CTCLs). We have taken advantage of a new, commercially available antibody that recognizes the T-cell receptor-γ (TCR-γ) subunit of the TCR in paraffin-embedded tissue. We have analyzed a series of 146 primary cutaneous T-cell lymphomas received for consultation or a second opinion in the CNIO Pathology Department. Cases were classified according to the World Health Organization 2008 classification as mycosis fungoides (MF; n=96), PCGD-TCLs (n=5), pagetoid reticulosis (n=6), CD30(+) primary cutaneous anaplastic large cell lymphomas (n=5), primary cutaneous CD8 aggressive epidermotropic CTCLs (n=3), primary cutaneous CTCL, not otherwise specified (n=4), and extranodal nasal-type NK/T-cell lymphomas primarily affecting the skin or subcutaneous tissue (n=11). Sixteen cases of the newly named lymphomatoid papulosis type D (LyP-D; n=16) were also included. In those cases positive for TCR-γ, a further panel of 13 antibodies was used for analysis, including TIA-1, granzyme B, and perforin. Clinical and follow-up data were recorded in all cases. Twelve cases (8.2%) were positive for TCR-γ, including 5 PCGD-TCLs, 2 MFs, and 5 LyP-Ds. All 5 PCGD-TCL patients and 1 MF patient died of the disease, whereas the other MF patient and all those with LyP-D were alive. All cases expressed cytotoxic markers, were frequently CD3(+)/CD8(+), and tended to lose CD5 and CD7 expressions. Eight of 12 and 5 of 11 cases were CD30(+) and CD56(+), respectively. Interestingly, 5/12 TCR-γ-positive cases also expressed TCR-BF1. All cases analyzed were negative for Epstein-Barr virus-encoded RNA. In conclusion, TCR-γ expression seems to be rare and is confined to cytotoxic primary cutaneous TCLs. Nevertheless, its expression is not exclusive to PCGD-TCLs, as TCR-γ protein can be found in other CTCLs. Moreover, its expression does not seem to be associated with bad prognosis by itself, as it can be found in cases with good and bad outcomes.

Post-transplant lymphoproliferative disorder (PTLD) is a lymphoid proliferation that develops as a complication of solid organ or bone marrow transplants. PTLD limited to the skin is very rare. Plasmacytoma-like PTLD is an uncommon variant of monomorphic PTLD. Its presentation in the skin is extraordinary with very few cases reported in the literature. We report a new case of plasmacytoma-like PTLD presenting as multiple skin nodules on the leg of a 74-year-old kidney transplant recipient. Histopathologic and immunohistochemical examination of one nodule revealed atypical plasmacytoid and plasmablastic cells that showed kappa light chain restriction and stained positive for CD138. Staging investigations excluded extracutaneous manifestations of the disease. This case is unusual for several reasons including involvement limited to the skin, plasmacytoid phenotype of the tumor, presentation 18 years following transplantation and Epstein-Barr virus negativity.

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Attention has been focused on new ways to understand and manage urticaria ever since the recent addition of novel drugs to the therapeutic arsenal, the updating of clinical practice guidelines, and the publication of pathophysiologic insights. The Andalusian Section of the Spanish Academy of Dermatology and Venereology (AEDV) has developed a clinical pathway that defines quality-of-care characteristics and makes recommendations on decision-making affecting patients with urticaria. We present a patient-centered approach to care, in which the patient's clinical pathway through the health care system includes links between primary and hospital care to ensure continuity-a key feature of quality.