Primary cutaneous CD30+ anaplastic large cell lymphoma treated with radiotherapy and methotrexate with development of xanthomas at the sites of prior disease.

Primary cutaneous anaplastic large cell lymphoma is a rare type of cutaneous T-cell lymphoma, and the involvement of the ocular adnexa is extremely rare. Secondary xanthoma-like changes after radiation therapy or chemotherapy have been rarely reported in association with large-cell T-cell anaplastic lymphoma. We report one case of a primary C-anaplastic large cell lymphoma affecting the eyelid with fast progression with multiple nodules in various anatomic sites and development of xanthoma-like lesions after treatment.

Specific skin infiltration as first sign of localized stage Hodgkin's lymphoma involving an epitrochlear node.

Cutaneous manifestation as the first sign of Hodgkin lymphoma (HL) is very rare and diagnostically challenging; especially, because the clinical presentation of specific skin involvement by HL is polymorphous. We present a 44-year-old man with erythematous indurate papules and plaques in the right forearm and arm where skin biopsy showed an HL. He also has an enlarged epitrochlear node, and later histopathologic study confirmed the diagnosis of HL subtype-mixed cellularity. Immunohistochemical stains in both biopsies showed that the atypical cells were positive for CD30 and CD15, and negative for CD20 and CD3. PAX5 stained the nuclei of the atypical large lymphoid cells weakly and Oct-2 staining was negative in the atypical cells. EBER and LMP1 protein were negative in both biopsies. Epitrochlear involvement in HL, like in our case, is a rare event (<1%). We reviewed data about prognosis, clinical appearance, and treatment of all the cases of HL specific skin involvement published after Sioutos et al, emphasizing the cases where HL specific skin involvement was the first sign of the disease as in our patient.

Psoriasis in humans is associated with down-regulation of galectins in dendritic cells.

We have investigated the expression and role of galectin-1 and other galectins in psoriasis and in the Th1/Th17 effector and dendritic cell responses associated with this chronic inflammatory skin condition. To determine differences between psoriasis patients and healthy donors, expression of galectins was analysed by RT-PCR in skin samples and on epidermal and peripheral blood dendritic cells by immunofluorescence and flow cytometry. In the skin of healthy donors, galectin-1, -3 and -9 were expressed in a high proportion of Langerhans cells. Also, galectins were differentially expressed in peripheral blood dendritic cell subsets; galectin-1 and galectin-9 were highly expressed in peripheral myeloid dendritic cells compared with plasmacytoid dendritic cells. We found that non-lesional as well as lesional skin samples from psoriasis patients had low levels of galectin-1 at the mRNA and protein levels, in parallel with low levels of IL-10 mRNA compared with skin from healthy patients. However, only lesional skin samples expressed high levels of Th1/Th17 cytokines. The analysis of galectin-1 expression showed that this protein was down-regulated in Langerhans cells and dermal dendritic cells as well as in peripheral blood CD11c(+) DCs from psoriasis patients. Expression of galectin-1 correlated with IL-17 and IL-10 expression and with the psoriasis area and index activity. Addition of galectin-1 to co-cultures of human monocyte-derived dendritic cells with autologous T lymphocytes from psoriasis patients attenuated the Th1 response. Conversely, blockade of galectin binding increased IFNγ production and inhibited IL-10 secretion in co-cultures of monocyte-derived dendritic cells with CD4(+) T cells. Our results suggest a model in which galectin-1 down-regulation contributes to the exacerbation of the Th1/Th17 effector response in psoriasis patients.

Empyema necessitatis revisited.

Empyema necessitatis (EN) is a rare disease, with unknown incidence, which has received little attention from a dermatological point of view but it is essential to recognize it because of the possibility of causing mortality if not treated properly and in time. We report a 32-year-old woman, diagnosed with nervous anorexia, with an enlarging mass on the anterior right thoracic wall. Cultures showed Actinomyces gerencseriae as the main etiological agent of her empyema "necessitatis". She was successfully treated with amoxicillin with clavulanic acid. We found that 1) M. tuberculosis (35%) is still the most frequent agent, but Actinomyces (25%) and MRSA (10%) are becoming more relevant; 2) the most frequent dermatological finding is a subacute erythematous mass on costal wall; 3) new treatments have lowered mortality. Any enlarging and painful subacute thoracic mass with fluctuation should be considered as an EN suspicious lesion and the diagnostic approach must include a chest X-ray to rule out lung infection. Dermatologists should know about this infrequent entity in order to properly identify the potentially life-threatening process under these cutaneous lesions, achieving an early diagnosis and proper treatment.

Identification of genes responsive to solar simulated UV radiation in human monocyte-derived dendritic cells.

Ultraviolet (UV) irradiation has profound effects on the skin and the systemic immune system. Several effects of UV radiation on Dendritic cells (DCs) functions have been described. However, gene expression changes induced by UV radiation in DCs have not been addressed before. In this report, we irradiated human monocyte-derived DCs with solar-simulated UVA/UVB and analyzed regulated genes on human whole genome arrays. Results were validated by RT-PCR and further analyzed by Gene Set Enrichment Analysis (GSEA). Solar-simulated UV radiation up-regulated expression of genes involved in cellular stress and inflammation, and down-regulated genes involved in chemotaxis, vesicular transport and RNA processing. Twenty four genes were selected for comparison by RT-PCR with similarly treated human primary keratinocytes and human melanocytes. Several genes involved in the regulation of the immune response were differentially regulated in UVA/UVB irradiated human monocyte-derived DCs, such as protein tyrosine phosphatase, receptor type E (PTPRE), thrombospondin-1 (THBS1), inducible costimulator ligand (ICOSL), galectins, Src-like adapter protein (SLA), IL-10 and CCR7. These results indicate that UV-exposure triggers the regulation of a complex gene repertoire involved in human-DC-mediated immune responses.

Late skin reaction to iodixanol (Visipaque): clinical manifestations, patch test study, and histopathological evaluation.

Late reactions to iodinated contrast media are frequent. Cutaneous manifestations are the commonest, in which maculopapular exanthema, a type of cutaneous presentation, is widespread. Controversy exists about the utility of the skin test in the management of these reactions. The aim of this study is to analyse the clinical characteristics, the histopathological findings, and the results of the patch test in patients who developed a late skin reaction (LSR) to the nonionic, dimeric, iodinated contrast media Visipaque. We retrospectively reviewed the patients with LSR to Visipaque, seen in the Dermatology Department between 1999 and 2005. A total of 12 patients participated in this study (7 men and 5 women), ages ranging from 39 to 76 years (mean 56). 11 of the patients had significant medical history. All the patients developed a maculopapular exanthema between 2 hr and 3 days after the radiological examination, involving the trunk and proximal limbs, although some of the patients showed involvement of distal areas. The skin biopsy, performed in 6 patients, showed nonspecific findings consistent with drug reaction. In 3 patients, patch tests to Visipaque and iodixanol were positive. The most frequent manifestation of LSR to iodixanol is a maculopapular exanthema, involving the trunk and the limbs, although distal involvement can be seen. Histopathological findings are nonspecific and cannot be distinguished from other drug reaction. Patch tests have a limited value, and in cases where they were negative, reintroduction of the drug triggered a new LSR.

Rosetoid schwannoma (neuroblastoma-like) in association with an anetoderma.

BACKGROUND: We report an additional case of an extremely uncommon but distinctive histological variant of benign schwannoma, which was previously designated as neuroblastoma-like schwannoma by Goldblum et al. METHODS: A 29-year-old woman referred to a 6-year-history of an atrofic macule. Its clinical appearance was similar to that of an anetoderma. RESULTS: A cutaneous biopsy showed findings consistent with a neuroblastoma-like schwannoma with the following peculiar features: (i) Being fully composed of rosette-like structures. (ii) Association to an anetoderma. CONCLUSIONS: Because neither the histological pattern nor the type of tumor allows a differential diagnosis with neuroblastoma, we propose the descriptive term of rosetoid schwannoma. And to our knowledge, this will be the first case reported of rosetoid schwannoma associated to anetoderma.