Search

Primary cutaneous follicular helper T (TFH)-cell lymphoma has recently been proposed, and is characterized by proliferation of malignant T cells expressing TFH-cell markers, such as CXCL13, accompanied by numerous reactive B cells. We report here a patient whose skin histology showed massive infiltration of both T and B cells, with a proliferation of arborizing high endothelial venules and follicular dendritic cells. Infiltrating T cells were positive for CXCL13, programmed death (PD)-1, inducible T-cell co-stimulator, and BCL-6. Southern blot analyses using DNA from the skin revealed monoclonality of both T and B cells. The patient had marked resistance to treatments, and complete remission was achieved only after allogeneic stem cell transplantation. The present case showed overlapping features with angio-immunoblastic T-cell lymphoma (AITL), although systemic symptoms were not observed. Further study is needed to define the criteria of this provisional entity, representing the cutaneous counterpart of the nodal follicular peripheral T-cell lymphoma or AITL.

Angiogenesis is a crucial process in the growth and progression of cancer, correlating with the metastatic potential of tumour cells. Angiopoietins are ligands for the endothelium-specific tyrosine kinase Tie2 receptor, which comprise 4 structurally related proteins, termed angiopoietin (Ang)-1, Ang-2, Ang-3 and Ang-4. The roles of Ang-1 and Ang-2 have recently been clarified as crucial in angiogenesis. In this report, we measured serum Ang-1 and Ang-2 levels in patients with cutaneous T-cell lymphoma (CTCL). Serum levels of Ang-2, but not Ang-1, in patients with Sézary syndrome were significantly higher than those in patch mycosis fungoides (MF), plaque/tumour MF, and healthy controls. In patients with CTCL, serum Ang-2 correlated with disease activity. Moreover, the numbers of Ang-2+ cells in lesional skin of CTCL were significantly larger than those in normal skin. These results suggest that Ang-2 may have important roles in the development of CTCL.

Many patients with cutaneous T-cell lymphoma (CTCL) experience severe pruritus. This study evaluated serum levels of nerve growth factor (NGF) and brain-derived neurotrophic factor (BDNF) in patients with CTCL. Although serum NGF and BDNF levels in patients with CTCL were not significantly higher than in healthy controls, serum NGF levels in patients with Sézary syndrome were higher than in those with mycosis fungoides and in healthy controls. Enhanced NGF expression by keratinocytes and increased dermal nerve fibres were detected in lesional skin of subjects with Sézary syndrome. Correlations between pruritus in CTCL and serum levels of NGF, BDNF, chemokine (C-C motif) ligand 1 (CCL1), CCL17, CCL26, CCL27, lactate dehydrogenase (LDH), IgE, and soluble interleukin-2 receptor were analysed. Serum CCL1, CCL26, LDH, and IgE levels correlated with pruritus in patients with CTCL. NGF may be associated with increased dermal nerve fibres and pruritus in Sézary syndrome, and CCL1, CCL26, and IgE may be associated with pruritus in CTCL.

CCR4, a member of the CC chemokine receptor family, is believed to play an important role in the pathogenesis of atopic dermatitis. To examine whether CCR4 single nucleotide polymorphism (SNP) is associated with susceptibility to atopic dermatitis, we investigated the allele and genotype frequencies of C1014T SNP of CCR4 in 198 Japanese patients with atopic dermatitis and controls by a PCR-restriction fragment length polymorphism method. There was no significant difference in allele or genotype frequencies between patients with atopic dermatitis and controls. Serum IgE levels and peripheral blood eosinophil counts were not significantly different among genotypes. There was also no significant difference in allele or genotype frequencies between the patient subgroup with and without asthma, with mild or moderate disease, with and without family history of atopic dermatitis, or with and without family history of atopic disorders. C1014T SNP of CCR4 does not appear to be associated with susceptibility to atopic dermatitis in Japanese patients.

© 2014 The Authors. doi: 10.2340/00015555-1675 Journal Compilation © 2014 Acta Dermato-Venereologica. ISSN 0001-5555 Patients with mycosis fungoides (MF) typically have a prolonged clinical course. A small proportion of cases progress over years through patch, plaque and tumour stages, followed by lymph node and visceral involvement (1). Some cases of MF are difficult to distinguish from other skin diseases, such as psoriasis and atopic dermatitis. Currently, tumour necrosis factor (TNF)-α inhibitors, such as infliximab, adalimumab, and etanercept, are widely used for treating patients with moderate to severe psoriasis (2). Blockade of TNF-α, however, is profoundly immuno suppressive, resulting in reactivation of tuberculosis and histoplasmosis, as well as the emergence of malignant lymphomas (3). In fact, some cases with MF manifested by treatment with TNF-α inhibitors have been reported (4–7). We describe here a 60-year-old woman diagnosed with psoriasis who showed exacerbation of MF with large cell transformation during treatment with infliximab.

The 2008 WHO–EORTC classification for cutaneous lymphomas identifies 3 main subtypes of primary cuta-neous B-cell lymphoma, including primary cutaneous dif-fuse large B-cell lymphoma, leg-type (PCLBCL, LT) (1), which is predominantly found in elderly people and has clinical features of erythematous or violaceous multiple tumours, frequent relapses, and a poor prognosis (1–3). Currently, CHOP (cyclophosphamide, doxorubicin, vin-cristine, prednisone) or rituximab plus CHOP (R-CHOP) chemotherapy is considered as a standard first-line therapy for PCLBCL, LT (1, 2, 4). Radiation therapy is less effective than in other subtypes of PCLBCLs. In case the condition of the patient does not allow application of chemotherapy, local irradiation of all visible skin lesions can be adopted. There is, however, no report or criteria for the selection of radiation treat -ment fields: involved field irradiation or extended field irradiation. This case report provides important insights for discussing frequent relapse and irradiation strategy in PCLBCL, LT.CASE REPORT

aggressive epidermotropic cy-totoxic T-cell lymphoma is rare, accounting for less than 1% of all cutaneous T-cell lymphoma (CTCL) (1). We report here a case of this type of lymphoma in a human T-cell leukaemia virus type-1 (HTLV-1) carrier. CASe rePorTA 58-year-old Japanese woman visited our hospital in 2006. She first noticed a red plaque on her left thigh approximately ten years previously. The number and the size of the plaques gradually increased despite treatment with topical steroid. At the initial visit, der-matological examination revealed irregularly shaped elevated erythematous plaques and ulcers with pus and crusts on her right breast (Fig. 1a). She also had multiple infiltrated red plaques with scales and crusts on her trunk and thigh (Fig. 1b). No superficial lymph nodes were swollen. Her general condition was good. She was from Kyushu, Japan, an area endemic for HTLV-1, and she was seropositive for the virus. The peripheral white cell count was normal without any atypical lymphocytes. Laboratory values were within normal limits except for a slightly increased level of se-rum soluble IL-2 receptor (965 U/ml, normal: 167–497 U/ml). Systemic examination revealed no visceral involvement. A skin biopsy specimen from the ulcer on the right chest showed diffuse infiltration of leukocytes in the dermis and subcuta-neous tissues. In the upper and middle part of the dermis, there was dense infiltrate of neutrophils, histiocytes and lymphocytes. Approximately half of infiltrating cells were CD3

© 2009 The Authors. doi: 10.2340/00015555-0665 Journal Compilation © 2009 Acta Dermato-Venereologica. ISSN 0001-5555 Sir, Folliculotropic mycosis fungoides (MF) is a rare variant of MF characterized by the presence of folliculotropic infiltrates and preferential involvement of the head and neck area. The prognosis of patients with folliculotropic MF is worse than that of patients with classical MF (1). Some patients with folliculotropic MF take an aggressive course with visceral involvement.

Sir, Dowling-Degos disease (DDD) is a rare disorder characterized by acquired pigmented macules and papules in a reticulate pattern, particularly affecting the flexural areas and other major skin folds (1, 2). A female patient presented with dotted and reticulate pigmentation of the axilla, and was diagnosed as DDD both clinically and histopathologically. However, the asymmetrical distribution of the eruption was distinct from classical DDD. In contrast to recent reports in Caucasian patients with DDD (3, 4), we found no evidence of mutation of exon 1 of the keratin 5 (KRT5) gene, suggesting an aetiological heterogeneity of this disorder.