原发性皮肤弥漫性大B细胞淋巴瘤一例

患者,男,51岁。左小腿红斑、结节、斑块伴疼痛3个月。组织病理及免疫组化确诊为原发性皮肤弥漫性大B细胞淋巴瘤,腿型。     

原发性皮肤弥漫大B细胞淋巴瘤,腿型

报告1例原发性皮肤弥漫性大B细胞淋巴瘤（腿型）。患者女,82岁。左小腿出现3个红色结节2个月,组织病理检查发现真皮内淋巴样细胞浸润,无嗜表皮现象,细胞体积大,肿瘤细胞CD20（＋）,CD79α（＋）,Bcl-2（＋）,Bcl-6（＋）,Ki-67 70％（＋）,MUM-1（＋）,Pax-5（＋）,CD10（-）,诊断为原发性皮肤弥漫性大B细胞淋巴瘤（腿型）,全身检查未发现皮肤以外系统受累证据,行局部肿瘤切除及口服糖皮质激素治疗。     

Primary cutaneous marginal zone B-cell lymphoma: a clinical,histopathological, immunophenotypic and molecular genetic study of 22 cases

BACKGROUND: Primary cutaneous marginal zone B-cell lymphoma (MZCL) has recently been described. Differentiation from follicular centre cell lymphomas and lymphocytomas is often difficult due to insufficient experience and a lack of large series of patients. OBJECTIVES: To characterize primary cutaneous MZCL better, we report clinical, histopathological, immunophenotypic and molecular genetics features in a series of 22 patients. METHODS: All patients were treated and followed up at the same institution. Diagnosis of MZCL was based on the World Health Organization classification criteria. All samples were routinely tested with a wide panel of monoclonal antibodies. DNA was extracted from every sample following standard methods. IgH rearrangement and t(14;18)(q32;q21) studies were performed in all samples. RESULTS: Twenty-two patients (20 men, two women; mean age 50 years, range 24-77) were included. The mean follow-up was 43 months. Seventy per cent of patients presented with characteristic skin lesions on the trunk or extremities, consisting of deep red to violaceous infiltrated plaques, nodules or tumours frequently surrounded by diffuse or annular erythema. Four patients presented with lesions on the head and neck area. Two patients had disseminated skin lesions. The main histopathological features were non-epidermotropic, dense lymphocytic infiltrates mainly distributed in a nodular pattern. Adnexal involvement was usually present, with eventual formation of lymphoepithelial complexes. Cytologically, the infiltrate was polymorphous with marginal zone B cells and B-monocytoid cells. Blastoid CD30+ cells were often observed. Colonized reactive germinal centres and lymphoplasmocytoid differentiation were frequently present. Neoplastic cells were CD20+, CD79a+, CD5- and CD10-. Monotypic expression of light chains was observed in 18 cases (13 kappa; five lambda). Clonal IgH rearrangements were detected in 14 cases. The bcl-2 mutation t(14;18)(q32;q21) was demonstrated in two cases. Most patients were treated with local radiotherapy. Complete response rate with this approach was 100%. Six patients (27%) had skin recurrences from 6 months to 8 years after first treatment. Five patients (23%) had extracutaneous involvement. Two of them had a large cell transformation and one died of lymphoma. Three of four patients with head and neck presentation developed extracutaneous disease. CONCLUSIONS: MZCL appears to be a well recognizable entity, clinically, histologically and immunophenotypically. Although prognosis is generally good, the disease has potential for skin as well as extracutaneous recurrences. Large cell transformation and head and neck presentation may be associated with a worse prognosis.     

原发性皮肤边缘区B细胞淋巴瘤

患者男,30岁.背部散在红色结节3年余.皮损组织病理检查:真皮内淋巴细胞样细胞呈片状岛屿状浸润增生,部分细胞可见异形性,表真皮间可见无浸润带,表皮大致正常.免疫组化结果:中心细胞样小淋巴细胞Bcl-2、CD20、CD79a、mum-1(++),Kap-pa:Lambda＞10;CD5、CD10、Bc1-6(-);结节周...     

大疱性类天疱疮并发原发性皮肤弥漫性大B细胞淋巴瘤

报告1例大疱性类天疱疮并发原发性皮肤弥漫性大B细胞淋巴瘤.患者男,80岁.全身水肿性红斑伴水疱、大疱,诊断为大疱性类天疱疮.在发病7个月后,右胫前出现多个暗紫红色结节,逐渐增多、增大,并扩展至右股内侧和背部.皮损组织病理检查示真皮全层及皮下脂肪小叶弥漫致密的淋巴细胞浸润,可见多数淋巴细胞体积增大,形态不规则,核大深染,可见核分裂相.免疫组化染色:CD20( ),bcl-2( ),bcl-6( ).诊断:原发性皮肤弥漫性大B细胞淋巴瘤.在肿瘤出现后1个月,患者大疱性类天疱疮的病情出现反复,通过CHOP方案治疗,病情明显好转.     

What is actinic prurigo in Britain?

Actinic prurigo (AP) belongs to the group of idiopathic photodermatoses sharing a predilection for occurring more commonly in females, and there is much controversy as to whether it is only a more severe form of polymorphous light eruption (PMLE) or whether it is a distinct entity in its own right. The condition is characterised by intensely itchy papules, plaques and nodules, along with excoriations and scars usually starting before puberty, and predominantly involves the sun-exposed areas although it may also affect covered sites. Seasonal exacerbations at the beginning of spring with improvement in the fall are typical, although the lesions frequently do not clear completely in the winter. The disorder may run a chronic course and persist into adulthood, but often spontaneous resolution occurs in late adolescence. Diagnosis is predominantly based on the clinical features, cutaneous irradiation tests and histology often being normal or non-specific. HLA typing has also been performed in both PMLE and AP patients, showing a strong association between HLA-DR4, in particular with the DRB1*0407 subtype, and AP; no HLA association has been found in PMLE. This HLA association is likely to be of pathogenic significance and strongly suggests a critical role for MHC-restricted antigen presentation in the development of photosensitivity AP.     

Intralesional rituximab for cutaneous B-cell lymphoma

Rituximab, a chimeric anti-CD20 monoclonal antibody, has been approved for systemic treatment of relapsed or refractory CD20-positive B-cell non-Hodgkin's lymphoma. As cutaneous B-cell lymphoma (CBCL) also expresses the CD20 molecule, three patients with histologically and immunohistochemically confirmed CBCL without systemic involvement were treated with low-dose intralesional rituximab in a pilot study. Single doses applied ranged from 10 to 30 mg per lesion, according to lesion extent, with a cumulative dose of up to 350 mg. Injections were given two or three times weekly for 3-5 weeks, with a second cycle after 6 weeks in one patient with incomplete remission. Complete and lasting remission was achieved in each patient; this has persisted for up to more than 1 year. The observed adverse events were of grade 1 severity. Results suggest that intralesional rituximab may be a safe and effective new therapy modality for CBCL.     

Treatment of primary cutaneous follicular centre lymphoma with rituximab: a report of two cases

Rituximab (anti-CD20 chimeric monoclonal antibody) has been demonstrated to have significant activity in nodal B-cell lymphomas, with very few associated adverse effects. Although primary cutaneous B-cell lymphomas (PCBCL) also express the CD20 antigen, relatively few reports of rituximab use in PCBCL have been published to date. We present two cases of primary cutaneous follicular centre lymphoma treated with rituximab (375 mg/m(2)/week intravenously for 4 weeks). Both cases responded to rituximab, with one partial response and one complete response seen. No adverse effects were observed. To date, disease progression has occurred in one case during overall follow up at 6 and 17 months. The potential role of rituximab in the treatment of these lymphomas is discussed.     

Diagnosis and treatment of actinic prurigo

Actinic prurigo (AP) is an idiopathic photodermatosis that affects mainly the mestizo population in Latin America. It has an early onset, a slight predominance in women, and affects the sun-exposed areas of the skin, causing erythematous papules and lichenified plaques secondary to intense and chronic pruritus. Lesions can be induced by both ultraviolet A (UVA) and ultraviolet B (UVB). An association with several human leukocyte antigen (HLA) alleles has been reported. AP is unique among all photodermatoses in its remarkable response to thalidomide. In the past the microscopic features of AP have been considered as nonspecific; however, the constant finding of dense lymphocytic inflammatory infiltrates and the immunogenetic features of AP support the existence of an immunologic mechanism in its pathogenesis.     

Adult-onset actinic prurigo in Thailand

BACKGROUND: Actinic prurigo (AP) is one of the rare idiopathic photodermatosis. It is said to be a familial disease and is usually seen in certain specific geographical areas. The adult-onset type of AP is reported less frequently in the Asian population and has never been reported in Thailand. METHODS: The study population comprised 30 patients. Demographic data were collected. Photo-tests and photo provocation tests for UVA, UVB and visible light were carried out on non-exposed skin. The other investigations included antinuclear antibody, anti-HIV antibody and urine porphyrin level. Histopathology studies were also carried out. RESULTS: There were 18 males and 12 females. The mean age of onset was 36.86 years. The duration of disease was from 1 month to 20 years. Forearms (27 patients) were the most frequently affected site. Other screening tests showed negative results. Five patients had abnormal MED to UVA and one patient had abnormal MED to UVA as well as UVB. Photo provocation tests showed positive responses to both UVA and UVB in 12 cases (40%), a positive response to UVA in 11 cases (37%), a positive response to UVB alone in four cases (13.3%) and a normal response in three patients (10%). None of the patients had a positive response to visible light. Skin biopsies were performed on nodular lesions in 23 cases. Histopathology from these 23 cases showed hyperkeratosis ortho- or parakeratosis and acanthosis in 20 of the 23 cases. CONCLUSIONS: Adult-onset AP in our country may have different geographic and racial distribution from previous reports or may be the tropical variant as described by Tham et al. It may not be an uncommon disease in our country, if there is increased awareness of this disease. Only 16.6% of patients had reduced MED. Photo provocation tests were positive in 90% of cases. Most of the positive wavelengths were UVA or both UVA and UVB. Therefore, photo provocation tests should be performed in cases suspected of AP. The prognosis for AP is not good, despite combinations of treatment. The disorder may run a chronic course. This may be because of our sunny climate and the sun-exposed occupations of patients.     

A case of actinic prurigo in Thailand

Actinic prurigo is a separate entity from the polymorphous light eruption that affects American Indians. It has been reported mainly from North and South America, with only few reported cases from Britain or Asia. We report a case of actinic prurigo in a Thai girl who showed cheilitis and pruritic papules on exposed areas for three years. We were able to induce populovesicular lesions by three consecutive irradiations with 100 J/cm2 UVA and 2 minimal erythematous dose of UVB. However, three weeks after irradiation, a prurigo papule developed at the UVB irradiated site.     

Primary cutaneous marginal zone B-cell lymphoma in a patient with chronic lymphocytic leukaemia

Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is a low-grade malignant lymphoma that presents in the skin with no evidence of extracutaneous localization at diagnosis. We present an 80-year-old woman with B-cell chronic lymphocytic leukaemia (CLL) who developed multifocal PCMZL lesions 14 months after CLL diagnosis. PCMZL was clonally similar to the original bone marrow (BM) CLL cells. The specific translocation t(14;18) (q32;q21) with breakpoints in IGH and BCL2 loci was found in a skin specimen, but was absent in BM and peripheral blood (PB) cells. In contrast, a 13q deletion was found in BM and PB CLL cells. The patient was treated with chlorambucil and complete response of PCMZL was achieved. To our knowledge this is the first patient with CLL in whom PCMZL has been diagnosed.     

Clearance of pediatric actinic prurigo with dupilumab

Actinic prurigo is a rare, idiopathic chronic photodermatosis of childhood characterized by excoriated papules, nodules, and plaques in sun-exposed areas. It is notoriously difficult to treat. The disorder involves a type IV hypersensitivity reaction driven by both Th1 and Th2 inflammatory pathways, the latter of which leads to secretion of IL-4, IL-5, IL-13, and production of B cells, IgE, and IgG4. Dupilumab, an IL-4 receptor antagonist, disrupts the Th2 pathway. We present a pediatric patient with severe, recalcitrant actinic prurigo who achieved rapid and sustained clearance with dupilumab.     

皮肤B细胞淋巴瘤

报告1例皮肤B细胞淋巴瘤。患者男,67岁。因右胫前暗红色斑块3个月、左胫前肿块2个月、牙龈肿胀20d就诊。双侧腹股沟可扪及淋巴结。皮损经组织病理学检查诊断为皮肤B细胞淋巴瘤。经全身化疗、局部放疗2个月后,患者病情好转。     

Case report of four patients with erythrodermic cutaneous T-cell lymphoma and severe photosensitivity mimicking chronic actinic dermatitis

The marked photosensitivity associated with chronic actinic dermatitis (CAD) is presumed to be due to a T cell-mediated response to ultraviolet (UV)-induced epidermal neoantigens. Photosensitivity is, however, a rare occurrence in cutaneous T-cell lymphoma (CTCL). We discuss a series of four patients with erythrodermic CTCL who exhibited marked photosensitivity mimicking CAD. Significantly, the tumour cells had a CD8 phenotype in half of these patients. All patients had T-cell clones in skin and also demonstrated identical peripheral T-cell clones in blood or lymph node involvement. Sézary cell counts ranged from 6% to 20%, CD4/CD8 ratios from 0·22 to 23·5. Clinical presentation was striking for a marked photosensitive distribution. Monochromator irradiation testing revealed reduced minimal erythema doses throughout UVB and UVA ranges, findings consistent with those seen in CAD. All patients subsequently died from systemic disease. These findings suggest that, rarely, malignant clonal T-cell populations may recognize a unique UV-induced neoantigen, resulting in the clinical features of severe photosensitivity mimicking those seen in CAD.