CD30 ± cutaneous T-cell lymphoma associated with sarcoidosis*

We observed a 61-year-old woman, presenting with deeply infiltrating, erythemato-cyanotic, sometimes ulcerated or crusted plaques and nodules, mainly located on the lower limbs. Similar lesions had been present for more than 4 years, with a typical evolution: fast growth, followed by ulceration and necrosis, resulting in hyperpigmented, atrophic patches. The patient had had Rx-confirmed, symptomatic lung sarcoidosis for 5 years. Histological examination of skin lesions showed diffuse dermal and hypodermal lympho-histiocytic infiltrate, mainly composed of pleomorphic cells. On immunohistochemistry, the infiltrate was mainly composed of CD2 ±, CD3 ±, CD45RO ±, CD4 ±, CD30 ±, CD25 - T-cells, with sparse CDla ± dendritic cells. The typical clinical evolution (relapsing, self-regressing plaques and nodules), morpho-immuno-logical features of skin lesions, and strong expression of CD30 antigen by neoplastic T-cells suggest the possible classification of this case as CD30 ± (Ki-1 ±) cutaneous T-cell lymphoma. The association with systemic sarcoidosis, together with a down-regulated cell-mediated immune response, suggests the possibility that this latter may be a common denominator in the development of the two diseases.     

Copper-induced cutaneous sarcoidosis

A 52-year-old woman presented with gradual painless enlargement of both earlobes. Indurated plaques were also present elsewhere. There was no evidence of a systemic granulomatous disorder. Histopathology showed foreign material associated with granulomatous inflammation. Scanning electron microscopy and energy-dispersive analysis of X-rays demonstrated foreign material composed primarily of copper. Deposition is likely to have been related to corrosion of copper-containing earrings, or from deposition at the time of ear piercing. The presence of clinically similar granulomatous lesions remote from the earlobes and not containing copper suggest the presence of underlying cutaneous sarcoidosis. The earlobe lesions are more likely to be caused by foreign matter acting non-specifically as a nidus for cutaneous sarcoidosis, rather than by a specific pro-inflammatory effect of dermal copper. After 3 months, the lesions have partially responded to oral hydroxychloroquine 200 mg/day.     

Transepithelial elimination in cutaneous sarcoidosis

A patient known to have sarcoidosis exhibited cutaneous lesions. Microscopic examination of serial sections showed histologic evidence of transepithelial elimination of the sarcoidal granuloma. The concept and possible mechanisms of trans-epithelial elimination are discussed.     

皮肤结节病的治疗

结节病(sarcoidosis)是一种病因不明的多器官系统性疾病,其特征为由于淋巴细胞和单核巨噬细胞在受累组织器官中的聚集,造成正常组织结构紊乱,许多脏器产生非干酪性的上皮样细胞肉芽肿损害.发病可能与感染、化学物质刺激、药物、变态反应、自身免疫和遗传等有关.而免疫功能的改变是其基本表现,包括皮肤抗原的迟发型变态反应受损(无反应性),辅助性T细胞从外周血至病变活动部位的移动异常和高丙种球蛋白血症.     

Correlation between cutaneous sarcoidosis and systemic sarcoidosis.

Twenty patients with cutaneous sarcoidosis and 21 patients carrying isolated skin sarcoids were studied. (We use the term 'sarcoid' to emphasize that exclusively skin was altered.) Both groups were compared by clinical and histological patterns and certain data concerning the state of the mononuclear phagocyte system (MPS). It was found that skin lesions in sarcoids and sarcoidosis do not differ regarding either in clinical or histological manifestations. The changes in the functional activity of monocytes and macrophages were the same. The data obtained allow us to suggest that sarcoids should be regarded as a systemic disease connected with changes in the MPS reaction.     

Evidence-based therapy for cutaneous sarcoidosis

Most of treatments for cutaneous sarcoidosis are extensions of the drugs tested for pulmonary sarcoidosis. There are some exceptions. These include drugs given topically and those with high concentrations in the skin. This review will examine the conventional treatments of cutaneous sarcoidosis and focus on the best available evidence to make recommendations on treatment.     

T-cell receptor gene analysis in cutaneous T-cell lymphomas

An essential property of the immune system is its ability to generate diverse antibody and T-cell mediated responses to virtually any potential foreign particle, The basic molecular mechanisms responsible for producing this extensive diversity have now been elucidated. Each T cell expresses a unique membrane hound T-cell antigen receptor (TCR) which combines with specific antigenic peptides and major histocompatibility complex molecules. The characterization of TCR usage now represents a focal point for many studies of inflammatory and neoplastic disorders. Such studies are helping to clarify the pathogenesis of T-cell mediated diseases and provide the basis for the development of specific therapies. This paper will review several techniques used to identify neoplastic T-cell clones in cutaneous T-cell lymphoma. Similar methods may be used to analyse TCR gene usage in cutaneous inflammatory dermatoses.     

皮肤T细胞淋巴瘤表观遗传学研究进展

表观遗传学主要是研究在不改变DNA序列的情况下,发生的基因表达水平可遗传的变化,主要包括DNA甲基化、组蛋白修饰和非编码RNA调控.皮肤T细胞淋巴瘤是一组原发于皮肤的T淋巴细胞恶性增殖性疾病,蕈样肉芽肿和Sezary综合征是最常见的皮肤T细胞淋巴瘤.近年来研究表明,表观遗传学不仅在皮肤T细胞淋巴瘤的发生发展中起重要作用,而且可以通过改变表观遗传达到治疗皮肤T细胞淋巴瘤目的.     

Aneuploidy in cutaneous T-cell lymphoma

A new method is described which makes it possible using skin specimens to perform flow cytometric analysis of DNA content. DNA content analysis was performed on 28 skin specimens and 9 blood samples from 18 patients with mycosis fungoides and Sézary syndrome. The reproducibility was fair, with almost identical results in 6 cases (mycosis fungoides and Sézary syndrome) where two samples (skin specimens or blood samples) were taken 6 hours to 10 days apart. Hyperdiploidy was found in 7 of 11 skin specimens from patients with mycosis fungoides stage I with negative histology. In 13 skin specimens and 3 blood samples from patients with mycosis fungoides stages II and IV, abnormalities including hyperdiploidy, near-tetraploidy and near-hexaploidy were found in 8 of 13 skin specimens and in 2 of 3 blood samples. Four patients with Sézary syndrome were studied: 2 patients in remission showed normal DNA histograms (2 skin specimens, 3 blood samples) and 2 patients with active disease showed aneuploidy in the 2 skin specimens examined and in 1 of 3 blood samples. These studies demonstrate: 1) the importance of flow cytometry as a diagnostic tool for use on skin specimens in the early stages of mycosis fungoides where routine histology is non-diagnostic; 2) the diagnostic and prognostic aid of flow cytometry during the course of mycosis fungoides and Sézary syndrome in addition to the probability of measuring the effect of treatment.