Kikuchi's disease (histiocytic necrotizing lymphadenitis) with cutaneous involvement

We report a case of Kikuchi's disease (histiocytic necrotizing lymphadenitis) in a 22-year-old Japanese man who presented with cervical and axillary lymphadenopathy and who developed edema of lips with desquamation and erosions and transient indurated erythematous papules on the face, neck and upper extremities. A lymph node biopsy specimen showed the typical necrotizing lymphadenitis of Kikuchi's disease. A skin biopsy specimen showed a dense, lymphohistiocytic, superficial, deep perivascular, and interstitial infiltrate, papillary dermal edema, and abundant nuclear debris with a conspicuous absence of neutrophils, paralleling the nodal histology of Kikuchi's disease. CD68 immunohistochemistry showed many plasmacytoid monocytes. Oral corticosteroid (prednisolone 40 mg) therapy improved the skin lesions and other clinical symptoms. Lip involvement by Kikuchi's disease has not been reported, and should be included as one of the possible cutaneous manifestations of Kikuchi's disease.     

误诊为系统性红斑狼疮的皮肤型Rosai-Dorfman-Destombes病一例

患者,女,60岁。面部对称性红斑1年,加重伴丘疹、斑块形成6个月。皮肤组织病理检查示：皮肤真皮深层及皮下组织弥漫性淋巴浆细胞浸润,组织细胞增生可见“伸入运动”,免疫组化S-100、CD68阳性,CD1a阴性,给予泼尼松、甲氨蝶呤、沙利度胺治疗2个月后好转。     

Histiocytic necrotizing lymphadenitis (Kikuchi's disease): the necrotic appearance of the lymph node cells is caused by apoptosis.

We report a case of histiocytic necrotizing lymphadenitis in a 28-year-old woman. The biopsy specimen of the enlarged lymph node showed lymphocytes, histiocytes, and a large amount of nuclear debris as well as marked eosinophilic deposits. We found DNA fragments by means of the modified TUNEL method, especially in the transitional area between intact cells and the foci of eosinophilic deposits and the cells positive for anti-Fas antibody in the biopsied lymph node. Therefore, the necrotic appearance of the lymph node was thought to be caused by apoptosis induced by the Fas-Fas ligand system. We hypothesize that the apoptosis was strongly related to the pathogenesis of this disease.     

A Case of Kikuchi's Disease Presenting with Unique Facial Involvement

Kikuchi''s disease (KD), or histiocytic necrotizing lymphadenitis, is a rare, self-limited lymphadenopathy, typically in young women, that usually remits spontaneously and does not recur. KD is clinically characterized by cervical lymphadenopathy and a high fever. Extranodal involvement (skin, arthritis, meningitis) rarely occurs. When KD is involved in a skin lesion, it presents with various shapes, but rarely manifests with symmetrically distributed, erythematous, firm nodules only on the face. In this report, we describe a patient with KD and unique skin manifestations.     

Undifferentiated connective tissue disease and its cutaneous manifestations

Undifferentiated connective tissue disease (UCTD, also named UCT syndrome, latent lupus or incomplete lupus) is regarded as an autoimmune disorder in which signs and symptoms are widely variable and evocative for connectivitis but not sufficiently evolved to fulfil any of the accepted classification criteria for the defined connective tissue diseases. In this paper we describe the case of a 47-year-old woman affected by UCTD according to the preliminary classification criteria supplied by Mosca et al. in 1999.     

Cutaneous lupus erythematosus and its association with systemic lupus erythematosus: A nationwide population-based cohort study in Korea

Although lupus erythematosus is known to be more common among women of color, the study populations in previous reports were predominantly Caucasian and there is scarce information on Asian patients. Therefore, we performed a retrospective study using a nationwide population-based cohort in South Korea. The average annual incidence of cutaneous lupus was 4.36/100 000. Among 634 patients with cutaneous lupus, 20.8% had systemic disease: cutaneous lupus was diagnosed before systemic lupus in 4.26% and after systemic lupus in 8.52%. More female patients than male patients developed systemic lupus erythematosus. The average time to progression to systemic lupus was 1.53 ± 1.46 years.     

Behçet's Disease as a Part of the Symptom Complex of SLE?

Systemic lupus erythematosus and Behçet's disease are known to be induced by immunoregulatory defects and to have some identical manifestations. Although the possibility of the coexistence of these disorders exists only one example of an association of these disorders has been described previously by Lee et al. Therefore, we describe an example of typical SLE with additional symptoms of Behçet's disease as a part of the symptom complex of SLE.     

Upregulation of epidermal surface molecule expression in primary and ultraviolet-induced lesions of lupus erythematosus tumidus

BACKGROUND: Lupus erythematosus tumidus (LET), a photosensitive skin disorder with characteristic clinical and histological features, has not been generally accepted as a subset of cutaneous lupus erythematosus (CLE). OBJECTIVES: To analyse the expression of epidermal surface molecules in skin biopsy specimens from patients with LET and to relate the results to other variants of CLE, such as discoid lupus erythematosus (DLE) and subacute CLE (SCLE). METHODS: In total, 45 patients with different subtypes of CLE were included in the study, and cryostat sections from primary and ultraviolet (UV) A- and UVB-induced skin lesions were investigated using immunohistochemical methods. RESULTS: In contrast to healthy controls, skin lesions of LET showed upregulation of intercellular adhesion molecule-1 (ICAM-1) and histocompatibility class II molecules (HLA-DR), with an expression pattern resembling that seen in DLE and SCLE. Furthermore, staining with a monoclonal antibody against 27E10, a distinct marker for cell activation and differentiation, revealed intense focal or band-like labelling of all epidermal layers independent of the type of lesion. CONCLUSIONS: Expression of epidermal surface molecules such as ICAM-1, HLA-DR and 27E10 is equally upregulated in primary and UV-induced lesions of patients with LET, DLE and SCLE. These results support our recent clinical findings that LET represents a distinct subset of CLE with a similar immunopathomechanism rather than a different disease.     

Kikuchi's disease and the skin: case report and review of the literature

We report scattered indurated erythematous lesions that presented in an 18-year-old Japanese man with Kikuchi's disease (KD; histiocytic necrotizing lymphadenitis). A skin biopsy showed a proliferation of histiocytes and abundant nuclear debris without the presence of neutrophils, which is characteristic of KD. The specific dermatological and pathological details of KD have been yet to be fully described. In order to assess the typical skin features of KD better, we have reviewed all the previously well-documented reports of such lesions. As the clinical and histopathological cutaneous findings in KD are so heterogeneous, it is important that scattered indurated erythematous lesions should be included as one of the possible cutaneous manifestations of this disease.     

Lupus erythematosus-like lesions in a carrier of X-linked chronic granulomatous disease.

Chronic granulomatous disease (CGD) is an inherited immunodeficiency disease. Carrier status of CGD has been reported in association with lupus erythematosus-type lesions. A 35-year-old woman, mother of a child with X-linked CGD presented an 8-year history of erythematous plaques with an arciform pattern on the upper trunk, back and arms. The nitroblue tetrazolium test revealed the carrier status of the patient. Haematological, biochemical and immunological tests (including ANA, DNA, SSA-Ro, SSB-La, RNP, SM and Jo1 antibodies) were normal or negative except for a polyclonal hypergammaglobulinaemia with high serum IgA. Histological examination showed a papillary and perifollicular lymphohistiocytic infiltrate. Direct immunofluorescence was negative. We report a female carrier of X-linked CGD who developed clinical subacute lupus erythematosus-like lesions. We review the literature and discuss the pathogenetic mechanisms involved in the condition.     

来氟米特治疗难治性结缔组织病15例疗效观察

目的：观察来氟米特治疗难治性结缔组织病的疗效。方法：应用小剂量来氟米特(20mg/d)开放性治疗15例难治性结缔组织病患者，包括系统性红斑狼疮(10例)、皮肌炎(4例)、白塞病(1例)。结果：对于常规治疗无效的顽同难治性患者，应用小剂量来氟米特疗效显著，能控制病情，减少糖皮质激素用量。结论：来氟米特为难治性结缔组织病提供了一种较好的治疗方案。     

系统性红斑狼疮408例临床和免疫学回顾性分析

目的：分析408例系统性红斑狼疮（SLE）患者的临床和免疫学指标特点。方法：建立2004--2007年南京医科大学第一附属医院408例SLE住院患者临床资料数据库,并且用SPSS13．0软件对数据进行分析。结果：408例SLE患者脏器损害中肾脏损害最常见,达82．1％,免疫学指标中抗核抗体（ANA）阳性最常见,达91．9％。而≥50岁患者的肺部受累或视觉障碍发生明显多于〈50岁组患者,蝶形红斑、盘状红斑、发热、ds—DNA抗体阳性率以及疾病活动指数≥9的发生率明显低于〈50岁组患者。男性组患者肾损害和低蛋白血症发生率明显高于女性组患者,而光敏感的发生率则明显低于女性组患者。结论：SLE是多系统、多脏器受累的疾病,在临床症状和免疫学指标上有其特征性改变,因而重视早期诊断,对延缓SLE患者病情发展,改善预后具有重要意义。     

Comparative analysis of subacute cutaneous lupus erythematosus and chronic cutaneous lupus erythematosus: clinical and immunological study of 270 patients

Background Lupus erythematosus (LE) is a chronic, autoimmune disease resulting from an interaction of genetic, environmental and hormonal factors and characterized by a spectrum of clinical forms with variable evolution from a localized cutaneous form to a life‐threatening systemic form. Objective To analyse and compare the prevalence and characteristics of the main clinical and immunological manifestations of subacute cutaneous LE (SCLE) and chronic CLE (CCLE). Methods A total of 270 patients with CLE (112 patients with SCLE and 158 patients with CCLE) were studied retrospectively. The clinical and serological characteristics of all the patients were collected in a chart review. Results The patients with SCLE had a higher prevalence of annular and papulosquamous lesions, Raynaud phenomenon, mucous membrane ulcers, malar rashes, photosensitivity, vasculitis and a lower frequency of discoid lesions and alopecia compared with patients with CCLE. Patients with SCLE had a higher prevalence of arthralgias (P < 0·001), xerophthalmia (P = 0·045), arthritis (P < 0·001), nephropathy (P = 0·003) and systemic LE (SLE) (P < 0·001) compared with patients with CCLE. Patients with SCLE also had a higher frequency of laboratory and serological abnormalities than patients with CCLE. Generalized discoid LE (DLE) was associated with a higher prevalence of photosensitivity (P < 0·001), panniculitis (P = 0·009) and SLE (P = 0·003) than localized DLE. In patients with SCLE and those with CCLE, photosensitivity, arthralgias, arthritis, nephropathy and xerophthalmia were associated with SLE. In patients with SCLE, significant correlations existed between clinical and immunological data. Conclusions In our series, differences in the expression of CCLE and SCLE were found with respect to the distribution and type of lesions, systemic features and immunological findings.     

Cutaneous lupus erythematosus: a review of etiopathogenic,clinical, diagnostic and therapeutic aspects

Cutaneous lupus erythematosus is an autoimmune disease of varied clinical expression, which may present as an exclusively cutaneous disease or be one of the multiple manifestations of systemic lupus erythematosus. Its classification includes acute, subacute, intermittent, chronic and bullous subtypes, which are usually identified based on clinical features and histopathological and laboratory findings. Other non-specific cutaneous manifestations may be associated with systemic lupus erythematosus and are usually related to disease activity. Environmental, genetic and immunological factors play a role in the pathogenesis of skin lesions in lupus erythematosus. Recently, considerable progress has been made in elucidating the mechanisms involved in their development, which allows for foreseeing future targets for more effective treatments. This review proposes to discuss the main etiopathogenic, clinical, diagnostic and therapeutic aspects of cutaneous lupus erythematosus, aiming to update internists and specialists from different areas.     

Elevated serum MFG‐E8 level is possibly associated with the presence of high‐intensity cerebral lesions on magnetic resonance imaging in patients with systemic lupus erythematosus

Human milk fat globule‐EGF factor 8 (MFG‐E8), also known as lactadherin, is a secreted glycoprotein that plays essential roles in the clearance of apoptotic cells and angiogenesis. It has been reported that serum MFG‐E8 levels are higher in systemic lupus erythematosus (SLE) patients compared with in healthy controls; however, a previous study reported no correlation between serum MFG‐E8 levels and SLE disease activity. The objective of this study was to assess serum MFG‐E8 levels and their clinical associations in patients with SLE. Serum MFG‐E8 levels in 49 Japanese patients with SLE, eight with cutaneous LE, and 28 healthy controls were examined. Serum MFG‐E8 levels in SLE patients were significantly higher than those in cutaneous LE patients and healthy individuals. In addition, serum MFG‐E8 levels were positively correlated with the SLE Disease Activity Index score, which reflects the disease activity of SLE. Notably, the frequency of the presence of high‐intensity cerebral lesions on MRI in the SLE patients with elevated serum MFG‐E8 levels was significantly higher than that in SLE patients with normal serum MFG‐E8 levels. These findings suggest that elevated serum MFG‐E8 levels may be associated with cerebrovascular diseases or neuropsychiatric SLE in patients with SLE, and that the measurement of serum MFG‐E8 levels in SLE patients is useful for risk stratification of cerebrovascular disease or cerebrovascular disease‐related neuropsychiatric SLE.     

Cutaneous CD30 (Ki-1)-positive anaplastic large cell lymphoma preceded by Hodgkin's disease

A 38-year-old female was diagnosed as Hodgkin's disease of the axillar lymph nodes, nodular sclerosis type, as evidenced by the presence of Reed-Sternberg cells positive for CD30 and CD15 and negative for CD3, CD20, and CD45. She achieved complete remission after combination chemotherapy. Two years later, she noticed a red papule on her public area without any lymph node involvement. The biopsy specimens showed diffuse proliferation of large-sized atypical lymphoid cells positive for CD30 and CD45, and negative for CD3, CD20 and CD15. These findings were mostly compatible with CD30 (Ki-1)-positive anaplastic large cell lymphoma (Ki-1 lymphoma). Our case is considered to be cutaneous Ki-1 lymphoma preceded by Hodgkin's disease.     

Cutaneous Crohn's disease with superimposed psoriasis: A unique case with overlapping histology

Crohn's disease (CD) is an idiopathic, chronic inflammatory disorder of the gastrointestinal tract. We recently encountered a unique case in which a patient with longstanding CD presented with skin lesions with histopathologic features of both psoriasis and granulomatous inflammation suggestive of cutaneous CD. To our knowledge, this has not been described concomitantly in the same patient, in the same lesions. Review of the literature suggests that the intersection of these 2 histopathological reaction patterns may not be pure coincidence. Clinical‐pathologic correlation of this case will be discussed, along with a review of the potential mechanisms of this unique disease presentation.     

Successful experience of rituximab therapy for systemic sclerosis‐associated interstitial lung disease with concomitant systemic lupus erythematosus

Previous studies have demonstrated that B cells play critical roles in autoimmune disorders including systemic sclerosis (SSc) and systemic lupus erythematosus (SLE). However, the effectiveness of rituximab (RTX), a chimeric anti‐CD20 antibody, for SSc‐associated interstitial lung disease (ILD) or SLE disease activity remains controversial. We herein report an SSc patient with severely progressed ILD and concomitant SLE treated by two cycles of RTX at baseline and half a year later. This treatment improved ILD and SLE activities, along with reduction of dermal sclerosis and serum anti‐topoisomerase I antibody levels. In addition, our detailed time‐course data indicate that half a year may be appropriate as an interval between each cycle of RTX therapy aimed at SSc‐associated ILD or SLE. Overall, the current report could pave the way to establish RTX as a disease‐modifying drug for patients with SSc and/or SLE showing resistance to other already approved medications.     

亚急性皮肤型红斑狼疮合并甲亢1例

患者女,35岁。全身红色皮疹伴发热2个月,上半身皮疹加重20d。查体:面颈部及双上肢环状红斑,双手震颤,突眼,甲状腺肿大;ANA1∶160,SSA3(+);T4,FT4↑,TSH↓,TG-Ab,TPO-Ab↑;甲状腺彩超示:甲状腺弥漫性肿大,血流丰富。皮损组织病理示:表皮变薄,基底液化变性,真皮浅层血管及附属器周围局灶性淋巴细胞为主的炎细胞浸润,可见个别噬黑素细胞,胶原轻度嗜碱变性。诊断:SCLE合并甲亢。SCLE合并甲亢尚未见报道,可能与两者有相同的遗传及免疫学基础有关。