丘疹鳞屑型亚急性皮肤型红斑狼疮

患者女,58岁。主诉：面颈部、上肢、肩背部鳞屑性斑丘疹2年。现病史：患者2年前无明显诱因面颈部出现散在三粒红色丘疹,逐渐扩大成大小不等形状不规则斑丘疹,上覆有细薄鳞屑,随后渐累及上肢、肩背部,伴有轻度瘙痒,偶日晒后加重。曾就诊于当地多家医院,拟“银屑病”、“多形性日光疹”予治疗（具体用药不详）,无明显好转。起病以来无脱发、手足发冷、关节酸痛等不适。     

亚急性皮肤红斑狼疮1例

患者女,41岁。颜面红斑伴光敏感16月。实验室检查:ANA阳性、抗SS-A(Ro)、抗SS-B(La)均阳性;组织病理示:表皮萎缩变薄,基底细胞点状液化变性,真皮浅层及附属器周围淋巴细胞浸润。诊断:亚急性皮肤红斑狼疮(环状红斑型)。     

Subacute Cutaneous Lupus Erythematosus Induced by Nifedipine

Background: Subacute cutaneous lupus erythematosus (SCLE) has been reported to be associated with the use of several drugs, including thiazides, terbinafine, and, rarely, calcium channel blockers. Objective: A case of SCLE induced by nifedipine is presented. Methods and Results: A 48-year-old white woman developed a papulosquamous and annular eruption in sun-exposed areas during the summer. The patient was taking nifedipine for essential hypertension for four years. Serology showed the presence of antinuclear and anti-Ro/SSA as well as antihistone antibodies. Histopathologic and immunopathologic (granular IgM deposits at the dermoepidermal junction) findings confirmed the diagnosis of SCLE. Nifedipine discontinuation led to rapid improvement with almost complete resolution of skin lesions in one month in the absence of active treatment. Reduction of antinuclear, anti-Ro/SSA, and antihistone antibody levels was documented after six months. Conclusion: Nifedipine can cause SCLE after a long period of administration. Antihistone antibodies may be associated with drug-induced SCLE.

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Antécédents: Des rapports font état de lapparition du lupus érythémateux cutané subaigu (LECS) parallèlement à la prise de certains médicaments, notamment le thiazidique, la terbinafine et, rarement, les inhibiteurs calciques. Objectifs: Présenter un cas de LECS provoqué par la nifédipine. Méthodes et résultats: Une femme de race blanche de 48 ans a développé une éruption papulo-squameuse et annulaire dans des régions exposées au soleil durant lété. La patiente prenait de la nifédipine contre lhypertension artérielle essentielle depuis quatre ans. Les épreuves sérologiques ont révélé la présence danticorps antinucléaires, anti-Ro/SSA et anti-histones. Les résultats histopathologiques et immunopathologiques (dépôt de IgM granulaire dans la jonction dermo-épidermique) ont confirmé le diagnostic de LECS. Larrêt de la nifédipine a entraîné des améliorations rapides, menant à une quasi-guérison des lésions cutanées en un mois, en labsence dun traitement actif. Une réduction du niveau des anticorps antinucléaires, anti-Ro/SSA et anti-histones a été perçue six mois plus tard. Conclusion: Un long traitement à la nifédipine peut causer le LEGS. Les anticorps anti-histones seraient associés au LEGS provoqué par des médicaments.

     

Simultaneous Occurrence of Subacute Cutaneous Lupus Erythematosus and Sweet Syndrome

Abstract: A 78-year-old man presented simultaneously with subacute cutaneous lupus erythematosus (SCLE) and Sweet syndrome (neutrophilic dermatosis). He was subsequently investigated for and found to have Sjögren syndrome. We believe that the simultaneous occurrence of the above cutaneous manifestations are very suggestive of and should prompt investigation for Sjögren syndrome.     

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。     

Cutaneous Lupus Erythematosus

Cutaneous lupus erythematosus (LE) may present in a variety of clinical forms. Three recognized subtypes of cutaneous LE are acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), and chronic cutaneous LE (CCLE). ACLE may be localized (most often as a malar or ‘butterfly’ rash) or generalized. Multisystem involvement as a component of systemic LE (SLE) is common, with prominent musculoskeletal symptoms. SCLE is highly photosensitive, with predominant distribution on the upper back, shoulders, neck, and anterior chest. SCLE is frequently associated with positive anti-Ro antibodies and may be induced by a variety of medications. Classic discoid LE is the most common form of CCLE, with indurated scaly plaques on the scalp, face, and ears, with characteristic scarring and pigmentary change. Less common forms of CCLE include hyperkeratotic LE, lupus tumidus, lupus profundus, and chilblain lupus. Common cutaneous disease associated with, but not specific for, LE includes vasculitis, livedo reticularis, alopecia, digital manifestations such as periungual telangiectasia and Raynaud phenomenon, photosensitivity, and bullous lesions. The clinical presentation of each of these forms, their diagnosis, and the inter-relationships between cutaneous LE and SLE are discussed. Common systemic findings in SLE are reviewed, as are diagnostic strategies, including histopathology, immunopathology, serology, and other laboratory findings. Treatments for cutaneous LE initially include preventive (e.g. photoprotective) strategies and topical therapies (corticosteroids and topical calcineurin inhibitors). For skin disease not controlled with these interventions, oral antimalarial agents (most commonly hydroxychloroquine) are often beneficial. Additional systemic therapies may be subdivided into conventional treatments (including corticosteroids, methotrexate, thalidomide, retinoids, dapsone, and azathioprine) and newer immunomodulatory therapies (including efalizumab, anti-tumor necrosis factor agents, intravenous immunoglobulin, and rituximab). We review evidence for the use of these medications in the treatment of cutaneous LE.     

Childhood Subacute Cutaneous Lupus Erythematosus Associated with Homozygous Complement 2 Deficiency

Abstract: We describe a 9-year-old boy with an Ro-positive, subacute, cutaneous lupus rash associated with homozygous C2 deficiency. His response to a mild topical steroid and sunscreen was excellent. Hereditary complement deficiency and its association with childhood lupus erythematosus are discussed.     

亚急性皮肤型红斑狼疮误诊为银屑病1例

患者女,72岁。颜面、躯干及四肢出现泛发性红斑鳞屑3月,初诊银屑病。背部皮损组织病理检查示:表皮角化过度、棘层肥厚与萎缩交替,基底层液化变性,真皮浅层淋巴细胞、噬色素细胞呈带状浸润,免疫学检查示:ANA1:320斑点型,抗Ro/SSA抗体2+,抗La/SSB抗体2+。诊断:亚急性皮肤型红斑狼疮。     

皮肤型红斑狼疮的治疗现状

皮肤型红斑狼疮是红斑狼疮病谱中相对较轻的一型,治疗上有别于系统性红斑狼疮,目前尚无固定的治疗模式。局部外用糖皮质激素是广泛采用的治疗手段之一,对各种皮肤型红斑狼疮均有效。较新型的外用制剂如他克莫司及吡美莫司,主要用于治疗对糖皮质激素和常规药物无效的皮肤型红斑狼疮。对外用药物治疗无效的皮损,可选择皮损内注射糖皮质激素,以快速发挥抗炎和免疫抑制作用。细胞毒药物和沙利度胺主要治疗复发或难治性皮肤型红斑狼疮。激光及光疗也能有效改善皮肤型红斑狼疮的皮损。     

Cutaneous Manifestations of Systemic Lupus Erythematosus

Sixty two cases of SLE were studied in a Medical Unit for a period of 4 years. Four different aspects of the cutaneous manifestations were analyzed and discussed. Butterfly rash, alopecia and photosensitive lesions were slightly more common than in Western series, whereas, Raynaud's phenomenon, discoid lesions and others were rarely found. Some other uncommon lesions, especially one case with bullae formation, one with gangrene of toe and another with subcutaneous calcinosis, were observed. Psoriasiform lesions and hyperkeratotic lesions were also found in this series. Skin lesions secondary to systemic disorders and therapy were also studied and the findings discussed.     

Clinical Manifestations of Cutaneous Lupus Erythematosus

Cutaneous lupus erythematosus (CLE) is a chronic inflammatory autoimmune disease with a broad spectrum of clinical manifestations and a variable course. In numerous investigations, it has been shown that exogenous factors, such as UV‐light and drugs, can induce this disease. However, not all clinical aspects can be explained and therefore, the pathogenesis of CLE is currently under extensive research. The various cutaneous manifestations of LE are divided into LE‐nonspecific and LE‐specific skin disease based on histologic criteria. LE‐nonspecific manifestations are mostly associated with systemic LE but can also occur in other diseases and include particularly vascular skin lesions such as pe‐riungual telangiectases. LE‐specific skin disease includes the subtypes of CLE such as acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), chronic cutaneous LE (CCLE), and intermittent CLE (ICLE). The subdivision of these subtypes with different prognosis and course is supported by genetic, clinical, histologic, and immunoserologic findings. The subtypes of CLE require a specific morphological and clinical analysis, which is described in the first part of this review. In the second part of this review, further diagnostic procedures and therapeutic strategies in patients with CLE are discussed.     

Subacute Cutaneous Lupus Erythematosus Presenting in Childhood: A Case Report and Review of the Literature

A 2‐year‐old African American, Hispanic boy presented with well‐defined, violaceous, annular dermal plaques without scale over the upper extremities, face, lower extremities, and buttocks. The clinical presentation and laboratory studies were consistent with a diagnosis of subacute cutaneous lupus erythematous (SCLE). SCLE presenting in childhood is exceedingly rare, with only eight cases previously reported. It is important to clinically differentiate SCLE from other eruptions more common to children, such as atopic dermatitis, urticarial drug eruptions, and psoriasis vulgaris, because progression to systemic lupus erythematous (SLE) may occur. SLE needs to be closely followed. We present the first case (to our knowledge) of SCLE in a child of African American or Hispanic descent and provide a table of other documented pediatric presentations of SCLE for comparison.     

Cutaneous Manifestations of Childhood Systemic Lupus Erythematosus

Abstract: We assessed the mucocutaneous signs in 57 children with classical systemic lupus erythematosus seen during a 6 year period. The female:male ratio was 4.2:1. Ages ranged from 4 to 15 years (mean 11.9 years) at the time of diagnosis. Cutaneous manifestations (77%) were the second most common finding, next to renal involvement (84%). The skin changes noted were malar rash (74%), oral ulcer (46%), vasculitis (42%), photosensitivity (40%), alopecia (32%), and discoid lupus erythematosus (LE) (19%). All 11 discoid LE patients were girls. Periungual erythema, Raynaud's phenomenon, periungual gangrene, nail involvement, and subacute LE were rare. Antinuclear antibody reaction was positive in 93% and anti-dsDNA was positive in 46%. Eight patients died, six from severe infection and two from renal failure.