Lupus panniculitis in an ANA-negative systemic Lupus Erythematosus patient: A case report

Introduction@#Lupus panniculitis (LP) is an unusual type of cutaneous lupus erythematosus (CLE) wherein the cutaneous inflam- matory reaction involves primarily the deeper dermis and subcutaneous fat. It is characterized by the appearance of recurrent, mostly asymptomatic, firm, nodules or plaques, involving the face, upper limbs, and buttocks.@*Case report@#In our case, a 30-year-old female presented with a non-tender, non-movable nodule on the left breast, 6 weeks prior to her admission. She had fever, chills, and joint pains. The patient later developed hyperpigmented plaques on the infra- clavicular area, and left flank extending to the abdomen. Urinalysis showed proteinuria, and RBC cast. She also had leukopenia, and anemia on CBC. Chest computerized tomography (CT) scan revealed a heterogeneously enhancing soft tissue mass in the base of the neck at the right infraclavicular region with malignant features. ANA titer was normal, while skin biopsy on two sites and direct immunofluorescence studies were compatible with lupus panniculitis. She was managed as a case of systemic lupus erythematosus (SLE) using a combination of hydroxychloroquine, and oral corticosteroids, which afforded temporary relief of symptoms. The patient however was lost to follow-up and opted for alternative medicine, and subsequently succumbed to the complications of SLE.@*Conclusion@#This case highlights the importance of a carefully made assessment after an accurate clinicopathological cor- relation was done. This case also emphasizes that although LP if associated with SLE may signify a milder condition, judicious monitoring and follow-up must still be undertaken since management is based on the disease activity.

Lúpus eritematoso crônico: uma abordagem clínica, epidemiológica, laboratorial e terapêutica / Chronic lupus erythematosus: a clinical approach, epidemiological, laboratory and therapeutic

O lúpus eritematoso cutâneo crônico, caracterizado, sobretudo, pelo lúpus discoide, é uma entidade clínica incomum, porém de elevada prevalência em mulheres em idade fértil. A sua etiologia é desconhecida, mas fatores genéticos, autoimunes, hormonais e ambientais compõem o processo fisiopatológico da doença. Os meios diagnósticos utilizados para que se possa iniciar o tratamento específico, composto de protetores solares, corticosteroides tópicos e, se preciso, medicações sistêmicas, principalmente os antimaláricos, são o exame clínico, a imunofluorescência direta e o estudo histopatológico. Este artigo descreve de maneira sucinta os principais aspectos epidemiológicos, clínicos, diagnósticos e terapêuticos do lúpus eritematoso cutâneo crônico, conforme revisão de literatura.

Clinical Features of the Skin Lesions of Patients with Chronic Cutaneous Lupus Erythematosus and Examination of the Factors That Are Relevant to Its Transformation to Systemic Lupus Erythematosus / 대한피부과학회지

BACKGROUND: Chronic cutaneous lupus erythematosus (CCLE) is a well-known autoimmune cutaneous disease that is part of the lupus erythematosus (LE) spectrum. OBJECTIVE: The aim of this study is to elucidate the clinical and laboratory features and the possible factors that are relevant to the aggravation of CCLE, as well as the possible precipitating factors for the transformation of cutaneous LE into multi-organ systemic disease. METHODS: A total of forty Korean patients with CCLE were selected for the study. We performed clinical examinations for the lesion-morphology and topographic distribution, as well as tests for the LE-related laboratory abnormalities, the precipitating factors for aggravation and the factors relevant to systemic lupus erythematosus (SLE). RESULTS: Among these forty CCLE patients, thirty-two patients (80.0%) had discoid erythema (DLE) lesions and 39 patients (97.5%) had cutaneous CCLE lesions that appeared on the head and neck areas. Twenty-nine patients (72.5%) had more than 3 CCLE lesions. All of the laboratory and clinical features of SLE were observed more frequently in the patients with widespread DLE with multiple lesions than in those patients with localized DLE. The most frequent aggravating factors that were recognized among these forty patients with CCLE were UV light, cold-exposure, physical trauma, pregnancy and smoking. Transformation into SLE developed in three patients (7.5%) during a 4-year period, and the most relevant findings related to the transformation into SLE were positive findings for leukopenia, antinuclear antibodies, anti-DNA antibodies and an increase of the erythrocyte sedimentation rate. CONCLUSION: Among these forty Korean cases of CCLE, multiple lesions of DLE on the head and neck areas were the most frequent clinical form, and this pattern is similar to the pattern seen in Caucasian people. The most frequent aggravating factor observed in individual patients was UV light. Patients with a widespread form of CCLE may have some laboratory findings of SLE such as leukopenia, antinuclear antibodies, anti-DNA antibodies or an elevated erythrocyte sedimentation rate. These patients should be observed closely because their disease might be transformed to SLE.

A Case of Tumid Lupus Erythematosus / 대한피부과학회지

Tumid lupus erythematosus is a rare subtype of chronic cutaneous lupus erythematosus. We report a case of tumid lupus erythematosus. A 51-year-old female patient, presented with a solitary walnut sized tender erythematous nodule on the left forehead. Histopathologic findings showed dense perivascular and periadnexal lymphocytic infiltration, mucin deposition between the dermal collagen fibers like cutaneous lupus erythematosus. But there was no involvement of epidermis or dermoepidermal junction. Those histopathologic findings suggest polymorphous light eruption, Jessner lymphocytic infiltration and reticulated erythematous mucinosis. Mucin deposition is seen in reticulated erythematous mucinosis and tumid lupus erythematosus. To distinguish tumid lupus erythematosus from reticulated erythematous mucinosis, clinicopathologic correlation is essential.

Clinical Features and Predilection Sites of the Lesions Observed in Chronic Cutaneous Lupus Erythematosus / 대한류마티스학회지

OBJECTIVE: The typical clinical feature of chronic cutaneous lupus erythematosus (CCLE) is 'discoid erythema'; however, several different clinical variants exist such as hypertrophic LE, LE profundus, chilblain LE, tumid LE and so on. The heterogeneity-spectrum of the lesional morphology of the cutaneous lesions and the predilection sites of the lesions of twenty Korean patients with CCLE were examined and tried to compare to those of CCLE patients of Caucasian origin. METHODS: Clinical examinations of twenty patients with CCLE were carried out by dermatologists to define the morphologic characteristics of cutaneous lesions and the anatomic predilection sites of the lesions. RESULTS: The most common cutaneous lesion-morphology noted in Korean patients with CCLE was classic discoid erythema. Three cases had concurrent cutaneous lesions of other clinical variants of CCLE. The predilection sites of all lesions were the face (cheeks) and head (frontal area). These clinical findings observed among this group of patients are similar to those features seen in people of Caucasian origin. CONCLUSION: The most frequent clinical feature of CCLE was discoid erythema and the predilections sites of the lesions found were the face and the scalp; indicating that the morphological heterogeneity-spectrum of cutaneous lesions and the predilection sites observed in Korean patients of CCLE seem to have no remarkable differences when compared with Caucasian cases with the same diagnoses.

A Case of Hypertrophic Lupus Erythematosus / 대한피부과학회지

Hypertrophic lupus erythematosus is a clinical variant of chronic cutaneous lupus erythematosus (CCLE). It is critical to differentiate hypertrophic lupus erythematosus from CCLE because of treatment and prognosis. We report a case of hypertrophic lupus erythematosus with CCLE occured on arms. Combination therapy with acitretin and intralesional corticosteroid injection resulted in control of previously refractory skin lesion.

Two Cases of Hypertrophic Discoid Erythemas in Patients with Chronic Cutaneous Lupus Erythematosus / 대한피부과학회지

Hypertrophic discoid erythema is a clinical variant of chronic cutaneous lupus erythematosus (CCLE) found most often in association with the more classical lesions of discoid erythemas. We report two cases of CCLE with hypertrophic discoid erythemas occurred on the arms; a rare form of cutaneous lesion of CCLE.

Chronic Cutaneous Lupus Erythematosus in Two Sisters / 대한피부과학회지

Scalp involvements occur in 30-60% of patients with chronic cutaneous lupus erythematosus (CCLE), and may result in irreversible cicartricial alopecia due to follicular destructions. The pathogenesis of CCLE is consiclered to be multifactorial; relevant factors would be immunogenetic, hormonal, and environmental ir fluences. Perhaps, the immunogenetic factors may be more important. We report a case of two sisters who had superficial scarring alopecia on the scalp, in the constitutions of underlying mixed connective tissue disease (MCTD) and undifferentiated connective tissue syndrome (UCTS), respectively. This sister-CCLE case is regarded to be the first report among familial CCLE in Korea.