Manifestações cutâneas no lúpus eritematoso: o que o clínico precisa saber / Skin manifestations in the lupus erythematosus: what the clinician need to know

O lúpus eritematoso é uma doença autoimune complexa que afeta diversos órgãos. A pele é o segundo local mais acometido e as manifestações cutâneas são divididas em específicas e não específicas. As primeiras possuem em comum o achado histopatológico de dermatite de interface com degeneração vacuolar da camada basal e infiltrado linfocitário na junção dermoepidérmica e são subdivididas em agudas, subagudas e crônicas. As manifestações não específicas compõem um grupo heterogêneo de condições que frequentemente está associado ao lúpus eritematoso sistêmico. O conhecimento e a interpretação adequada de tais manifestações é importante, pois, além da relevância diagnóstica, as lesões cutâneas nos dão informações prognósticas. Portanto, é imprescindível que o clínico conheça as principais manifestações cutâneas do lúpus eritematoso para a melhor condução desses pacientes.

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.

Correção cirúrgica de cicatriz atrófica de paniculite lúpica pela técnica da W-plastia / Surgical correction of atrophic scar of lupic paniculitis by the W-plasty technique

Contexto: A técnica cirúrgica chamada de W-plastia é indicada quando o objetivo é melhorar cicatrizes na região facial, principalmente em áreas convexas muito aparentes. Descrição do caso: Mulher de 53 anos, em acompanhamento por cicatriz em face secundária à paniculite lúpica, foi submetida à correção de cicatriz por W-plastia. Discussão: Esta técnica simples consiste no avanço de triângulos uniformemente interpostos, produzindo um padrão em ziguezague. Conclusão: Relatamos este procedimento cirúrgico visando demonstrar sua utilidade no manejo de cicatriz inestética.

Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort study

Abstract Objective: To evaluate prevalence, clinical manifestations, laboratory abnormalities, treatment and outcome in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients with and without panniculitis. Methods: Panniculitis was diagnosed due to painful subcutaneous nodules and/or plaques in deep dermis/subcutaneous tissues and lobular/mixed panniculitis with lymphocytic lobular inflammatory infiltrate in skin biopsy. Statistical analysis was performed using Bonferroni correction(p < 0.004). Results: Panniculitis was observed in 6/847(0.7%) cSLE. Painful subcutaneous erythematosus and indurated nodules were observed in 6/6 panniculitis patients and painful subcutaneous plaques in 4/6. Generalized distribution was evidenced in 3/6 and localized in upper limbs in 2/6 and face in 1/6. Cutaneous hyperpigmentation and/or cutaneous atrophy occurred in 5/6. Histopathology features showed lobular panniculitis without vasculitis in 5/6(one of them had concomitant obliterative vasculopathy due to antiphospholipid syndrome) and panniculitis with vasculitis in 1/6. Comparison between cSLE with panniculitis and 60 cSLE without panniculitis with same disease duration [2.75(0-11.4) vs. 2.83(0-11.8) years,p = 0.297], showed higher frequencies of constitutional involvement (67% vs. 10%,p = 0.003) and leukopenia (67% vs. 7%,p = 0.002). Cutaneous atrophy and hyperpigmentation occurred in 83% of patients. Conclusions: Panniculitis is a rare skin manifestation of cSLE occurring in the first three years of disease with considerable sequelae. The majority of patients have concomitant mild lupus manifestations.

Lupus eritematoso sistémico masculino / Systemic male lupus erythematosus

El Lupus Eritematosos Sistémico es el paradigma del síndrome autoinmune sistémico, cuya etiología está lejos de ser aclarada, aunque el conocimiento de su patogenia ha avanzado en estos últimos años inexorablemente, como el de los secretos más ocultos del funcionamiento del sistema autoinmune. Es mucho más frecuente en mujeres (10:1) y suele presentarse en la adolescencia tardía y a los 50 años, también es más frecuente y grave en algunos grupos étnicos, en especial afroamericanos e hispanos; su carácter crónico, su gran variedad clínica, sus episodios de activaciones y remisiones, la presencia de numerosos anticuerpos y la respuesta al tratamiento inmunosupresor son muestra de su naturaleza autoinmune. Presentamos el caso de un paciente de sexo masculino, de 38 años de edad, de origen hispano, que debutó con serositis, y que respondió de forma adecuada al tratamiento inmunosupresor instaurado.

Systemic Lupus Erythematosus is the paradigm of systemic autoimmune syndrome, whose etiologyis farfrom being clarified, although the knowledge of its pathogenesis has inexorably advanced in recent years, such as the most hidden secrets of the functioning of the autoimmune system. It is much more common in women (10: 1) and usually occurs in late adolescence and at age 50, is also more frequent and severe in some ethnic groups, especially African American and Hispanic; its chronic nature, its great clinical variety, its episodes of activation and remission, the presence of numerous antibodies and the response to immunosuppressive treatment are indicative of its autoimmune nature. We present the case of a 38-year-old male patient of Hispanic origin, who debuted with serositis, and who responded adequately to the immunosuppressive treatment instituted.

A Case of Lupus Erythematosus Panniculitis of the Scalp Presenting as Doughnut-shaped Alopecia along Blaschko's lines / 대한피부과학회지

A 9-year-old girl presented with a 2-year history of doughnut-shaped hair loss on the frontal scalp, resembling the symmetric distribution of Blaschko's lines. Physical examination showed an alopecic patch with mild scalp induration. Histopathology revealed lymphoplasmacytic infiltration of the perifollicular dermis and subcutaneous lobules with abundant mucin deposition, consistent with a diagnosis of lupus erythematosus panniculitis. Three cases of linear lupus erythematosus panniculitis of the scalp presenting as alopecia along Blaschko's lines have been documented in Korean dermatologic literature. However, there have been no reports of doughnut-shaped alopecia until now. Herein, we present an interesting case of a pediatric patient with a unique feature of doughnut-shaped alopecia along Blaschko's lines.

Adalimumab-induced Lupus Erythematosus Profundus in a Rheumatoid Arthritis Patient / 대한피부과학회지

The clinical manifestations and immunohistologic findings of drug-induced lupus erythematosus (DILE) are similar to those of idiopathic lupus. However, DILE is different from idiopathic lupus because it is induced after continuous drug exposure and resolves after discontinuation of the causative drug. DILE can be divided into systemic lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus (CCLE). Lupus erythematosus profundus is a subtype of CCLE, and drug-induced CCLE is very rarely reported in the literature. Herein, we report a rare case of adalimumab-induced lupus erythematosus profundus developed in a rheumatoid arthritis patient. The patient is a 43-year-old Korean woman who had multiple tender nodules and plaques on her face, trunk, and both extremities after using adalimumab for rheumatoid arthritis. She was diagnosed with adalimumab-induced lupus erythematosus profundus, and her condition improved after discontinuation of adalimumab.

Adalimumab-induced Lupus Erythematosus Profundus in a Rheumatoid Arthritis Patient / 대한피부과학회지

The clinical manifestations and immunohistologic findings of drug-induced lupus erythematosus (DILE) are similar to those of idiopathic lupus. However, DILE is different from idiopathic lupus because it is induced after continuous drug exposure and resolves after discontinuation of the causative drug. DILE can be divided into systemic lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus (CCLE). Lupus erythematosus profundus is a subtype of CCLE, and drug-induced CCLE is very rarely reported in the literature. Herein, we report a rare case of adalimumab-induced lupus erythematosus profundus developed in a rheumatoid arthritis patient. The patient is a 43-year-old Korean woman who had multiple tender nodules and plaques on her face, trunk, and both extremities after using adalimumab for rheumatoid arthritis. She was diagnosed with adalimumab-induced lupus erythematosus profundus, and her condition improved after discontinuation of adalimumab.

A study of the clinico-histopathological features of erythematous tender nodules predominantly involving the extremities.

Background: Erythematous tender nodules predominantly involving extremities are frequently encountered in dermatology and rheumatology practice. They are diagnosed based on distinct clinical and histopathological features. However, in clinical situations, considerable overlap is observed that poses a diagnostic challenge. We undertook a study on clinico-histological patterns of inflammatory nodules over extremities. Methods: After detailed history and examination, a preliminary clinical diagnosis was made in 43 cases, followed by skin biopsy from representative nodules. Histological diagnosis made was correlated with clinical features. Results: Of 43 cases, a single clinical diagnosis was made in 25 (58.5%) cases while in the remaining cases more than one diagnosis was considered. On correlating with the histopathological diagnosis, concordance was observed in 51% cases while the remaining showed either histological discordance with clinical diagnosis (14% cases) or were kept in the undecided category (35% cases). Conclusion: Considerable clinico-histological overlap was observed in inflammatory nodules over extremities. Histopathology alone was not helpful in differentiating one entity from another at all times since variable histo-pathological patterns were seen.

Lupus Profundus with Segmental Distribution on Leg, Following the Lines of Blaschko in a Child / 대한피부과학회지

Lupus profundus is a rare subtype of chronic cutaneous lupus erythematosus, which shows a tender subcutaneous nodule or plaque. The face, arm, buttock, trunk and thighs are frequently involved. Lupus profundus can be associated with or without systemic lupus erythematosus. But due to its rarity, it is difficult to diagnosis and there are not many reports regarding its characteristics, including its distribution. In this case, a 13-year-old boy has visited with several violaceous non-tender nodules on Lt. upper thigh with segmental distribution, which follows the Blaschko's line. Through skin biopsy and its immunoflourescent study, we diagnosed lupus profundus. We here report a case of lupus profundus with segmental distribution following the lines of Blaschko's in a child.

Lupus Erythematosus Profundus Associated with Kikuchi's Disease / 대한피부과학회지

Kikuchi's disease (KD), histiocytic necrotizing lymphadenitis, is a rare self-limited lymphadenopathy, which usually affects young women. KD has been reported to precede, coexist with or follow the diagnosis of other entities, such as systemic lupus erythematosus (SLE), adult-onset Still's disease, Hashimoto's disease, and viral infections. In a few cases, KD is associated with cutaneous lupus erythematosus (CLE), without systemic involvement. Herein, we report the first Korean case of KD associated with lupus erythematous profundus in a 9-year-old boy.

Calcifying Lupus panniculitis in a patient without manifestations of systemic lupus Erythematosus / Paniculitis Lúpica calcificante en un paciente sin manifestaciones de lupus eritematoso sistémico

Lupus panniculitis or lupus profundus is a variant of lupus Erythematosus cutaneous that primarily affects subcutaneous tissue. Clinically, it is characterized by one or several firm subcutaneous nodules and/or plaques with or without overlying epidermal changes. It is reported to occur with a frequency of 2-3% in patients with Systemic Lupus Erythematosus (SLE). Between 10 and 50 percent of patients with lupus panniculitis will have or eventually develop Systemic Lupus Erythematosus. In nearly all cases there are deep, erythematosus plaques and nodules, and some of them ulcers, which usually involve the proximal extremities, trunk, breasts, buttocks, and face. These lesions may be tender and painful and frequently heal with atrophy and scaring, turning as a chronic condition and subsequently heal with disfigurement. We describe a patient who suffers from lupus panniculitis with no association to SLE symptoms and complicated by several progressive and disabling cutaneous lesions.

La paniculitis lúpica o también llamada lupus profundus es una variante del lupus eritematoso cutáneo que afecta el tejido celular subcutáneo. Se caracteriza clínicamente por uno o varios nódulos subcutáneos que son firmes y/o placa con o sin cambios epidérmicos. Se ha informado su frecuencia en 2% a 3% de casos de Lupus eritematoso sistémico. Entre el 10 al 50% de los casos de paniculitis lúpica va a desarrollar lupus eritematoso sistémico. En casi todos los casos hay placas eritematosas y/o nódulos que en algunos casos se ulceran y que usualmente están localizados en las áreas proximales de las extremidades, tronco, mamas, nalgas y la cara. Estas lesiones pueden ser clínicamente dolorosas y sensibles a la presión y frecuentemente cicatrizan con desfiguración del área circundante. Describimos un paciente que padece de paniculitis lúpica sin asociación de lupus eritematoso sistémico y que se complicó con varias lesiones cutáneas progresivas y discapacitantes.

A Case of Lupus Panniculitis-Misdiagnosed as Alopecia Areata

We present a 24-year-old woman with systemic lupus erythematosus presented with multiple patches of non-scarring alopecia closely resembling alopecia areata. Scalp biopsy showed a predominantly subcutaneous and dermal lymphocytic infiltrate that surrounded the deep follicular segments and hair bulb. This pattern is capable of producing a temporary hair loss, clinically misdiagnosed as alopecia areata. The clinical history, presence of erythema on bald patches and scalp tenderness as well as the biopsy findings were important clues in diagnosis of lupus erythematosus. We report here a case of lupus panniculitis that presented as multiple bald patches misdiagnosed as alopecia areata.

Lupus erythematosus panniculitis: clinical and histopathological analysis of 22 cases / 中国医学科学院学报

<p><b>OBJECTIVE</b>To explore the clinical and histopathological manifestations, therapy and prognosis of lupus erythematosus panniculitis (LEP).</p><p><b>METHOD</b>We retrospectively reviewed the clinical data and histopathological features of 22 cases of LEP diagnosed at Peking Union Medical College Hospital from January 2008 to February 2010.</p><p><b>RESULTS</b>The lesions appeared as atrophy, erythema, subcutaneous nodules, infiltrated erythema, and ulceration over the scalp, face, upper limbs, and buttock. Histopathological features were lobular panniculitis in the subcutaneous fat; sometimes septal panniculitis could be seen. Hydroxychloroquine sulfate and corticosteroid were effective treatment; most patients responded well to the treatment, while a few experienced recurrence when the treatment tapped or discontinued.</p><p><b>CONCLUSIONS</b>LEP is a rare cutaneous variant of lupus erythematosus. The diagnosis of LEP is mainly based on clinical findings and pathological features. Hydroxychloroquine and low- and middle-dose corticosteroid are effective for the treatment of LEP.</p>

Case 1

A 7 - year - old Caucasian girl presented with a 3-month history of nodules on her thighs and buttocks. During a one-month period, the lesions gradually increased in size and number. They became indurate and tender. Similar nodules appeared on forearms after 4 months. The lesions gradually healed with a depressed scar. On physical examination, we found many indurate areas 10-40 mm in diameter .The surface of some of them was erythematous and scaly. The lesions were not ulcerated and did not have a discharge [figure 1]. On examination, she was afebrile and healthy, organs such as heart, lungs, abdomen, joints and muscles were normal. Laboratory evaluation findings including complete blood cell count, liver and renal function test values, ESR, CRP,VDRL, BUN, Creatinin, complement were all normal .The titers for FANA, Anti ds DNA, Anti Sm, Anti Scl-70, Anti -RNP, Anti-Ro, Anti-La, Anti -Jo1, Anti Phospholipid Ab, Anti cardiolipin Ab, c- ANCA, p- ANCA were negative. A skin biopsy was taken [figures 2]