Effects of lipografts in immune-driven lipoatrophies. Case studies

Lipodystrophy is a pathological condition associated with an abnormal body adipose tissue redistribution. Facial lipoatrophy can be a consequence of congenital, acquired, or involutional. The lipograft is an autologous fat transplant, which constitutes a treatment option that provides volume, tissue regeneration, and advantages in relation to other fillers in autoimmune diseases. The aim is to highlight the filling action and the metabolic effect of facial lipotransfer, due to the grafted adipocytes survival, and the adipose tissue derived stem cells regenerative activity obtained by nano-fat in patients with facial lipoatrophy. Lipoinjection improves the architecture of the new dermis and increases its functional capacity. It is a treatment with autologous tissue (fatty graft) with great efficacy in relation to other alloplastic filler materials capable of exacerbating an inflammatory response mediated by antibody production.

La lipodistrofia es una condición patológica asociada a una redistribución anómala del tejido adiposo en el cuerpo. La lipoatrofia facial puede ser consecuencia de defectos congénitos, adquiridos o involutivos. El lipoinjerto es el trasplante de grasa autógena y constituye una opción de tratamiento que aporta volumen, regeneración tisular y ventajas en relación con otros materiales de relleno en enfermedades autoinmunes. Se busca resaltar la acción de relleno y el efecto metabólico de la lipotransferencia facial, por la supervivencia de los adipocitos injertados y la actividad regenerativa de las células madre provenientes del tejido adiposo obtenidas por nanofat en pacientes con lipoatrofia facial. La lipoinyección mejora la arquitectura de la nueva dermis y aumenta su capacidad funcional, es un tratamiento con tejido autógeno (injerto graso) con gran eficacia en relación con otros materiales de relleno aloplásticos capaces de exacerbar una respuesta inflamatoria mediada por la producción de anticuerpos.

Lupus eritematoso cutáneo subagudo psoriasiforme. Reporte de caso / Subacute psoriasiform cutaneous lupus erythematosus. Case report

El Lupus eritematoso sistemico (LES) subagudo representa el 10% del total de los casos y en su variedad psoriasiforme resulta poco frecuente. Presentamos el caso de una paciente femenina de 22 años con el diagnóstico previo de LES y nefropatía lúpica hace 2 años, que refiere cuadro clínico de +/- aproximadamente 2 meses de evolución posterior a la suspensión de micofenolato de mofetilo. Ccuadro caracterizado por la aparición progresiva de lesiones dérmicas discoides, costrosas y descamativas que comprometen aproximadamente el 80% de la superficie corporal acompañado de orina espumosa. Examen físico: lesiones corporales respetando palmas y plantas, dolorosas a la digitopresión. Llenado capilar > a 2 segundos. Laboratorios: leucocitos 5930 y Granulocitos 90%. Examen general de orina: infeccioso. Se trata de LES cutáneo subagudo psoriasiforme. El tratamiento fue antibiótico, inmunosupresor y antihipertensivo. Se otorgó el Aalta hospitalaria con micofenolato y ciprofloxacino. El seguimiento debe realizarse por personal médico especializado en reumatología, nefrología y dermatología.

Subacute systemic lupus erythematosus (SLE) represents 10% of all cases and is rare in its psoriasiform variety. We present the case of a 22-year-old female patient with a previous diagnosis of SLE and lupus nephropathy 2 years ago, who reported a clinical picture of +/- 2 months of evolution after the suspension of mycophenolate mofetil. Table characterized by the progressive appearance of discoid, crusty and scaly dermal lesions that involve approximately 80% of the body surface accompanied by foamy urine. Physical examination: bodily injuries respecting palms and soles, painful on acupressure. Capillary filling> 2 seconds. Laboratories: leukocytes 5930 and Granulocytes 90%. General urine test: infectious. This is subacute psoriasiform cutaneous SLE. The treatment was antibiotic, immunosuppressive and antihypertensive. Hospital discharge with mycophenolate and ciprofloxacin. Follow-up should be by medical personnel specialized in rheumatology, nephrology, and dermatology.

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.

Quinacrine: An Effective Addition to the Treatment of Refractory Cutaneous Lupus Erythematosus

Summary@#Treatment of refractory cutaneous lupus is challenging. When conventional therapy, including hydroxychloroquine (HCQ), corticosteroids and immunosuppressants, has failed, the addition of quinacrine may be a promising option. We describe a case of refractory chronic cutaneous lupus erythematosus (CCLE) who responded well to quinacrine.

Lupus eritematoso asociado a eritema multiforme: Síndrome de Rowell. Reporte del primer caso en Chile

Rowell's syndrome is characterized by an association of lupus erythematosus and erythema multiforme, with distinctive laboratory findings. Its treatment is similar to lupus. We report a 16-year-old female presenting with skin lesions, laboratory and histology consistent with this entity. Because of the spread of cutaneous involvement and development of epidermal detachment, she required therapy with intravenous corticosteroids and gamma globulin.

Lupus eritematoso cutáneo y lupus eritematoso sistémico: revisión de una historia sin fin / Cutaneous lupus erythematosus and systemic lupus erythematosus: endless story review

l lupus eritematoso es una afección que se conoce desde antiguo, referida en especial al compromiso cutáneo, pero ha sido mejor definida desde principios del siglo XIX, constituyéndose como una enfermedad sistémica con una base autoinmune. Mecanismos patogénicos inmunogenéticos desempeñan un papel en la susceptibilidad a la enfermedad, sobre la que actuarían variaciones hormonales y factores ambientales. De esta interacción deriva la producción de múltiples anormalidades inmunológicas, cuya acción sobre los tejidos da origen a la expresión clínica de la enfermedad. La relación entre el compromiso cutáneo, en especial crónico discoide, y sistémico, ha sido un factor de polémica, importante de dilucidar por el significado terapéutico y de pronóstico para el enfermo. De esta revisión se puede concluir que las diferentes manifestaciones del lupus constituyen una misma enfermedad, pero probablemente factores etiopatogénicos genéticos, hormonales y ambientales marcan la diferencia.

Lupus erythematosus is a condition that has been known since ancient times, especially referring to skin involvement, but has been better defined since the beginning of the 19th century, constituting itself as a systemic disease with an autoimmune basis. Immunogenetic pathogenic mechanisms play a role in disease susceptibility, on which hormonal variations and environmental factors would act. From this interaction derives the production of multiple immunological abnormalities, whose action on the tissues gives rise to the clinical expression of the disease. The relationship between cutaneous involvement, especially chronic discoid, and systemic, has been a controversial factor, important to elucidate due to its therapeutic and prognostic significance for the patient. From this review it can be concluded that the different manifestations of lupus constitute the same disease, but genetics, hormonal and environmental etiopathogenic factors probably make the difference.

Diseases, conditions, and drugs associated with cicatricial ectropion / Doenças, condições e drogas associadas ao ectrópio cicatricial

ABSTRACT Cicatricial ectropion may be a consequence of certain systemic diseases as well as the result of drug use. Our goal here was to research the different causes of this condition as reported in the literature, including more recently suspected etiologies. A detailed PubMed literature search indicated many different etiologies were associated with cicatricial ectropion development, from severe cases of systemic diseases, such as ichthyosis and lupus erythematosus, to reversible scenarios secondary to anti-glaucomatous drug use. More recently reported connections include periorbital necrotizing fasciitis, frontal osteomyelitis, and antineoplastic agents. Indeed, cicatricial ectropion may be highly symptomatic; being able to determine its real etiology is imperative to managing patients properly. In this investigation, we felt that an explicitly multidisciplinary approach was essential, especially for cases associated with systemic conditions.

RESUMO O ectrópio cicatricial pode ser uma consequência de certas doenças sistêmicas, bem como o resultado do uso de drogas. Nosso objetivo aqui foi pesquisar as diferentes causas desta condição, conforme relatado na literatura, incluindo mais recentemente suspeitas de etiologias. Uma pesquisa bibliográfica detalhada do PubMed indicou que muitas etiologias diferentes estavam associadas ao desenvolvimento do ectrópio cicatricial, desde casos graves de doenças sistêmicas, como ictiose e lúpus eritematoso, até cenários reversíveis secundários ao uso de drogas antiglaucomatosas. Conexões relatadas mais recentemente incluem fasceíte necrosante periorbitária, osteomielite frontal e agentes antineoplásicos. De fato, o ectrópio cicatricial pode ser altamente sintomático; ser capaz de determinar sua etiologia real é imperativo para o manejo adequado dos pacientes. Nesta investigação, sentimos que uma abordagem explicitamente multidisciplinar era essencial, especialmente para casos associados a doenças sistêmicas.

Lupus eritematoso neonatal. Reporte de caso de lactante concebida por inseminación artificial y madre con artritis reumatoidea / Lupus eritematoso neonatal Report of the infant case conceived by artificial insemination and mother with rheumatoid arthritis

El Lupus Eritematoso Neonatal es una enfermedad de origen autoinmune caracterizada por rash cutáneo transitorio, bloqueo cardíaco congénito permanente, función hepática anormal con o sin enfermedad biliar y compromiso hematológico asociado a la presencia de autoanticuerpos maternos contra la ribonucleoproteinas solubles (SSB/La, SSA/Ro y Anti-RNP). Se presenta el caso de una niña de cinco meses de edad con hallazgos clínicos e histopatológicos de Lupus eritematoso neonatal. Es una condición que no suele dejar secuelas, aunque se han reportado casos de atrofia cutánea e hiperpigmentación

Neonatal Lupus Erythematosus is an autoimmune disease characterized by transitory cutaneous rash, congenital heart block, abnormal liver function test with or without cholestasis and hematologic features associated to anti-Ro and anti-La autoantibodies. A 5-month-old female is brought to the hospital with clinical and histopathology findings of Neonatal lupus. This medical condition does not leave long term physical damage, however there have been cases reported with cutaneous atrophy and hyperpigmentation

Periodontal disease defects and mandibular cortical index in lupus patients / Defeitos ósseos periodontais e índice da cortical mandibular em pacientes com lupus

Introduction: Systemic lupus erythematosus is an autoimmune disease that affects multiple organs. It is well known that lupus patients have higher risk of osteoporosis, but if the disease affects mandibular cortical bone and alveolar bone is not fully established. Objective: The objective of this study was to evaluate periodontal disease defects and mandibular osteoporotic alterations in patients with lupus as compared to healthy patients using panoramic radiographs. Material and Methods: The panoramic radiographs of 72 patients with lupus and 360 healthy patients were evaluated for the presence of bone loss secondary to periodontal disease, classified as horizontal and vertical bone loss. We also assessed mandibular osteoporotic alterations by using the mandibular cortical index. Logistic regression analysis was applied to estimate the risk of mandibular osteoporotic alterations as well as horizontal and vertical bone loss in patients with lupus as compared to healthy patients. Results: There were no statistically significant differences between groups in the presence of horizontal bone defects and mandibular cortical indexes. However, patients with lupus demonstrated that patients with lupus were 2.17 more likely to present vertical bone loss than healthy patients. Conclusions: Patients with lupus might have higher risk of vertical bone loss than healthy patients due to pathophysiology of their disease. Further larger prospective studies should be performed to confirm our findings (AU)

Introdução: Os lúpus eritematoso sistêmico é uma doença autoimune que afeta múltiplos órgãos. Pacientes com lúpus têm maior risco de osteoporose, mas é necessário elucidar-se como a doença afeta o esqueleto maxilo-mandibular. Objetivo: O objetivo deste estudo foi avaliar defeitos ósseos por doença periodontal e alterações osteoporóticas mandibulares em pacientes com lúpus, em comparação com pacientes saudáveis, utilizando-se radiografias panorâmicas. Material e Métodos: As radiografias panorâmicas de 72 pacientes com lúpus e 360 pacientes saudáveis foram avaliadas quanto à presença de defeitos ósseos verticais e horizontais por doença periodontal. Foram também avaliadas as alterações osteoporóticas da mandíbula por meio do índice da cortical mandibular. A regressão logística foi aplicada para estimar o risco de alterações osteoporóticas mandibulares, bem como a perda óssea horizontal e vertical em pacientes com lúpus, em comparação com pacientes saudáveis. Resultados: Não houveram diferenças estatisticamente significantes entre os grupos no tocante à presença de defeitos ósseos horizontais quanto à redução da densidade mineral óssea aferida por meio do índice da cortical mandibular. No entanto, pacientes com lúpus apresentaram 2,17 mais risco à perda óssea vertical do que pacientes saudáveis. Conclusões: Pacientes com lúpus podem ter maior risco de apresentar defeito ósseo vertical do que pacientes saudáveis devido à fisiopatologia de sua doença. Novos estudos prospectivos devem ser realizados para confirmar estes achados (AU)

Anti TNF como inductor de lupus cutáneo: caso clínico / Anti TNF as inducer of cutaneous lupus: clinical case

El mayor acceso a las terapias biológicas para el tratamiento de múltiples enfer-medades autoinmune trae consigo el mayor riesgo de padecer eventos adversos relacionados al uso de estos2,4. Presentamos un caso clínico de una paciente con diagnóstico de artritis reumatoide en tratamiento con ANTI TNF

The greater access to biological therapies for the treatment of multiple autoim-mune diseases brings with it the greatest risk of suffering adverse events related to the use of these (2,4). We present a clinical case of a patient diagnosed with rheumatoid arthritis in treatment with ANTI TNF

Caracterización clínicae histopatológica de pacientes con tumor de conjuntiva / Histopathological characterization of patients with conjunctival tumor

Introducción:Eltumor de conjuntiva es el más frecuente de los tumores oftálmicos y se reporta un aumento de su incidencia en los últimos 5 años. Objetivo:Caracterizar a los pacientes con tumor de conjuntiva atendidos en el Hospital Abel Santamaría durante el periodo de enero de 2014 a enero del 2017 desde el punto de vista clínico e histopatológico.Métodos:Se realizó una investigación observacional, descriptiva, transversal, en pacientes con diagnóstico de tumor de conjuntiva. Las variables estudiadas fueron: edad, sexo, diagnóstico anatomo patológico, factores de riesgos biológicos y externos. El universo estuvo constituido por 1660 pacientes que acudieron a consulta de oculoplastia y la muestra conformada por 366 pacientes con el diagnóstico presuntivo de tumor de conjuntiva. Resultados:En el grupo de edades de 40-60 años estuvieron el 52.7 %de los pacientes que fueron estudiados, el 57.7 % eran del sexo femenino. Los antecedentes patológicos familiares de presencia de la entidad estuvieron presentes en el 18.6%de la muestra estudiada y la exposición a los rayosultravioletas en el 71%, el 56.4 % pertenecieron a la zona urbana. El Pterigion estuvo representado en el 95.9 % de los casos con diagnostico histopatológico de tumor de conjuntiva.Conclusiones:El tumor deconjuntiva es frecuente en la mediana edad y en el sexo femenino, la exposición mantenida a los rayos ultravioletas sigue constituyendo un factor de riesgo importante para padecerlo y se reporta el pterigion con una elevada incidencia en el estudio

Introduction:Conjunctival tumor is the most frequent of the ophthalmic tumors reported, which has increased its incidence in the last 5 years. Objective:To characterize, from the histopathological point of view, patients with conjunctivaltumor treated at Abel Santamaría Cuadrado General Teaching Hospital throughout the period 2014-2017. Methods:An observational, descriptive, cross-sectional research was performed in patients with a diagnosis of conjunctival tumor. The variables studied were: age, sex, and pathological diagnoses, biological along with external risk factors. The target group consisted of 1660 patients who attended the oculoplasty office and the sample consisted of 366 patients with the presumptive diagnosis of conjunctivaltumor. Results:Patients between 40-60 years old prevailed (52.7%), out of them 57.7% was female gender. The familial pathological history (18.6%) and exposure to ultraviolet rays (71%) were the risk factors mostly referred to. Conclusions:The high incidence of conjunctival tumors is frequent in middle age; family history and exposure to ultraviolet rays were the most common risk factors. Pterygium was the most represented conjunctival tumor.

Hidroxicloroquina y retinopatía en pacientes reumatológicos / Hydroxychloroquine use and retinopathy in rheumatologic patients

: relacionar el uso de hidroxicloroquina con la presencia de retinopatía en pacientes reumatológicos. Métodos: se realizó un estudio retrospectivo y descriptivo, de revisión de historias clínicas de pacientes reumatológicos en tratamiento con hidroxicloroquina. Se seleccionaron aquellos con evaluación oftalmológica previa al inicio del tratamiento y estudios como la OCT-SD. Se recolectaron variables clínico epidemiológicas, cálculo de dosis diaria y acumulada del fármaco, y duración del tratamiento. Resultados: se revisaron 150 historias, de las cuales 47 cumplieron con los criterios de inclusión; 44 (93,6%) eran del género femenino y 3 (6,4%) del género masculino. La edad promedio fue de 47 ± 14 años. La hipertensión arterial fue la comorbilidad más frecuente. La patología reumatológica más frecuente fue el Lupus (53,2%). La dosis diaria de hidroxicloroquina fue ≤ 6,5 mg/kg/día en los 47 pacientes; el tiempo promedio de consumo fue de 5 años; y la dosis acumulada promedio fue de 498,5 ± 503,68 gramos. Se detectó toxicidad retiniana en 18 pacientes (38,3%), de los cuales: 17(36,2%) tuvo daño precoz y 1 (2,1%) daño moderado. Se observó relación estadística significativa entre toxicidad retiniana y dosis acumulada menores a 1.000 gramos (p= 0,032) y un tiempo de consumo mayor o igual a 5 años (p = 0,045) Conclusiones: las alteraciones iniciales en las capas externas de la retina ayudan a la detección precoz de toxicidad retiniana por hidroxicloroquina, siendo la OCT-SD un método sensible y fácil de realizar en la práctica clínica(AU)

To establish the relationship between the use of hydroxychloroquine and retinopathies in rheumatologic patients. Methods: This is a retrospective, descriptive study of the medical charts of rheumatologic patients' who were receiving hydroxychloroquine. We selected patients previously seen in ophthalmologic services, and ophthalmologic coherence tomography (SD OCT) had been realized. We collected clinical and epidemiologic variables such as daily doses, accumulated doses and prescription duration. Results: we selected 47 medical charts; the female gender is 44 and three gender male. Mean age was 47 +14 years.Hypertension was the most frequent comorbidity.Lupus was the most frequent rheumatologic illness.Hydroxychloroquine daily dose was < 6.5 mg/Kg/day and treatment`s mean duration was 5 years; average accumulated dosage was 498.5 + 503.68 grs. We established retinal toxicity in 18 patients (38.3%), in which 17 (36.2%) had early damage, and 1(2.1%) had moderated damage. There was a statistical correlation between retinal toxicity and accumulated doses of less than 1.000 grs. (p: 0.032) as well as with time of use > 5 years (p:0.045) Conclusions: Early alterations of retinal superficial layers help in the detection of early retinal toxicity due to hydroxychloroquine use. OCTSD is a feasible and sensible study in daily medical practice(AU)

Lupus ampolloso: un verdadero reto diagnóstico y terapéutico / Lupus bullous: a true diagnostic and therapeutic challenge

El lupus eritematoso sistémico (LES) esuna enfermedad de tipo autoinmune en donde existen múltiples factores que inducen una respuesta inmunológica no controlada en un individuo que genéticamente está predispuesto, presentándose una variedad de manifestaciones clínicas que muchas veces se convierten en un verdadero reto diagnóstico y terapéutico como es el caso de sus presentaciones en piel. Describiremos a continuación un caso de un paciente género masculino con diagnostico LES con lesiones cutáneas extensas severas con diagnóstico clínico yhistopatológico sugestivo de lupus ampolloso refractarias a tratamiento estándar con corticoterapia e inmunomoduladores con evolución tórpida en su curso, que requiere posteriormente inicio de terapia biológica con Belimumab observándose una remisión clínica significativa de las lesiones y contribuyendo además a disminuir las dosis de corticoides utilizadas desde su ingreso . Se revisaránaspectos en relación del LES en hombres en lo que refiere a epidemiologia, manifestaciones cutáneas, hallazgos histopatológicos, diagnóstico diferencial y opciones terapéuticas actuales.

Systemic lupus erythematosus (SLE) is an autoimmune disease in which multiple factors induce an uncontrolled immune response in an individual who is genetically predisposed, presenting a variety of clinical manifestations that often become a real diagnostic challenge and Therapeutic as in the case of their skin presentations. We will now describe a case of a malepatient with a SLE diagnosis with severe extensive skin lesions with clinical and histopathological diagnosis suggestive of blistering lupus refractory to standard treatment with corticosteroid therapy and immunomodulators with a morphologic evolution in their course, which then requires the initiation of biological therapy with Belimumab observed a significant clinical remission of the lesions and also contributing to decrease the doses of corticosteroids used since their entry. We will review aspects related to SLE in men in terms of epidemiology, cutaneous manifestations, histopathological findings, differential diagnosis and current therapeutic options

Cutaneous Lupus Erythematosus Presenting as Localized Grouped Papules Mimicking Herpes Zoster on the Back

No abstract available.

Lupus eritematoso infantil serie de 6 casos / Lupus Erythematosus in children: a series of six cases

Introducción: El lupus eritematoso infantil incluye las distintas variedades de lupus eritematoso del adulto y además una forma exclusiva de la edad pediátrica: el lupus neonatal. Aunque el lupus eritematoso sistémico, subagudo y discoide pediátricos comparten las características esenciales de las formas correspondientes en los adultos, presentan algunas peculiaridades. Casos: Describimos cinco casos clínicos de lupus infantil, de distintos tipos, dos casos del subtipo discoide, dos subagudos y un ampollar. Discusión: Entre las diferencias más importantes cabe destacar que en el lupus sistémico infantil es más frecuente encontrar nefropatía, fiebre y linfadenopatías al inicio de la enfermedad que en adultos. Durante el desarrollo de la enfermedad es más común el exantema malar. El lupus subagudo es excepcional en la infancia.

Introduction: Systemic lupus erythematosus in children includes adult different varieties plus and exclusive pediatric presentation, neonatal lupus. Case: We describe five cases of SLE in children, two cases of discoid subtype, two subacute and one blistering. Discusion: Although pediatric systemic lupus erythematosus discoid and subacute share the essential features of the corresponding forms in adults, they also have some peculiarities. Among the most frequent are nephropathy, fever and lymphadenopathy at the onset of the disease, whereas, during the development of the disease malar rash is quite common.

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.

Systemic Lupus Erythematosus Presenting as Stevens-Johnson Syndrome: A Case Report / 대한피부과학회지

Acute cutaneous lupus erythematosus (ACLE) on the face is a usual pattern of presentation. However, it can rarely present with a generalized distribution. A hyperacute form of ACLE can mimic Stevens-Johnson syndrome (SJS) and toxic epidermal necrosis (TEN). A 33-year-old man presented with erythematous eroded papules and patches on his head, neck, and upper chest over 2 months. Moreover, he showed hemorrhagic crusted erosions on his lips, and buccal and nasal mucosa, in addition to conjunctival injection. A skin biopsy from his cheek showed a mild degree of vacuolar alteration, thickening of the basement membrane, perivascular and periadnexal lymphohistiocytic infiltration, and stromal mucin deposition. Direct immunofluorescence (DIF) demonstrated IgG and IgM deposits along the basement membrane zone. Laboratory investigations demonstrated pancytopenia, positive antinuclear antibody (ANA), anti-double stranded DNA (anti-dsDNA), and anti-Ro antibodies. The patient was diagnosed with systemic lupus erythematosus (SLE) based on clinical, histological, and laboratory markers of autoimmune disease. We report a rare case of SLE presenting as SJS.