IL-1β and IL-17 in cutaneous lupus erythematous skin biopsies: could immunohistochemicals indicate a tendency towards systemic involvement?

Abstract Background: Only a fraction of patients with cutaneous lupus erythematosus (CLE) will eventually progress toward systemic disease (SLE). Objective: To find inflammatory biomarkers which could predict the progression of cutaneous lupus erythematosus (CLE) into systemic lupus erythematosus (SLE) using immunohistochemical (IHC) assays. Methods: Immunohistochemical markers for cytotoxic, inflammatory, and anti-inflammatory responses and morphometric methods were applied to routine paraffin sections of skin biopsies, taken from lesions of 59 patients with discoid lupus, subacute lupus, and lupus tumidus. For the diagnosis of SLE, patients were classified by both the American College of Rheumatology (ACR-82) and the Systemic Lupus International Collaborating Clinics (SLICC-12) systems. Results: Skin samples from CLE/SLE +patients presented higher expression of IL-1β (ARC-82: p = 0.024; SLICC-12: p = 0.0143) and a significantly higher number of cells marked with granzyme B and perforin (ARC: p = 0.0097; SLICC-12: p = 0.0148). Biopsies from CLE/SLE- individuals had higher expression of IL-17 (ARC-82: p = 0.0003; SLICC-12: p = 0.0351) and presented a positive correlation between the density of granzyme A+and FoxP3+ cells (ARC-82: p = 0.0257; SLICC-12: p = 0.0285) and CD8+ cells (ARC-82: p = 0.0075; SLICC-12: p = 0.0102), as well as between granulysin-positive and CD8+ cells (ARC-82: p = 0.0024; SLICC-12: p = 0.0116). Study limitations: Patients were evaluated at a specific point in their evolution and according to the presence or not of systemic disease. The authors cannot predict how many more, from each group, would have evolved towards SLE in the following years. Conclusions: In this cohort, immunohistochemical findings suggested that patients with a tendency to systemic disease will show strong reactivity for IL-1β, while those with purely cutaneous involvement will tend to express IL-17 more intensely.

IL-1ß and IL-17 in cutaneous lupus erythematous skin biopsies: could immunohistochemicals indicate a tendency towards systemic involvement?

BACKGROUND: Only a fraction of patients with cutaneous lupus erythematosus (CLE) will eventually progress toward systemic disease (SLE). OBJECTIVE: To find inflammatory biomarkers which could predict the progression of cutaneous lupus erythematosus (CLE) into systemic lupus erythematosus (SLE) using immunohistochemical (IHC) assays. METHODS: Immunohistochemical markers for cytotoxic, inflammatory, and anti-inflammatory responses and morphometric methods were applied to routine paraffin sections of skin biopsies, taken from lesions of 59 patients with discoid lupus, subacute lupus, and lupus tumidus. For the diagnosis of SLE, patients were classified by both the American College of Rheumatology (ACR-82) and the Systemic Lupus International Collaborating Clinics (SLICC-12) systems. RESULTS: Skin samples from CLE/SLE+patients presented higher expression of IL-1ß (ARC-82: p=0.024; SLICC-12: p=0.0143) and a significantly higher number of cells marked with granzyme B and perforin (ARC: p=0.0097; SLICC-12: p=0.0148). Biopsies from CLE/SLE- individuals had higher expression of IL-17 (ARC-82: p=0.0003; SLICC-12: p=0.0351) and presented a positive correlation between the density of granzyme A+and FoxP3+ cells (ARC-82: p=0.0257; SLICC-12: p=0.0285) and CD8+ cells (ARC-82: p=0.0075; SLICC-12: p=0.0102), as well as between granulysin-positive and CD8+ cells (ARC-82: p=0.0024; SLICC-12: p=0.0116). STUDY LIMITATIONS: Patients were evaluated at a specific point in their evolution and according to the presence or not of systemic disease. The authors cannot predict how many more, from each group, would have evolved towards SLE in the following years. CONCLUSIONS: In this cohort, immunohistochemical findings suggested that patients with a tendency to systemic disease will show strong reactivity for IL-1ß, while those with purely cutaneous involvement will tend to express IL-17 more intensely.

German Shorthaired Pointer dogs with exfoliative cutaneous lupus erythematosus develop immune-complex membranous glomerulonephropathy.

German Shorthaired Pointer (GSHP) dogs with a UNC93B1 gene mutation develop exfoliative cutaneous lupus erythematosus (ECLE) and kidney disease resembling lupus nephritis in humans. The objective of this study was to characterize the kidney disease by light microscopy, immunofluorescence, and electron microscopy in a population of GSHP dogs with ECLE. Medical records were reviewed, and light microscopy of kidneys from 7 GSHP dogs with a previous histologic diagnosis of ECLE was performed. Immunofluorescence of fresh-frozen kidney from 1 dog and transmission electron microscopy of kidney from that dog and 2 additional dogs were performed. Five of 7 dogs had proteinuria diagnosed by urinalysis or urine protein-to-creatinine ratio. Two of 7 dogs were intermittently hypoalbuminemic, and none were azotemic. Histologic findings included early (2 dogs) to late (5 dogs) membranous glomerulonephropathy characterized by mild-to-severe glomerular capillary loop thickening and tubular proteinosis. In all 7 cases, trichrome staining revealed red granular immune deposits on the subepithelial surface of the glomerular basement membrane. Immunofluorescence revealed strong granular labeling for immunoglobulins and complement protein C3. Electron microscopy demonstrated subepithelial electron-dense immune deposits encircled by the remodeled glomerular basement membrane. These findings are diagnostic of immune-complex membranous glomerulonephropathy and are similar to class V lupus in humans. This cohort of GSHP dogs with ECLE developed immune-complex membranous glomerulonephropathy, which we hypothesize is a manifestation of systemic lupus erythematosus. GSHP dogs with ECLE should undergo clinical evaluation of renal function for early identification and treatment.

Modulation of Immune Cells as a Therapy for Cutaneous Lupus Erythematosus.

Cutaneous lupus erythematosus (CLE) is an autoimmune disorder like systemic lupus erythematosus (SLE). Both SLE and CLE characterize autoantibody secretion and immune cell recruitment. In particular, CLE can be divided into three more frequent types, varying in the severity of the skin lesions they present. The role of type I IFN was shown to be one of the leading causes of the development of this pathology in the skin. Different treatments have been developed and tested against these different variants of CLE to decrease the increasing levels of CLE in humans. In this article, a literature revision discussing the similarities between SLE and CLE is carried out. In addition, new advances in understanding the development of CLE and the leading treatments being evaluated in animal models and clinical trials are reviewed.

Chilblain lupus with nail involvement: a case report

Chilblain lupus is a rarely manifested variant of chronic lupus. Its appearance can be sporadic or hereditary associated with an autosomal dominant mutation. The diagnosis is clinical and histopathological. The case is presented of a patient with systemic lupus erythematosus presenting with chilblain lupus and nail involvement, despite the use of antimalarials and immunomodulators.

La perniosis lúpica es una variante del lupus crónico que se manifiesta con poca frecuencia, su aparición puede ser esporádica o hereditaria, asociada con una mutación autosómica dominante, en tanto que su diagnóstico es clínico e histopatológico. Se reporta el caso de una mujer con lupus eritematoso sistémico con manifestación de perniosis lúpica y compromiso ungueal, a pesar del uso de antimaláricos e inmunomoduladores.

Areata-Like Lupus as a Clinical Manifestation of Cutaneous Lupus Erythematosus.

Introduction: Lupus erythematosus (LE) is a chronic autoimmune disease that frequently causes hair loss and scalp lesions. Hair loss can be scarring and nonscarring, diffuse, or patchy. The nonscarring patchy alopecia is usually related to systemic LE (SLE) and may simulate alopecia areata (AA), reason why it is named areata-like lupus. Our case was diagnosed with areata-like lupus but did not meet criteria for SLE. Case Report: A 63-year-old woman presented with irregular nonscarring patchy alopecia in the temporal and frontoparietal scalp. Trichoscopy showed exclamation mark hairs, vellus hairs, and sparse yellow dots. Histology revealed epidermal vacuolar interface dermatitis, lymphohistiocytic infiltrate around the bulbs of anagen follicles, and eccrine glands. Direct immunofluorescence showed deposits of C3, IgA, and IgG in the basement membrane zone. Discussion: Patients with cutaneous LE can also manifest as nonscarring patchy alopecia that is clinically similar to AA, despite the absence of systemic manifestations. Areata-like lupus is secondary to the lupus autoimmune infiltrate that affects the skin including the hair follicles. Trichoscopy, histology, and direct immunofluorescence are important to differentiate this form of alopecia from AA, which is believed to have a higher incidence in lupus patients.

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)

Lupus erythematosus tumidus in childhood treated with antimalarials / Lupus eritematoso tumidus en la infancia tratado con antimalárico

Abstract Lupus erythematosus tumidus is a rare dermatosis. It is considered a subtype of chronic cutaneous lupus erythematosus of uncertain pathogenesis, favorable prognosis and rare association with systemic lupus erythematosus. Clinically, it manifests as urticarial-like plaques in photo exposed areas, mainly affecting adults, being extremely rare in pediatric age. Herein, we present two cases of six and nine-year-old male patients with clinical and histological characteristics typical of lupus erythematosus tumidus and poor response to first-line treatment (topical, intralesional steroids and topical calcineurin inhibitors); therefore, it was decided to start systemic therapy with antimalarials, obtaining a very good response.

Resumen El lupus eritematoso tumidus es una dermatosis poco frecuente. Es considerada una variante del lupus eritematoso cutáneo crónico, de patogénesis incierta, pronóstico favorable y rara asociación con lupus eritematoso sistémico. Clínicamente, se manifiesta como placas de aspecto urticarial en zonas fotoexpuestas, que principalmente afectan a los adultos, siendo extremadamente rara en edad pediátrica. A continuación presentamos dos casos de pacientes de sexo masculino de seis y nueve años, con características clínicas e histológicas típicas de lupus eritematoso tumidus y poca respuesta al tratamiento de primera línea (esteroides tópicos, intralesionales e inhibidores de calcineurina tópica), por lo que se decidió iniciar manejo sistémico con antimalárico, obteniendo muy buena respuesta terapéutica.

Necrólisis epidérmica tóxica y lupus eritematoso sistémico / Toxic epidermal necrolysis and systemic lupus erythematosus

Resumen El lupus eritematoso sistémico con manifestación tipo necrólisis epidérmica tóxica es una entidad descrita recientemente y cada vez aparecen más reportes en la literatura. Se describe el caso de una paciente de 15 años con lupus eritematoso sistémico quien presentó una necrólisis epidérmica tóxica extensa, cuyas lesiones iniciales eran tipo eritema multiforme. Se discute el caso a la luz de la literatura actual sobre esta nueva entidad.

Abstract Toxic epidermal necrolysis (TEN)-like systemic lupus erythematosus is a recently described entity and more cases are being published in the literature today. We describe the case of a 15-year old patient with systemic lupus erythematosus who developed TEN that initially started with erythema multiforme (EM)-like lesiones. We discuss this case according to the published literature on this new entity.

Chronic cutaneous lupus erythematosus presenting as atypical acneiform and comedonal plaque: case report and literature review.

Introduction Chronic cutaneous lupus erythematosus (CCLE) usually presents as characteristic erythematous patches and infiltrated coin-shaped plaques. However, there are some atypical clinical variants that may mimic other dermatological conditions. Haroon et al. reported in 1972 an unusual presentation of CCLE with hypertrophic follicular scars seen in acne vulgaris. Acneiform presentation is one of the most rarely reported and one of the most confusing, as it resembles a very common inflammatory skin disease. A brief review of the literature using PubMed found only nine other reports. Case report A 32-year-old woman presented with two-year pruritic infiltrated acneiform and comedonal eruption on the right chin treated as acne with isotretinoin without improvement. On examination the patient presented with erythematous-infiltrated plaque, papules, open comedones, pitting scars and hypopigmented atrophic scars on the right chin area and scalp hair loss. An incisional skin biopsy on the chin and scalp lesions was performed and the anatomopathological and immunofluorescence exam showed findings that are consistent with CCLE. Additional tests ruled out systemic involvement. The patient was treated with prednisone and chloroquine diphosphate with great improvement. After four years the lesion is stable, with some scarring. Discussion In a literature review we found nine other cases of acneiform presentation of lupus erythematosus: Three cases were systemic lupus erythematosus (SLE) and seven others were diagnosed as CCLE (including our patient). All three patients who had SLE tested positive for antinuclear antibodies (ANA), and only one patient with CCLE, had a low titer of positive ANA (1:80). Ages varied from 24 to 60 years old, with a median of 32 years old, the same as our patient's age and consistent with the literature. Seven were females and three were males, with a ratio of 2.3:1. Most cases, such as our patient, showed acneiform lesions mainly on the face, a common site of typical CCLE. The present case and literature review illustrates the need to expand the differential diagnosis of atypical acneiform and comedonal lesions. CCLE should be considered especially in a localized lesion, which can be itchy and does not improve with conventional treatment for acne vulgaris.

Classifying discoid lupus erythematosus: background, gaps, and difficulties.

To inform our ongoing efforts to develop defining features to be incorporated into a novel set of classification criteria for discoid lupus erythematosus (DLE), we conducted a literature review using the Ovid MEDLINE database. A search was performed to identify studies reporting criteria used to distinguish DLE from other cutaneous lupus erythematosus subtypes. We examined which clinical, histopathologic, and serologic features have data to support their use as effective features in distinguishing DLE from other potential disease mimickers and cutaneous lupus subsets. Through our search, we were also able to identify gaps that exist in the literature which can inform future directions for research endeavors. We found that localization of lesions, characteristic features of damage, and the absence of high titer Ro/SSA antibody seem most effective in differentiating DLE from other cutaneous lupus erythematosus subtypes. Histopathologic features and class of immunoreactant deposition appear to be less helpful.

Lupus cutáneo inducido por adalimumab / Adalimumab-induced cutaneous lupus

La terapia antifactor de necrosis tumoral se ha convertido en los últimos años en uno de los pilares fundamentales para el tratamiento de la artritis reumatoide. El adalimumab es un anticuerpo monoclonal humanizado empleado en el tratamiento de la artritis reumatoide. Se describe un caso de lupus cutáneo inducido por adalimumab.

Tumor necrosis factor inhibitors have become one of the most important treatments of rheumatoid arthritis. Adalimumab is a monoclonal antibody used for the treatment of this condition. A case is described of adalimumab induced cutaneous lupus.

Classifying discoid lupus erythematosus: background, gaps, and difficulties.

To inform our ongoing efforts to develop defining features to be incorporated into a novel set of classification criteria for discoid lupus erythematosus (DLE), we conducted a literature review using the Ovid MEDLINE database. A search was performed to identify studies reporting criteria used to distinguish DLE from other cutaneous lupus erythematosus subtypes. We examined which clinical, histopathologic, and serologic features have data to support their use as effective features in distinguishing DLE from other potential disease mimickers and cutaneous lupus subsets. Through our search, we were also able to identify gaps that exist in the literature which can inform future directions for research endeavors. We found that localization of lesions, characteristic features of damage, and the absence of high titer Ro/SSA antibody seem most effective in differentiating DLE from other cutaneous lupus erythematosus subtypes. Histopathologic features and class of immunoreactant deposition appear to be less helpful.

[Cutaneous lupus erythematosus, a multidimensional entity]. / Lupus eritematoso cutáneo, una entidad multidimensional.

Skin lesions caused by systemic lupus erythematosus are among the most frequent manifestations of this disease. These lesions show great variability in both their clinical and histological expression, making their understanding and study difficult. Patients presenting with cutaneous lupus do not necessarily have serious systemic complications, but they do have significant morbidity from impact on quality of life given the extent of the lesions, chronic tendency, and the risk of scarring; hence the importance of establishing a fast and effective treatment. This paper addresses the different varieties of specific injuries attributed to lupus erythematosus, correlation with systemic activity, quality of life, and the treatments available.

Las lesiones en la piel producidas por el lupus eritematoso sistémico constituyen una de las manifestaciones más frecuentes de esta enfermedad. Estas lesiones muestran una gran variabilidad, tanto en su expresión clínica como histológica, lo que dificulta su comprensión y estudio. Los pacientes que cursan con lupus cutáneo no necesariamente tienen complicaciones sistémicas graves, pero sí importante morbilidad por afección en la calidad de vida dada la extensión de las lesiones, tendencia a la cronicidad y el riesgo de cicatrices; de ahí la importancia de instaurar un tratamiento rápido y eficaz. El presente trabajo aborda las diferentes variedades de lesiones específicas atribuidas al lupus eritematoso, su correlación con actividad sistémica, calidad de vida y los tratamientos disponibles.

Apoptosis in chronic cutaneous lupus erythematosus, discoid lupus, and lupus profundus.

INTRODUCTION: Lupus erythematosus is a multisystemic disease that is characterized by autoantibody production and immune complex deposition in such tissues as the mucosa, joints, the central nervous system, and skin. Cutaneous lupus erythematosus is categorized as acute, subacute, and chronic. Chronic cutaneous lupus erythematosus comprises discoid lupus erythematosus (DLE) and lupus profundus (LP). AIM: To analyze the expression of proapoptotic molecules in patients with lupus erythematosus discoid and lupus profundus. MATERIAL AND METHODS: Descriptive study, the study groups comprised 10 cases of LP and 10 cases of DLE, and a control. Skin samples of cases and controls were processed for immunohistochemistry and by TUNEL technique. The database and statistical analysis was performed (statistical test X(2)) SPSS (Chicago, IL, USA). RESULTS: Apoptotic features were broadly distributed along the skin biopsies in epidermal keratinocytes as well as at dermis. By immunohistochemistry the expression of Fas receptor and Fas-L was higher in the skin of lupus patients compared with controls. We also noted differences in Fas-L, -Fas, and -Bax proteins expression intensity in discoid lupus erythematosus patients in the epidermis, and hair follicles. CONCLUSIONS: Fas and Fas-L are expressed similarly in LP and DLE.

Associação entre o polimorfismo rs7700944 no gene TIM-4 e artrite reumatoide em Zahedan, sudeste do Irã / Association between the rs7700944 polymorphism in the TIM-4 gene and rheumatoid arthritis in Zahedan, southeast Iran

INTRODUÇÃO: Recentemente, relatou-se uma associação entre artrite reumatoide (AR) e a variante rs7700944 G>A nos domínios imunoglobulina e mucina de células T (TIM-4). OBJETIVO: Investigar o impacto desse polimorfismo na suscetibilidade a AR em uma amostra da população iraniana. PACIENTES E MÉTODOS: Este estudo caso-controle foi conduzido em 120 pacientes com AR e 120 indivíduos saudáveis. O polimorfismo rs7700944 do gene TIM-4 foi determinado usando-se o ensaio tetra amplification refractory mutation system-polymerase chain reaction (T-ARMS-PCR). RESULTADOS: Não se observou diferença significativa quanto ao polimorfismo rs7700944 do gene TIM-4 entre os pacientes com AR e os indivíduos saudáveis. Nas mulheres, não houve associação significativa quanto ao polimorfismo rs7700944 do gene TIM-4 nos dois grupos. Nos homens, o genótipo GA+AA, em comparação ao GG, aumentou o risco para AR (OR = 5,15; IC 95% = 1,30-20,48; P = 0,020). Além disso, os resultados mostraram que o alelo rs7700944 A aumentou o risco para AR (OR = 4,39; IC 95% = 1,43-13,54; P = 0,009). CONCLUSÃO: Nossos resultados não confirmam a existência de associação entre AR e o polimorfismo rs7700944 do gene TIM-4. Uma interação entre esse polimorfismo e sexo sugere uma associação sexo-específica entre AR e esse polimorfismo de nucleotídeo único, que ainda requer elucidação.

INTRODUCTION: Recently, an association between rheumatoid arthritis (RA) and the rs7700944 G>A variant in the T-cell immunoglobulin and mucin domains 4 (TIM-4) has been reported. OBJECTIVE: The present study aimed at investigating the impact of that polymorphism on susceptibility to RA in a sample of the Iranian population. Patients and methods: This case-control study was conducted on 120 patients with RA and 120 healthy subjects. The rs7700944 polymorphism in the TIM-4 gene was determined using tetra amplification refractory mutation system-polymerase chain reaction (T-ARMS-PCR) assay. RESULTS: No significant difference was observed regarding the rs7700944 polymorphism of the TIM-4 gene between patients with RA and normal individuals. In females, no significant association was found between the groups concerning the rs7700944 polymorphism of the TIM-4 gene. In males, the GA+AA genotype increased the risk of RA in comparison with the GG genotype (OR = 5.15, 95% CI = 1.30-20.48, P = 0.020). Furthermore the results showed that the rs7700944 A allele increased the risk of RA (OR = 4.39, 95% CI = 1.43-13.54, P = 0.009). CONCLUSION: Our results do not support an association between the rs7700944 polymorphism of the TIM-4 gene and RA. An interaction between this polymorphism and sex suggests a sex-specific association between this single nucleotide polymorphism and RA, which remains to be fully elucidated.

Vasculite mesentérica em paciente com lúpus eritematoso sistêmico juvenil / Mesenteric vasculitis in a juvenile systemic lupus erythematosus patient

A vasculite mesentérica lúpica (VML) é uma rara causa de dor abdominal aguda. Há poucos relatos de caso demonstrando VML em adultos e, particularmente, em crianças e adolescentes. No entanto, para o nosso conhecimento, a prevalência dessa grave vasculite em uma população pediátrica com lúpus ainda não foi estudada. Portanto, dados de 28 anos consecutivos foram revisados e incluídos 5.508 pacientes em seguimento no Hospital da Faculdade de Medicina da Univesidade de São Paulo (FMUSP). Identificamos 279 (5,1%) casos que preencheram critérios de classificação diagnóstica do American College of Rheumatology para lúpus eritematoso sistêmico (LES) e um (0,4%) desses apresentou VML. Este paciente recebeu diagnóstico de LES aos 11 anos de idade. Aos 13 anos foi hospitalizado com dor abdominal difusa e aguda, náuseas, vômitos biliosos, distensão e rigidez abdominal, com descompressão brusca positiva. O paciente foi prontamente submetido à laparotomia exploradora, identificando isquemia intestinal segmentar, com edema de parede intestinal e aderências. Foi realizada ressecção parcial de intestino delgado, com lise das aderências e pulsoterapia com metilprednisolona. A análise histopatológica identificou arterite de vasos mesentéricos. Após 13 dias, apresentou recorrência de dor abdominal difusa intensa, sendo novamente submetido à laparotomia exploradora, identificando obstrução em intestino delgado por aderências, com gangrena intestinal. Nova ressecção intestinal foi realizada, além de pulsoterapia com metilprednisolona e infusão de imunoglobulina. Portanto, VML é uma rara e grave manifestação abdominal na população com lúpus pediátrico, e pode ser a única manifestação de atividade da doença. Além disso, este estudo reforça a importância do diagnóstico precoce e do tratamento imediato.

Lupus mesenteric vasculitis (LMV) is a rare cause of acute abdominal pain. Few cases of LMV have been reported in adults, children and adolescents. However, to our knowledge, the prevalence of that severe vasculitis in a pediatric population with lupus is yet to be studied. This study reviewed data from 28 consecutive years and included 5,508 patients being followed up at the hospital of the Faculdade de Medicina of the Univesidade de São Paulo (FMUSP). We identifi ed 279 (5.1%) patients meeting the American College of Rheumatology classifi cation criteria for the diagnosis of systemic lupus erythematosus (SLE), one of whom (0.4%) had LMV. That male patient was diagnosed with SLE at the age of 11 years. At the age of 13 years, he was hospitalized with diffuse and acute abdominal pain, nausea, bilious vomiting, abdominal distension, rebound tenderness, and abdominal muscle guarding. The patient underwent laparotomy immediately, and segmentary intestinal ischemia with intestinal wall edema and adhesions were identifi ed. Partial small bowel resection with lysis of the adhesions was performed, as were pulses of intravenous methylprednisolone. The histopathologic analysis evidenced mesenteric arteritis. After 13 days, the diffuse and intense abdominal pain recurred, and the patient underwent a new laparotomy, during which adhesive small bowel obstruction with intestinal gangrene was identifi ed. New intestinal resection was performed, and the patient received pulses of intravenous methylprednisolone and infusion of immunoglobulin. Thus, LMV is a rare and severe abdominal manifestation of the pediatric population with lupus, and can be the only manifestation of disease activity. In addition, this study stresses the importance of the early diagnosis and immediate treatment.

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.

Primary cicatricial alopecias: a review of histopathologic findings in 38 patients from a clinical University Hospital in Sao Paulo, Brazil

BACKGROUND: Scarring alopecias are classified into primary and secondary types according to the initial site of inflammation. In primary scarring alopecias, the hair follicle is the main target of destruction; the term secondary cicatricial alopecia implies that follicular destruction is not the primary pathologic event. AIMS: To review the histopathologic diagnoses of cases of cicatricial alopecia in order to classify them according to the North American Hair Research Society. PATIENTS AND METHODS: Patients with biopsy specimens diagnosed as cicatricial alopecia seen from 2000 to 2005 at the Dermatologic Department of Hospital das Clinicas, São Paulo University Medical School had hematoxylin and eosin, Periodic acid-Schiff and Weigert stained slides reevaluated and sub-typed into different primary cicatricial alopecias. RESULTS: Thirty-eight cases of primary cicatricial alopecias were reclassified as: chronic cutaneous lupus (17), lichen planus pilaris (4), pseudopelade of Brocq (12), folliculitis decalvans (3), dissecting folliculitis (1), and non-specific scarring alopecia (1). In our cases, the methods employed allowed an accurate diagnosis in 12 of 13 cases (92.3 percent) previously classified as non-specific cicatricial alopecias. CONCLUSIONS: Even in the late, pauci or non-inflammatory phases, an approach with systematic evaluation of a constellation of criteria in routine hematoxylin and eosin stain, Periodic acid-Schiff and Weigert stain allowed for a more accurate diagnosis of cicatricial alopecias.

Lupus eritematoso cutáneo subagudo (LECSA): a propósito de 17 casos con anticuerpo anti-Ro positivo / Subacute cutaneous lupus erythematosus (SCLE): 17 patients with anti-Ro antibodies

Se presentan, analizan y comparan con otras series los hallazgos dermatológicos, clínicos, histopatológicos e inmunológicos de 17 casos de lupus eritematoso cutáneo subagudo (LECSA) con anticuerpos (Ac) anti-Ro positivos.Las manifestaciones dermatológicas de nuestros pacientes fueron lesiones papuloescamosas policíclicas, más frecuentes que las psoriasiformes. Se localizaban preferentemente en miembros superiores, dorso y zonas de exposición sola. Presentaban un borde eritematovesiculoso bien delimitado. Otras lesiones adoptaron la forma de reloj de arena. Estas manifestaciones fueron recurrentes con períodos de actividad y calma. También fueron relevantes la fotosensibilidad, la hipopigmentación y, en algunos casos, discreta atrofia. Histopatológicamente lo más destacable fue la degeneración vacuolar de la capa basal de queratinocitos y la ausencia de hiperqueratosis folicular, diferenciándose del lupus eritematoso discoide crónico (LEDC). Las restantes lesiones se parecían a las del lupus eritematosos sistémico (LES), pero con menor intensidad. Hubo poco engrosamiento de la menbrana basal PAS positiva, y el infiltrado linfocitario subpapilar y perivascular fue escaso. En algunos casos se demostró edema dérmico alcian blue positivo, poniendo de manifiesto la presencia de mucina. A diferencia de lo relatado por otros autores, encontramos discreta atrofia en 4 pacientes. Lo más significativo de los exámenes inmunológicos fue la presencia del Ac anti-Ro en el 100% de los enfermos y el anti-La solo en el 17,6%. Fue criterio de inclusión para esta serie tener anti-Ro positivo con anti-ADN y anti-Sm negativos. El FAN fue positivo en el 70%. Con respecto a las manifestaciones clínicas generales, se observaron artritis/artralgias en el 100% de los casos. Las lesiones fueron simétricas, no erosivas ni deformantes. Un solo enfermo tuvo pleuresia y glomerulonefritis crónica difusa...

Clinical, histopathologic, and immunological findings in 17 patients with subacute cutaneous lupus erythematosus (SCLE) and Roantibodies are described. Skin manifestations consisted of recurrent, polycyclical, circumscribed papulosquamous, psoriasiform, or hourglass-like lesions with vesiculoerythematous borders, mainly on the upper limbs, back, and sun-exposed areas. Photosensitivity, hypopigmentation, and occasionally mild atrophy were also noted. Histopathologic features included vacuolar degenerative changes involving keratinocyte basement membrane. Unlike chronic discoid lupus erythematosus lesions, follicular hyperkeratosis was absent. Although other characteristics were similar to those seen in systemic lupus erythematosus patients, specimens from SCLE showed less basement membrane thickening and inflammatory cell infiltrates. A few samples revealed positive alcian blue staining for dermal mucin. Only four patients had moderateatrophy. Since only Ro-positive, and DNA- and Sm-negative patients were assessed, immunological studies showed Ro antibodies in 100%of cases and La antibodies in 17.6%. FAN measurements using rat liver and Hep-2 cells were positive in 70% of patients. SCLE was associated with symmetrical, non-erosive, and non-deforming arthritis/arthralgia in all patients, vasculitis in three (17.8%), Raynaud’s syndrome in two (11.8%), pleuritis and chronic glomerulonephritis in one, and panniculitis in one. Ro antibody screening tests were reviewed.