An unusual case of late-onset systemic lupus erythematosus presenting with digital gangrene

@#Digital gangrene is an uncommon initial manifestation of systemic lupus erythematosus (SLE) and rarely in late-onset disease. This case presents a 50-year-old woman who developed digital gangrene and was subsequently diagnosed with SLE. Early treatment with immunosuppression and anticoagulation halted the progression of the digital ischemia. This case highlights that late-onset SLE, often described as having a more benign and insidious course, can also present with catastrophic limb-threatening manifestations.

TAFRO syndrome mimicking systemic lupus erythematosus: Case report and literature review

TAFRO syndrome is a very rare disease, with less than 100 cases reported in the literature. It is classified as a type of idiopathic multicentric Castleman disease, but it has clinical, paraclinical, and histopathological characteristics that differentiate between TAFRO and idiopathic forms of Castleman disease not otherwise specified. However, it is a challenging exclusion diagnosis. TAFRO syndrome is characterized by systemic inflammatory involvement, often severe, which can present with kidney failure, and become a severe disease with a high mortality rate. The clinical manifestations of TAFRO can be confused with hematology malignancies or various autoimmune diseases. Although there are some reports of TAFRO syndrome associated with autoimmune compromise, there is no published consensus for the diagnosis or treatment. The case presented is a patient who meets the criteria to be classified as SLE, and with manifestations with significant clinical involvement, but with no improvement with standard treatment. It was found that the patient's systemic involvement was due to TAFRO, and that therefore the TAFRO syndrome could simulate SLE, something previously not described in the literature.

El síndrome TAFRO es una enfermedad muy poco común, con menos de 100 casos reportados en la literatura. Se clasifica como un tipo de enfermedad de Castleman multicéntrica idiopática, pero tiene características clínicas, paraclínicas e histopatológicas que permiten diferenciarla de las formas de la enfermedad Castleman idiopática no clasificadas de otra manera; sin embargo, es un diagnóstico de exclusión difícil de hacer. El síndrome TAFRO se caracteriza por compromiso inflamatorio sistémico, en muchas ocasiones severo, que puede presentarse con falla renal y convertirse en una enfermedad grave, con una alta tasa de mortalidad. Las manifestaciones clínicas de TAFRO pueden confundirse con neoplasias hematológicas o varias enfermedades autoinmunes. En la literatura existen algunos reportes de síndrome TAFRO asociados con compromiso autoinmune, pero no se ha publicado un consenso para su diagnóstico ni para su tratamiento. El caso que se presenta es un paciente que cumple con los criterios para ser clasificado como LES, que tenía manifestaciones con gran compromiso clínico, pero sin mejoría con el tratamiento estándar. Se encontró que el compromiso sistémico del paciente era por TAFRO y que, por lo tanto, el síndrome TAFRO podría simular LES, algo no descrito previamente en la literatura.

Non-healing ulcers as an atypical presentation of Lupus Vulgaris in an adult Filipino: A case report

@#Cutaneous tuberculosis (TB) occurs rarely, comprising only approximately 1% of all extrapulmonary TB cases worldwide. This report presents an atypical clinical manifestation of Lupus vulgaris, the most common form of cutaneous TB. Typically, Lupus vulgaris presents as chronic erythematous plaques over the head and neck area. The patient, a 24-year-old male with limited support and financial resources, presented with chronic painful, non-healing ulcerated lesions on his left upper extremity. Diagnostic tests specific to TB infection, including culture, all turned out negative for TB. No improvement in the lesions was noted with antibacterial and antifungal therapies. Clinical course of the condition and histopathologic findings, though non-specific to TB, became the basis for diagnosis and treatment. Patient currently is on the 3rd month of anti-Koch’s treatment with noted gradual improvement in the character and appearance of the lesions. Lupus vulgaris could appear as ulcers and over the extremities though much less common, posing challenges in both diagnosis and treatment. Malignant transformation could develop if left untreated. Thus, careful and thorough examination of the patient and diligent follow-up and re-evaluation of lesions while considering the patient’s values, concerns and financial capacity, were necessary in successfully addressing both the biomedical and psychosocial aspect of the illness.

Lupus miliaris disseminatus faciei: when a rare skin disease involves the eyelid / Lupus miliaris disseminatus faciei: quando uma rara dermatose acomete a pálpebra

ABSTRACT The authors present a case of lupus miliaris disseminatus faciei , a rare skin disease of unknown etiology, which may cause unaesthetic scarring due to its difficult treatment. The histopathological examination of epithelioid granulomas with caseating necrosis, together with the clinical features, are important for diagnosis and early treatment with better results. Despite difficult and unsatisfactory treatment, there are ongoing studies on therapy to improve aesthetic and social impairment. This case report describes an initial misdiagnosis delaying appropriate treatment, and highlights the value of physical examination and clinical judgment for another pathological examination, whenever necessary, aiming at better treatment outcomes in daily practice.

RESUMO Os autores apresentam um caso de lupus miliaris disseminatus faciei , uma dermatose rara, de etiologia desconhecida, que pode deixar cicatrizes não estéticas, pela dificuldade de tratamento. O exame histopatológico de granulomas compostos por células epitelioides, com necrose caseosa, e as características clínicas, são importantes para o diagnóstico e tratamento precoce, com melhores resultados. Apesar do tratamento difícil e insatisfatório, há estudos em andamento sobre terapias para melhorar o comprometimento estético e social. Este relato de caso descreve um diagnóstico inicial errôneo, que atrasou o tratamento adequado, e destaca o valor do exame físico e raciocínio clínico para solicitar outro exame anatomopatológico, quando necessário, de forma a obter melhores desfechos com o tratamento, na prática diária.

Paciente con dermatitis y artritis durante sus embarazos / A patient with dermatitis and arthritis during her two pregnancies

Paciente femenina que presentó durante sus dos embarazos eritema nodoso, úlceras nasales y artritis. Debido a la persistencia del eritema nodoso después del segundo parto, se practicó determinación de ANA y anti-DNA ­ IF, resultando este último positivo, por lo que se plantea el diagnóstico de lupus eritematoso y es referida al Centro Nacional de Enfermedades Reumáticas, (CNER) donde se le practicó biopsia de piel que resultó compatible con lepra: borderline o dimorfa (BB-BL) en estado reaccional tipo II (eritema nodoso leproso: ENL). Se discuten algunos de aspectos de la lepra haciendo especial énfasis en síntomas que presentó la paciente y la exacerbación de los mismos durante los embarazos, posiblemente en relación con algunos cambios inmunológicos adaptativos que son generados para mantener la tolerancia materno fetal(AU)

Female young patient with erythema nodosum, nasal ulcers and arthritis during her two pregnancies. Due to the persistence of the erythema nodosum after the second delivery, ANA and anti-DNA - IF determination were done, the latter being positive, and she was diagnosed with lupus erythematosus and was referred to the National Center for Rheumatic Diseases, where skin biopsy showed to be compatible with leprosy: borderline or dimorphic (BB-BL) in type II reactional state (leprous erythema nodosum: LEN). Some aspects of leprosy are discussed with special emphasis on symptoms that the patient presented and their exacerbation during pregnancies, possibly in relation to some adaptive immunological changes that was generated to maintain maternal fetal tolerance(AU)

Tuberculosis cutánea a forma de lupus vulgar causada por vacuna de bacilo Calmette-Guérin. A propósito de un caso pediátrico / Lupus vulgaris as a form of cutaneous TB caused by the Bacille Calmette-Guérin vaccine. A pediatric case / Tuberculose cutânea sob forma de lúpus vulgar causada pela vacina de bacilo Calmette- Guérin. A propósito de um caso pediátrico

La tuberculosis cutánea es una enfermedad infecciosa crónica y poco frecuente ocasionada por Mycobacteriun tuberculosis. Existen varias formas de la infección cutánea, siendo el lupus vulgar la más frecuente y común en los países industrializados. Las variantes clínicas dependerán de la vía de llegada del bacilo a la piel, del estado inmunológico del paciente y del medio ambiente. En el lupus vulgar, además de M. tuberculosis puede estar implicado M. bovis y menos frecuentemente el bacilo de Calmette y Guérin. Esta entidad presenta una evolución crónica, es de carácter progresivo y es rara de ver en niños. Se presenta el caso de una niña en la cual se diagnosticó una forma de tuberculosis cutánea denominada lupus vulgar provocada por el bacilo de Calmette y Guérin. Caso clínico: niña de 10 años que presentaba lesiones cutáneas de diferentes características, de evolución tórpida y asintomáticas; la de mayor tamaño se localizaba en la cara externa del deltoides derecho en forma de placa eritematosa de 20 por 10 cm con bordes sobreelevados presentando en su centro micropápulas que coalescen dejando una zona de atrofia central. Esta había comenzado en etapa neonatal luego de la vacunación por el bacilo de Calmette y Guérin. Desde hacía tres años presentaba una segunda lesión más pequeña en cara externa de muslo derecho de similares características y otras de carácter múltiple en dorso con forma de micropápulas rojo vinosas. Los exámenes de valoración general fueron normales, al igual que la radiografía de tórax y de los miembros. El test de la tuberculina mostró una induración de 15 mm y las baciloscopias fueron negativas. La biopsia de la lesión de piel del deltoides mostró granulomas caseosos y las técnicas moleculares diagnosticaron Mycobacterium bovis atenuado. Con ese diagnóstico, recibió tratamiento antituberculoso por diez meses con buena tolerancia y con mejoría de las lesiones.

Cutaneous TB is a rather unusual chronic infectious disease caused by Mycobacteriun tuberculosis. There are several forms of cutaneous infection, lupus vulgaris being the most frequent and ordinary one in developed countries. Clinical variations will depend on the site of infiltration of the skin by the bacillus, the patient's immune condition and the environment. In the case of lupus vulgaris, apart from Mycobacteriun tuberculosis, M. bovis may be involved, and the Bacille Calmette Guérin involvement is less frequent. The latter presents a chronic evolution, being progressive and unusual in children. The study presents the case of a girl who was diagnosed with a form of cutaneous tuberculosis called lupus vulgaris caused by the Bacille Calmette-Guérin vaccine. Clinical case: a 10 year old girl who evidenced skin lesions of different kinds, of slow evolution and asymptomatic: the largest one was in the external part of the right deltoid muscle as an erythematous plaque of 20 by 10 cm with higher borders evidencing micro papules in the center that coalesce leaving a central atrophy zone. The latter had started in the neonatal stage after the Bacille Calmette-Guérin vaccine. For three years she had evidenced a second smaller lesion in the outer face of the right thigh of similar characteristics and other multiple lesions in the back with the shape of vinous red micro papules. Assessment tests were generally normal, as well as the chest and limbs X-ray. The tuberculin test evidenced a 15 mm induration and smears were negative. The deltoid muscle skin lesion biopsy showed caseous granulomas and molecular techniques diagnosed attenuated Mycobacteriumbovis. Given that diagnose the patient received anti-TB treatment during 10 months, tolerance being good and the lesions improved.

A tuberculose cutânea é uma doença infecciosa crônica e pouco frequente causada pelo Mycobacteriun tuberculosis. Existem várias formas de infecção cutânea, sendo o lúpus vulgar a mais frequente e comum nos países industrializados. As variantes clínicas dependerão da via de entrada do bacilo à pele, do estado imunológico do paciente e do ambiente. No lúpus vulgar, além do M. tuberculosis podem estar implicados o M. bovis e mais raramente o bacilo de Calmette-Guérin. Esta entidade apresenta uma evolução crônica, é progressiva e é rara em crianças. Apresenta-se o caso de uma paciente de sexo feminino com 10 anos que foi diagnosticada com uma forma de tuberculose cutânea denominada lúpus vulgar provocada pelo bacilo de Calmette-Guérin. Caso clínico: paciente de sexo feminino com 10 anos que apresentava lesões cutâneas de diferentes características, com evolução letárgica e assintomática; a maior lesão estava localizada na face externa do deltoide direito sob a forma de placa eritematosa de 20 x 10 cm com bordes sobrelevados apresentando no centro micropápulas que coalescem deixando una zona de atrofia central. Esta lesão começou na etapa neonatal depois da vacinação com o bacilo de Calmette-Guérin. Desde 3 anos apresentava uma segunda lesão menor na face externa da coxa direita com características similares e outras múltiplas no dorso com forma de micropápulas com coloração de manchas em vinho do Porto. Os exames de avaliação geral foram normais, bem como as radiografias de tórax e de membros. O teste da tuberculina mostrou uma induración de 15 mm e as baciloscopias foram negativas. A biopsia da lesão de pele dos deltoides mostrou granulomas caseosos e as técnicas moleculares diagnosticaram Mycobacterium bovis atenuado. Com esse diagnóstico recebeu tratamento antituberculoso por 10 meses com boa tolerância e melhoria das lesões.

Gastrointestinal Vasculitis in Systemic Lupus Erythematosus: A successful treatment with Belimumab

Introduction@#Gastrointestinal (GI) vasculitis among systemic lupus erythematosus (SLE) patients is a rare manifestation confounded with non-specific clinical symptoms and laboratory parameters which delay diagnosis leading to increase morbidity and mortality. This is a case report of a patient with of GI vasculitis who was successfully treated with belimumab@*Case presentation@#We describe a case of a Chinese descent, 24-year-old female who was diagnosed with SLE presenting as malar rash, arthritis, intermittent fever, positive antinuclear antibody (ANA), high titer anti double-stranded DNA and low serum complement. She had gastrointestinal manifestations of colicky abdominal pain, vomiting and diarrhea, with computed tomography scan findings of diffuse mucosal edema involving both small and large intestines, with “target” sign strongly suggestive of GI vasculitis. She initially showed good response to methylprednisolone pulse therapy, but with recurrence of abdominal pain after three months. Repeat abdominal CT showed perforated viscus at the ileal segment with pneumoperitoneum, requiring emergency exploratory laparotomy and colostomy placement. Subsequent clinical course was marked by intolerance to oral steroid requiring regular administration of high dose intravenous steroids. Belimumab was started months after surgery and maintained for two years now. She was successfully shifted to oral prednisone, tapered to lowest dose and underwent uneventful intestinal reanastomosis with closure of colostomy.@*Discussion@#Gastrointestinal (GI) vasculitis is one of the most serious gastrointestinal complications SLE presenting as acute abdominal pain. Our patient was diagnosed based on abdominal CT scan which led to an accurate diagnosis and prompt treatment. Supportive measures, surgery, and belimumab impacted the outcome of this patient.@*Conclusion@#Gastrointestinal (GI) vasculitis in SLE has a good outcome with early diagnosis and intervention. Our patient responded well with surgery, steroid and belimumab, a fully human recombinant immunoglobulin G (IgG) 1λ monoclonal antibody. This case report showed that belimumab is a good alternative treatment for lupus GI vasculitis.

Mielitis longitudinal asociada a lupus eritematososistémico / Longitudinal myelitis associated with systemic lupus erythematosus

Se presenta el caso de una mujer de 33 anos ˜ de edad con el diagnóstico de mielitis longitudinalsecundaria a lupus eritematoso sistémico. Presentó paraplejía e hipoestesia parasensibilidad térmica y dolorosa a nivel del dermatoma T6, recibió manejo con pulsos demetilprednisolona y ciclofosfamida con respuesta parcial al tratamiento. La mielitis es unaenfermedad inflamatoria que produce una lesión en la médula espinal, la mielitis longitudinalhace referencia a la participación continua de la médula espinal, con implicación de 3o más segmentos medulares contiguos. El tratamiento consiste en dosis altas de glucocorticoidescombinados o no con inmunosupresores o plasmaféresis...

Lupus vulgaris of centrofacial area - a peculiar presentation

Lupus vulgaris is a cutaneous manifestation of Mycobacterium tuberculosis infection. It assumes various clinical forms including plaque, ulcerative, hypertrophic, vegetative, papular and nodular type. We here present an unusual case of a ten-year-old girl diagnosed as hypertrophic lupus vulgaris of centrofacial area including nose. Histopathological examination and bacteriological studies were carried out to establish the diagnosis. Unlike plaque type, hypertrophic variety is quite uncommon and only few cases have been reported. Its peculiar appearance on the face which is hardly described in previous literatures and well response to antitubercular therapy prompted us to report it

A Case of Lupus Vulgaris Diagnosed with Interferon-gamma Release Assay / 대한피부과학회지

No abstract available.

Esclerosis Lupoide / Lupoid sclerosis

La esclerosis lupoide es una entidad de exclusión que reúne características fisiopatológicas, clínicas e imagenológicas comunes entre e lupus eritematoso sistémico (LES) y esclerosis múltiple (EM). El propósito del artículo es revisar aspectos inmunológicos, de laboratorio y diagnósticos diferenciales que faciliten comprobar la EL como una entidad clínica y definir las mejores posibilidades terapéuticas.

Lupoid sclerosis is a diagnosis of exclusion with similar clinical, inmunological and imagenological findings between erithematous systemic lupus (SLE) and multiple sclerosis (MS). The aim of this article is to review immunology, laboratory differential diagnosis and treatment to facilitate the understanding of EL as a clinical entity.

Mast Cells in Non-neoplastic Skin Lesions.

Background: Mast cells are found in tissues throughout body, particularly in proximity to surfaces that interface with the external environment. They are found at all levels of dermis, where they are grouped around blood vessels, nerves and appendages. Increase in mast cell number have been reported in various cutaneous diseases. Aims: We aim to identify and analyse the morphological aspects of distribution of mast cells and their value in non-neoplastic skin lesions. Material and Methods: Ninety seven biopsies of various skin lesions were studied for mast cells by Toluidine Blue at pH 3.9. Result: All the skin lesions showed significant increase in mast cell number. Psoriasis showed maximum number of mast cells (127/mm2) followed by Lichen planus (108/mm2). Among the cases of cutaneous bacterial infections, mast cells were maximum in leprosy cases (99/mm2) and lowest in cutaneous tuberculosis. A comparative decrease in number of mast cells was seen from lepromatous leprosy to tuberculoid leprosy. A wide range of morphological alterations of mast cells were observed in these skin lesions. Psoriasis and bullous pemphigoid cases showed maximum spindle cell morphology. Conclusion: Tissue mast cell number, distribution and morphology vary in different skin lesions depending on the degree of mast cell activation. This may have a bearing on diagnosis and management.

Cutaneous tuberculosis and squamous-cell carcinoma / Tuberculose cutânea e carcinoma de células escamosas

The incidence of all forms of cutaneous tuberculosis, including lupus vulgaris (the most common form) decreased progressively in developed countries during the twentieth century, this change being attributed to improved living standards and specific therapy. Despite the decrease in cutaneous tuberculosis, some cases are still found and correct diagnosis and management are fundamental, both for the patients and for public health. Long lasting, misdiagnosed or untreated cutaneous tuberculosis may lead to different forms of cancer. This case report involves a 74-year old male farmer with lupus vulgaris on his face. During anti-tuberculosis treatment he developed a tumor on his forehead, which was histologically confirmed as a squamous cell carcinoma.

A incidência de todas as formas de tuberculose cutânea, incluindo o lúpus vulgar (a forma mais comum) diminuiu progressivamente nos países desenvolvidos durante o século XX e esta mudança foi atribuída a melhores condições de vida e uso de terapia específica. Apesar da diminuição da TBC cutânea, alguns casos ainda são detectados hoje em dia e o diagnóstico acurado e o tratamento apropriado são fundamentais para os doentes e a saúde pública. A TBC cutânea quando prolongada, diagnosticada erroneamente ou não tratada pode levar a diferentes neoplasias. Este é o relato de um caso de lúpus vulgar na pele do rosto de uma paciente do sexo masculino, agricultor, de 74 anos de idade. Durante a terapia antituberculosa ele desenvolveu um tumor na pele da testa, histologicamente confirmado como carcinoma de células escamosas.

Lupus vulgaris on the buttock mimicking tinea corporis

Lupus vulgaris is the most common form of cutaneous tuberculosis which usually occurs in patients previously sensitized to mycobacterium tuberculosis. It is often clinically and histopathologically confused with various cutaneous disorders. Here, we present a 28-year-old man attended in our medical college with slowly progressive, asymptomatic, annular erythematous skin lesions on the buttocks for 8 years. He consulted with many physicians and was improperly treated with an oral antifungal agent for several months under the diagnosis of tinea corporis, but no cure was observed. A diagnosis of lupus vulgaris was made based on the histopathological examination and the polymerase chain reaction. Anti-tuberculosis therapy was administered and the lesions started to regress