Coexistence of chronic cutaneous lupus erythematosus and frontal fibrosing alopecia

Abstract: Lupus erythemathosus is a chronic, relapsing disease with acute, subacute, and chronic lesions. Effluvium telogen occurs in the setting of systemic activity of the disease, and cicatricial alopecia results from discoid lesionsin on the scalp. Other types of alopecia, like alopecia areata, may rarely be found in lupus. Frontal fibrosing alopecia is characterized by frontotemporal hairline recession and eybrow loss. Histophatologically, it cannot be differentiated from lichen planopilaris.It is controversial whether frontal fibrosing alopecia is a subtype of lichen planopilaris.. A pacient with chronic lichenoid lupus erythematosus is described with clinical, histophatological and dermoscopic features of frontal fibrosing alopecia.We have not been able to find in the literature cases of frontal fibrosing alopecia as a clinical manifestation of lupus.

Frontal fibrosing alopecia in association with Sjögren's syndrome: more than a simple coincidence

Abstract Frontal fibrosing alopecia is a distinctive form of scarring alopecia considered to be a clinical variant of lichen planopilaris. It predominantly occurs in postmenopausal women and has a slowly progressive course. It was first described by Kossard in 1994. Since then the number of reported cases has increased significantly. Coexistence of frontal fibrosing alopecia and autoimmune disorders - such as discoid erythematosus lupus and Sjögren's syndrome - may suggest a common pathogenic background among the diseases.

Lesão palpebral como manifestação primária do lúpus eritematoso discóide / Eyelid lesion as a primary manifestation of discoid lupus erythematosus

O lúpus eritematoso discóide é uma desordem autoimune que geralmente afeta áreas da pele expostas ao sol. A apresentação de lesões palpebrais na ausência de outras anormalidades cutâneas é incomum, sendo o envolvimento da pálpebra inferior prevalente em apenas 6% dos pacientes com lúpus eritematoso cutâneo crônico. Relatamos o caso de uma paciente do sexo feminino de 40 anos, com hiperemia, madarose e ulceração na pálpebra inferior do olho esquerdo refratária ao tratamento para blefarite. Inicialmente, outra lesão semelhante havia sido descrita na pálpebra superior do olho direito. No entanto, as biópsias incisionais mostraram-se inconclusivas. Diante de uma lesão migratória palpebral de características semelhantes à primeira, a hipótese clínica de lúpus eritematoso discóide foi aventada e o diagnóstico confirmado por meio de revisão histopatológica. Uma forte suspeita clínica e o reconhecimento precoce podem evitar erros diagnósticos, complicações clínicas e tratamentos inapropriados, como descrito neste caso de lesão palpebral como manifestação primária do lúpus eritematoso discóide.

The discoid lupus erythematosus is an autoimmune disorder which generally affects the sun-exposed skin. Presentation of lesions on the eyelids in the absence of any other cutaneous abnormality is uncommon and the lower-eyelid involvement is seen in 6% of patients with cronic cutaneous lupus erythematosus. We have reported the case of a 40 year-old, woman who presented hyperemia, madarosis and ulceration on the lower eyelid of the left eye. She was treated for blepharitis without resolution. Before that, another similar lesion had been described on the upper eyelid of the right eye. Nevertheless, the incisional biopsies of that eyelid were inconclusive. Faced with a migratory lesion similar to the first one, the clinical hypothesis of discoid lupus erythematosus was suggested and diagnosis was confirmed by histopathological review. A high index of suspicion and early recognition may prevent misdiagnosis, clinical complications and inappropriate treatment, as described in the case of eyelid lesion as a primary manifestation of discoid lupus erythematosus.

Desprendimiento de retina seroso con lupus fijo discoides y nefropatía por inmunoglobulina A / Serous retinal detachment in a patient with discoid fixed lupus and nephropathy by immunoglobulin A

El desprendimiento seroso de retina produce disminución de la agudeza visual debido al paso de fluido procedente de la coroides hacia el espacio subretiniano. Esta enfermedad tiene varias causas, entre las que se encuentran las idiopáticas, congénitas, posquirúrgicas, secundarias e uveítis infecciosas, autoinmune, vascular, hematológicas y neoplásicas.Objetivo: describir el caso inusual de un paciente joven con desprendimiento seroso de retina asociado a lupus fijo discoide y nefropatía por inmunoglobulina A con respuesta satisfactoria al uso de esteroides.Caso clínico: se presenta un paciente masculino de 18 años de edad que acudió a consulta de retina del centro oftalmológico del Hospital Universitario Manuel Ascunce Domenech, por disminución súbita de la agudeza visual en ambos ojos con diagnóstico inicial de coroidopatia serosa central, que posteriormente evolucionó hacia desprendimientos serosos bilaterales con afectación de todo el polo posterior. Por sus antecedentes se interconsultó con el especialista en dermatología y nefrología, donde se decidió iniciar tratamiento con esteroides a altas dosis, vitaminoterapia endovenosa, así como antiinflamatorios y midriáticos ciclopléjicos tópicos con respuesta excelente a la terapia

Serous retinal detachment causes visual acuity decrease due to the passage of fluid from the choroid into the subretinal space. This disease has several causes including idiopathic, congenital, postoperative, and secondary to infectious uveitis, autoimmune, vascular, hematological and neoplastic ones.Objective: to describe an unusual case of a young patient with serous retinal detachment associated with discoid fixed lupus and nephropathy by immunoglobulin A with satisfactory response to steroids use.Clinical case: a male patient of 18 years old was presented in the retina office of the Ophthalmological Center at the University Hospital Manuel Ascunce Domenech with sudden decrease of visual acuity in both eyes with an initial diagnosis of serous central choroidopathy, which later progressed into bilateral serous detachments with entire posterior pole involvement. By his antecedents, a specialty consultation in dermatology and nephrology was carried out, deciding to initiate treatment with high-dose steroids, intravenous vitamin therapy, and anti-inflammatory and topical cycloplegic mydriatic with excellent response to therapy

Granuloma formation in discoid lupuserythematosus: a novel microscopic feature

Lupus erythematosus is an autoimmune disease with marked pleotropism. If several systems are involved then the disease is named as systemic lupus erythematosus [SLE] and if skin is exclusively involved the term discoid lupus erythematosus [DLE] is used. One of the several histopathological features of DLE includes periappendageal inflammation. This may at times ; completely wipe out sebaceous glands forming sebaceous granulomas. To determine the frequency of sebaceous granulomas formation in discoid lupus erythematosus. In this prospective observational study was conducted at the Departments of Dermatology and Pathology, Pakistan Institute of Medical Sciences, Islamabad. 100 cases of DLE spanning over two years and with the age range of 3 years to 70 years were examined for the presence of sebaceous granuloma. Other features of DLE like hyperkeratosis, follicular plugging, epidermal atrophy, basal layer vacuolization, basement membrane deposits, pigmentary incontinence, perivascular inflammation, periappendageal inflammation, and collagen damage were also noted., Out of these 100 cases, 8 cases contained sebaceous granulomas. These granulomas were,, . composed of epithelioid cells, foreign body giant cells containing partially digested sebaceous material and a few lymphoctytes. Sebaceous granulomas formation was seen in 8% cases of DLE cases. This feature must be recognized both by dermatologists and pathologist so that diagnosis of DLE may not be distracted and erroneous diagnosis due to presence of granulomas may not be rendered

A case of lupus-associated pancreatitis in Jamaica / Un caso de pancreatitis asociada con lupus en Jamaica

Pancreatitis complicating a diagnosis of systemic lupus erythematosus (SLE) is rarely reported in the literature and there are no known published cases thus far in the Caribbean. A 50-year old female diagnosed with SLE and discoid lupus erythematosus (DLE) since 1990, presented in February, 2009, to the University Hospital of the West Indies (UHWI), Kingston, Jamaica, with symptoms suggestive of lupus pancreatitis. Serum amylase level was 2341 IU/L and serum lipase was 203 IU/L. Pancreatitis has a 3-8% rate of occurrence in adult patients with SLE. Aetiology and management of this entity remains controversial in these cases, but one must bear the diagnosis in mind, when faced with a SLE patient presenting with abdominal pain, vomiting and diarrhoea.

La pancreatitis que complica el diagnóstico del lupus eritematoso sistémico (LES), raramente se reporta en la literatura, y hasta hoy no se conoce de caso alguno publicado en el Caribe. Una mujer de 50 años de edad, a quien se le diagnosticara lupus eritematoso sistémico (LES), y lupus eritematoso discoide (LED) desde 1990, acudió en febrero de 2009 al Hospital Universitario de West Indies, Kingston, Jamaica, con síntomas que sugerían una pancreatitis por lupus. El nivel de amilasa sérica fue 2341 IU/L y el de lipasa sérica fue 203 IU/L. La pancreatitis tiene una tasa de ocurrencia de 3-8% en pacientes adultos con LES. La etiología y el tratamiento de esta entidad siguen siendo controversiales en estos casos, pero se debe tener presente el diagnóstico frente a pacientes de SLE que presenten dolor abdominal, vómito y diarrea.

Eyelid involvement in disseminated chronic cutaneous lupus erythematosus.

Eye involvement in chronic cutaneous lupus erythematosus (CCLE) has been reported infrequently. We describe two cases of disseminated CCLE, a 50-year-old woman and a 45-year-old man, with eyelid involvement and disease duration of 4 and 20 years respectively. The first patient showed an excellent response to chloroquine while there was lack of response in the second patient.

An uncommon presentation of systemic lupus erythematosus.

A patient who had discoid facial lesion for 10 years developed a right sided pleural effusion in which LE cells were demonstrated. The case is reported due to progression of DLE into SLE after a long interval and rare occurrence of LE cells in pleural fluid.

Lúpus discóide com fenômenos trombembólicos como evidência de sistematizaçäo / Discoid lupus erythematosus with thromboembolic phenomena as evidence of systematization

Os autores relatam caso de paciente com lúpus eritematoso discóide que apresentou fenômenos tombembólicos como evidência de sistematizaçäo. Säo discutidos os achados clínicos, radiológicos e laboratoriais e sua relaçäo com os anticorpos antifosfolípides

Lúpus eritematoso discóide associado a adenocarcinoma de mama / Discoid lupus erythematosus associated with adenocarcinoma of the breast

Apresenta-se um caso de lúpus eritematoso (forma discóide) associado a adenocarcinoma ductal de mama e citam casos similares de lúpus eritematoso gyratum repens, também associados a lesöes malignas internas