Dermoscopic criteria of discoid lupus erythematosus: An observational cross?sectional study of 28 patients

Background: Discoid lupus erythematosus (DLE) affects mainly the head and neck and lesions heal with scaring. Early diagnosis of DLE is crucial; dermoscopy may enable early diagnosis and help to assess the prognosis of well-established lesions. Aims: To describe the dermoscopic features of DLE and to correlate them with the histological findings, site and duration of DLE. Material and Method: This study included 28 patients diagnosed as DLE based on clinical and histopathological examination. We examined the lesions clinically, dermoscopically and histopathologically. Evaluated dermoscopic variables were based on data in the available literature and on our observations. Results: Whitish scales (89.3%), arborizing blood vessels (85.7%), follicular plugging (82.1%), and pigmentation (82.1%) were the commonest dermoscopic findings. Radial arrangement of arborizing blood vessel in between a radially arranged perifollicular whitish halo (starburst pattern) (39.3%) was noticed for the first time in this study. Rosettes (57.1%) were also seen. There was significant agreement between many dermoscopic and pathological findings with high sensitivity and specificity of many dermoscopic variants in the diagnosis of DLE. Follicular plugging, perifollicular whitish halo, starburst pattern, follicular red dots and rosettes were detected in early stages of the disease but structureless whitish areas and telangiectasia need more time to develop. Limitations: We examined our patients at the time of presentation only without prospective monitoring and we had a relatively small sample size. Conclusion: Dermoscopy helps in the diagnosis of DLE at different body sites

The mechanism of chloroquine/hydroxychloroquine and its application in stomatological diseases / 口腔疾病防治

@#Chloroquine and hydroxychloroquine are both classic 4-aminoquinoline antimalarial drugs with similar chemical structures and mechanisms of action. As the toxicity and side effects of hydroxychloroquine are lower than those of chloroquine, hydroxychloroquine is the main clinical application at present, with good efficacy and safety. Chloroquine and hydroxychloroquine are widely used in the clinic because of their immunosuppressive, anti-inflammatory, antiviral, antitumor and photoprotective effects. The main mechanisms by which chloroquine/hydroxychloroquine inhibits immunity include inhibiting lysosome activity, autophagy, immune response signaling pathways production of proinflammatory cytokines. Chloroquine stabilizes the lysosomal membrane and reduces the release of lysosomal enzymes. As a prostaglandin antagonist, chloroquine can reduce the production of prostaglandins and leukotrienes, thus playing an anti-inflammatory role. Chloroquine/hydroxychloroquine can inhibit virus proliferation in the early stage of virus replication by inhibiting the glycosylation of the angiotensin converting enzyme 2 receptor. At present, hydroxychloroquine has been found to have significant efficacy in discoid lupus erythematosus, oral lichen planus, chronic cheilitis, pemphigus foliaceus, Sjögren’s syndrome and other stomatological diseases. However, eye damage is the most important adverse reaction of hydroxychloroquine, and its occurrence is related to the cumulative dose of drugs.

A Curious Case of Diffuse Systemic Sclerosis with Discoid Lupus Erythematosus-Like Lesions: Enigma of an Overlap

@#Overlap syndrome is a term used to describe a condition wherein a patient has features of more than one classic inflammatory rheumatic disease like systemic lupus erythematosus, polymyositis, scleroderma and rheumatoid arthritis. Individuals with an overlap syndrome may, but need not meet, complete diagnostic criteria for one or more than one classic rheumatic disease. Mixed connective tissue disease is a specific subset of overlap syndrome wherein patients have antibodies to the U1 small nuclear ribonuclear protein (anti- U1RNP) and clinical features like hand edema, synovitis, Raynaud phenomenon, acrosclerosis and biologically or histologically proven myositis. We came across an interesting case showing clinical features of both Systemic Sclerosis and Discoid Lupus erythematosus (DLE). On complete evaluation, a final diagnosis of Diffuse Systemic Sclerosis - DLE overlap was made on the basis of histopathopathological and serological findings. Patient was started accordingly on systemic and topical medications and responded well.

Three Fibrotic Conditions Treated with 5% Topical Imiquimod Cream as an Antifibrotic Agent / 대한피부과학회지

Imiquimod, a toll-like receptor agonist, is a topical immunomodulator that induces the production of several cytokines including interferon-alpha, which shows antifibrotic properties. We hypothesized that the antifibrotic effect of imiquimod would soften fibrotic skin lesions. Therefore, we applied topical imiquimod with topical tacrolimus or systemic acitretin in patients with refractory lesions of myxedema, angiolymphoid hyperplasia, and generalized discoid lupus erythematosus and confirmed improvement in fibrotic lesions in these patients. Thus, we conclude that use of imiquimod improves fibrotic skin lesions. This report describes our experience with the treatment of this condition along with a review of the related literature.

Vitiligo in a Patient with Systemic Lupus Erythematosus: A Case Report / 대한피부과학회지

Vitiligo is a multifactorial disorder. Neural, biochemical, and autoimmune mechanisms have been hypothetically suggested as etiopathological contributors to this condition. Autoimmunity focuses primarily on genetic factors and the association between vitiligo and other autoimmune disorders including autoimmune thyroid disease, rheumatoid arthritis, psoriasis, type 1 diabetes, pernicious anemia, and Addison's disease. We describe a 35-year-old man with systemic lupus erythematosus who developed concurrent vitiligo and discoid lupus erythematosus suggesting the possible autoimmune association between these 2 different diseases.

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.

Lupus Eritematoso Cutáneo Discoide en una paciente con Esclerodermia Sistémica limitada / Cutaneous Discoid Erythematosus Lupus in a patient with limited Systemic Scleroderma

La esclerodermia sistémica (ES) puede asociarse con otros trastornos del tejido conectivo, como dermatomiositis o poliomiositis, síndrome de Sjôgren, artritis reumatoide y lupus eritematoso sistémico. Con menos frecuencia se ha descrito asociación con lupus eritematoso cutáneo discoide(LECD). Cuando un mismo paciente cumple simultáneamente los criterios para dos o más enfermedades del tejido conectivo, se realiza el diagnóstico de síndrome de superposición (SS). Presentamos el caso de una mujer de 32 años, que consultó por presentar placas deprimidas hipopigmentadas en zonas fotoexpuestas de 2 años de evolución, y fenómeno de Raynaud (FR) de 1 año de evolución. Además, telangiectasias en tronco, labios y cara. En manos se observan úlceras digitales dolorosas en pulpejos de los dedos, edema, esclerodactilia y esclerosis del dorso. En laboratorio destacó ANA (1/80 con patrón moteado), perfil ENA (133 con anticuerpo anti-Ro/SS-A en 47) y biopsia de piel compatible con lupus discoide. Se diagnostica SS cutáneosistémico, con LECD y ES limitada. Se realiza una breve revisión bibliográfica de los casos publicados con esta asociación, con un total de 20 casos reportados al año 2010. Las enfermedades reumatológicas pueden manifestarse inicialmente de manera inespecífica, presentando clínica común entre ellas, como es el caso del FR. Es por esta razón que es relevante determinar un diagnóstico en forma temprana, para definir el manejo y búsqueda de complicaciones asociadas a este. Pese a esto el diagnóstico puede ir modificándose en el tiempo principalmente por la aparición de nueva sintomatología.

Systemic Scleroderma (SS) can be associated to other connective tissue disordes such as Dermatomyositis, Polymiositis, Sjogren Syndrome, Rheumatoid Arthritis and Systemic Erythematosus Lupus. With less frecuency the association to Cutaneus Discoid Erithematosus Lupus (CDEL) has been described. When the same patient presents two or more criteria for a connective tissue disorder Overlap Syndrome (OS) is diagnosed. We present the case of a 32 year-old woman that consulted by presenting hypopigmented depressed plates in sun-exposed áreas for two years and Raynaud phenomenon (RPh) for one year. Also she had chest , lips and facial telangiectasia. She presented painful digital ulcers in her finger pads and edema, sclerodactyly and dorsal sclerosis. Laboratory findings showed ANA (1/80 motted pattern ) , ENA profile ( 133 with anti-Ro/SS-A in 47 ) and a cutaneous biopsy compatible with Discoid Lupus. The diagnosis was Cutaneo-Systemic OS with DCEL and limited SS. In this context we made a bibliographyc review of this published association finding a total of 20 cases in year 2010. Rheumatologic diseases can initially manifest in inespecific ways showing common clinical findings such as RPh. This is why it is relevant to make an early diagnosis in order to define treatment and search for associated complications. Despite this the diagnosis can be modified in time mostly because of the appearance of new symtoms.

Immunofluorescence profile of discoid lupus erythematosus.

The direct immunofluorescence (DIF) of skin in conjunction with histopathology gives the best diagnostic yield. It is invaluable in confirming the diagnosis of small vessel vasculitides and bullous lesions of the skin and can be used as an additional tool to pinpoint the diagnosis of systemic and localized autoimmune diseases involving the skin. This study was undertaken to analyze the strength of DIF vis‑à‑vis histopathology in the diagnosis of discoid lupus erythematosus (DLE) and at the same time to elaborate the specific immunofluorescence findings in the lesions of DLE. The clinical profile and cutaneous lesions of 75 patients with DLE are described. DIF was positive in 68% and histopathology in 60% of cases. The most common immunoreactant was IgG at the dermoepidermal junction, followed by IgM and IgA. A conclusive diagnosis of DLE could be achieved satisfactorily in 64 cases (85%) by a combination of the two techniques.

Lupus palpebral: a propósito de dos casos / Palpebral lupus: apropos of two cases

El lupus eritematoso es una dermatosis inflamatoria, crónica, aguda o subaguda que evoluciona por brotes. Puede afectar a todos los órganos pero más frecuentemente a la piel. Presentamos a dos pacientes con lesión en forma de placa, localizada en zona interna y media del borde libre palpebral inferior, de bordes elevados, depresión central con fondo costroso y pérdida de las pestañas a ese nivel. Se decide -por la localización, extensión de la lesión, los antecedentes personales y la aplicación de tratamientos previos sin mejoría del cuadro clínico- realizar biopsia incisional para un mejor estudio. Los resultados anatomopatológicos revelaron el diagnóstico de lupus discoide palpebral. La evolución posterior al tratamiento médico fue satisfactoria, sin recidiva de la lesión durante el primer año de seguimiento.

Erythematosus lupus is an inflammatory dermatosis of chronic, acute or subacute nature that occurs in outbreaks. It can affect all the body organs, but mainly the skin. Here are two patients with plaque-form lesions located in the internal middle area of the free inferior palpebral border, thick contours, central depression with scabby bottom and loss of eyelash. Taking into account the location, the extension of the lesion, personal histories and application of previous treatment without improved clinical picture, it was decided to perform incisional biopsy for better examination. The anatomopathological results showed the diagnosis of palpebral discoid lupus. The recovery of the patient was satisfactory after the medical treatment, with no relapse during the first follow-up year.

Lesão palpebral de lúpus discoide como manifestação única da doença / Eyelid?s discoid lupus lesion as the only manifestation of the disease

Lúpus eritematoso discoide é uma desordem autoimune benigna da pele e representa a forma clínica mais comum de lúpus eritematoso cutâneo. A apresentação do lúpus eritematoso discoide com acometimento palpebral exclusivo, como neste caso, é extremamente rara, o que dificulta o diagnóstico precoce da doença. O desconhecimento dessa apresentação de lúpus e o consequente retardo do seu diagnóstico podem contribuir com a progressão da doença e resultar em cicatrizes inestéticas, o que pode prejudicar a função palpebral.

Hanseníase tratada como lúpus: relato de caso. A importância do diagnóstico diferencial de hanseníase em um país endêmico / Leprosy treated as lupus: case report. The importance of differential diagnosis of leprosy in an endemic country

A hanseníase ainda constitui relevante problema de saúde pública brasileira, apesar da redução drástica no número de casos. O diagnóstico e tratamento tardios dificultam uma ação decisiva sobre a doença. Relatamos o caso de uma paciente com diagnóstico e tratamento para lúpus eritematoso discoide (LED), que se tratava de hanseníase...

Lupus eritematoso discoide crónico hipertrófico / Hypertrophic chronic discoid lupus erythematosus

El lupus eritematoso discoide crónico hipertrófico (LEDCH) es una variedad poco frecuente del lupus eritematoso discoidecrónico (LEDC) y se manifiesta con lesiones hiperqueratósicas que comprometen áreas fotoexpuestas. Generalmente el cursoclínico está marcado por la cronicidad y resistencia al tratamiento. Presentamos una paciente con lesiones de LEDCH querespondió satisfactoriamente al tratamiento con fotoprotección, corticoides tópicos y sistémicos y antimaláricos. Las lesiones desaparecieron, dejando secuelas pigmentarias residuales.

Hypertrophic chronic discoid lupus erythematosus (LEDCH) is a rare variety of chronic discoid lupus erythematosus (LEDC)characterized by hyperkeratotic lesions that compromise photoexposed areas. Clinical course is marked by chronicity andtreatment resistance. A female patient with LEDCH successfully treated with photoprotection, topical and systemic corticosteroidsand antimalarials is presented. Lesions disappeared leaving residual pigmentary sequelae.

Vitiligo Developed in a Patient with Discoid Lupus Erythematosus / 대한피부과학회지

Vitiligo has been associated with various disorders, including pernicious anemia, diabetes, hyperthyroidism, alopecia areata, and Addison's disease. Discoid lupus erythematosus (DLE), the most common form of chronic cutaneous lupus erythematosus, is an autoimmune disease characterized by skin involvement. A 64-year-old man presented with multiple DLE lesions on the face and the neck, as well as photosensitive eczema on the nape of the neck. After treatment, the DLE lesions improved, but poliosis and several depigmented patches appeared on the vertex, the nape of the neck, and on the right hand. Few reports are available about the concurrence of DLE and vitiligo in other countries, and only one case, which showed coexisting systemic lupus erythematosus and vitiligo, has been reported in the Korean literature. The pathogenesis of concurrence of these two diseases has not been fully elucidated. Herein, we present a case of vitiligo that developed in a patient with DLE.

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.

Generalized Discoid Lupus Erythematosus Occurring in a Systemic Lupus Erythematosus Patient / 대한피부과학회지

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by multisystem involvement. Skin is the second most frequently affected organ in SLE, and various forms of cutaneous LE (CLE), such as acute, subacute, and chronic CLE, are observed in higher than 75% of patients with SLE. Discoid LE (DLE) is the most common form of chronic CLE, and a small subset of patients with SLE also has concomitant DLE. A 32-year-old woman diagnosed with SLE 6 years ago visited our clinic with a 2-month history of multiple hyperpigmented macules and patches over the entire body. She also complained of arthralgia, auditory, and visual hallucinations. Her clinical presentation, laboratory findings, and histopathologic features were consistent with generalized DLE and aggravation of her SLE. Herein, we report an unusual case of generalized DLE occurring in a patient with SLE.

Discoid Lupus Erythematosus in a Patient with Systemic Sclerosis / 대한피부과학회지

Systemic sclerosis is often associated with other autoimmune diseases such as autoimmune thyroiditis, primary Sjogren syndrome, dermatomyositis and autoimmune hepatitis, as the so-called scleroderma overlap syndrome. However, coexistence of scleroderma and discoid lupus erythematosus (DLE) is extremely rare. Association of scleroderma and DLE has not been described definitely, but it is suggested that various factors such as genetic factor, sex hormone, environmental factor, immunologic factor, viral infection and HLA typing may contribute. A 40-year-old woman presented with an 18-year history of Raynaud's phenomenon and generalized hypopigmented sclerotic patches over the entire body. Skin biopsy and other examinations showed characteristics of scleroderma. The diagnosis was diffuse systemic sclerosis. During treatment, erythematous and erosive patches and plaques on face and arms appeared. The clinical features, histopathologic presentation, and laboratory findings were consistent with DLE.

Discoid Lupus Erythematosus-like Eruption Induced by Tegafur - Uracil (UFT) / 대한피부과학회지

The second-generation oral anticancer agent UFT is a combination of uracil, which has fluorouracil's (5-FU) degradation-inhibitory effect, and tegafur, which is slowly converted to 5-FU in vivo, and UFT shows a higher 5-FU concentration in the tumor tissues than is achieved by tegafur alone or with comparable doses of intravenous 5-FU. Mucocutaneous side reactions induced by UFT are rare and these include photosensitivity of the lichenoid and eczematous types, acral erythema, hyperpigmentation, palmoplantar keratoderma and scleroderma-like reactions, and discoid lupus erythematosus (DLE)-like eruption. However, there has been no report in the Korean medical literature on patients presenting with a DLE-like eruption associated with UFT. So, we report here a case of DLE-like eruption induced by oral UFT.

Four Cases of Facial Discoid Lupus Erythematosus Successfully Treated with Topical Pimecrolimus or Tacrolimus

Discoid lupus erythematosus (DLE), which is a cutaneous form of lupus erythematosus (LE), is generally refractory to a wide range of topical or systemic therapies. Although the main treatment option for DLE is topical steroids, it is often ineffective or likely to produce long-term side effects. New drugs, including tacrolimus and pimecrolimus, have been developed to overcome the adverse effects of steroids and treat the lesions of DLE for a prolonged period. We herein report 4 cases of facial DLE successfully treated with therapeutic adjuvants, topical tacrolimus or pimecrolimus.

Lupus eritematoso cutáneo discoide crónico en un paciente con psoriasis vulgar / Chronic discoid lupus erythematosus in a patient with psoriasis vulgaris

Se presentó paciente de sexo femenino de 48 años de edad con antecedentes de psoriasis de 15 años de evolución (confirmada con estudio histopatológico). Su primer brote se relacionó con evento estresante personal. Hace un año comenzó con lesiones diseminadas, que empeoraban con el sol, producían cicatrices y no respondían al tratamiento habitual. Por tal motivo acude nuevamente a nuestro centro. En esta ocasión se le diagnosticó lupus eritematoso, el cual se confirmó con la histología. La paciente no aquejaba sintomatología articular ni sistémica. Todos sus estudios (hemáticos, hemoquímicos, inmunológicos y de función renal) fueron normales. Finalmente se concluyó con el diagnóstico de lupus eritematoso cutáneo discoide crónico asociado a psoriasis vulgar.

We present a 48 years old female patient, with a personal history of psoriasis since 15 years ago (confirmed by histological study). The first episode was related with a personal stressant event. Since one year ago the patient presented lesions that shown a different location and get worse with sun exposure, healed with scars, and present a poor response to the habitual treatment. At that moment the patient was diagnosed with discoid lupus erythematous confirmed by histological study, she never complaint any articular or systemic symptoms and the haematological and immune lab findings and renal tests were normal. We concluded that the diagnosis was discoid lupus erythematous associated to psoriasis vulgaris.

Lupus discoide / No presenta

El Lupus Eritematoso Sistémico (LES) es una enfermedad que afecta a muchos órganos y sistemas, en su patogenia están implicados múltiples autoanticuerpos, y su etiología aún es desconocida. El lupus eritematoso discoide afecta la piel únicamente. Se caracteriza por manchas rojas, como brotes, que aparecen sobre ambas mejillas y el puente de la nariz, dando la impresión de una mariposa con las alas abiertas. Algunas de las lesiones más severas dejan cicatrices y cambian la pigmentación de la piel. Estas manchas pueden aparecer en otras partes del cuerpo, especialmente en las partes expuestas al sol. Esta forma de lupus también puede causar pérdida irregular del cabello, la cual no es necesariamente irreversible.

The Systemic Lupus Erythematosus (SLE) is a disease that affects many organ systems, in its pathogenesis are involved multiple auto antibodies, and its etiology remains unknown. The discoid lupus erythematosus affects the skin only. It is characterized by red spots, such as outbreaks, which appear on both cheeks and the bridge of the nose, giving the impression of a butterfly with open wings. Some of the most severe injuries leave scars and change the pigmentation of the skin. These spots may appear in other parts of the body, particularly in parts exposed to the sun. This form of lupus can also cause irregular hair loss, which is not necessarily irreversible.