Manifestações cutâneas no lúpus eritematoso: o que o clínico precisa saber / Skin manifestations in the lupus erythematosus: what the clinician need to know

O lúpus eritematoso é uma doença autoimune complexa que afeta diversos órgãos. A pele é o segundo local mais acometido e as manifestações cutâneas são divididas em específicas e não específicas. As primeiras possuem em comum o achado histopatológico de dermatite de interface com degeneração vacuolar da camada basal e infiltrado linfocitário na junção dermoepidérmica e são subdivididas em agudas, subagudas e crônicas. As manifestações não específicas compõem um grupo heterogêneo de condições que frequentemente está associado ao lúpus eritematoso sistêmico. O conhecimento e a interpretação adequada de tais manifestações é importante, pois, além da relevância diagnóstica, as lesões cutâneas nos dão informações prognósticas. Portanto, é imprescindível que o clínico conheça as principais manifestações cutâneas do lúpus eritematoso para a melhor condução desses pacientes.

Different distribution patterns of plasmacytoid dendritic cells in discoid lupus erythematosus and lichen planopilaris demonstrated by CD123 immunostaining

Abstract Background: Clinical and histological features may overlap between lichen planopilaris-associated and discoid lupus erythematosus-associated scarring alopecia. Objectives: The aim of this study was to demonstrate the cutaneous infiltration of plasmacytoid dendritic cells and to compare their distribution pattern in discoid lupus erythematosus and lichen planopilaris. Methods: Twenty-four cases of discoid lupus erythematosus and 30 cases of lichen planopilaris were examined for immunostaining of the CD123 marker. The percentage and distribution pattern of plasmacytoid dendritic cells and the presence of the plasmacytoid dendritic cells clusters were evaluted in the samples. Results: The number of plasmacytoid dendritic cells was higher in the discoid lupus erythematosus specimens. Aggregations of 10 cells or more (large cluster) were observed in half of the discoid lupus erythematosus specimens and only 2 lichen planopilaris, with 50% sensitivity and 93% specificity for differentiating discoid lupus erythematosus from lichen planopilaris. Study limitations: Incidence and prevalence of discoid lupus erythematosus-associated scarring alopecia in the scalp are low, so the samples size of our study was small. Conclusions: We suggest that a plasmacytoid dendritic cells cluster of 10 cells or more is highly specific for distinguishing discoid lupus erythematosus from lichen planopilaris. It also appears that CD123 immunolabeling is valuable in both active and late stages of the disease.

Upper lip squamous cell carcinoma arising from discoid lupus erythematosus treated with standard wide excision

@#Introduction: Squamous cell carcinoma (SCC) is a rare complication of long-standing discoid lupus erythematosus (DLE). The reported risk factors associated with the malignant transformation include chronic inflammation, scarring, immunosuppression, and the dysfunctional healing already evident in discoid lesions. If underdiagnosed and left untreated, this may lead to higher rates of recurrences, metastases and death. Objective: The main objective of this article is to report the diagnosis and management of a DLE-associated SCC on the upper lip of an elderly Filipino female. A short discussion on the pathogenesis and treatment of this disease entity is also presented.ugs/topical medications, family history and accompanying systemic symptoms were reviewed and analyzed. Case: A 78-year-old female, pawid maker, developed a rapidly growing verrucous tumor measuring 4.0 x 2.0 x 2.0 cm from a depigmented atrophic plaque located on the upper lip. Initial histopathologic findings were consistent with SCC and DLE. Considering the tumor size and the challenging anatomical location, the surgical oncology and plastic surgery team carried out a wide excision with 5 mm margin of the entire upper lip mass and surrounding plaque, followed by lip reconstruction. Biopsy findings of the excised mass were consistent with the initial findings. Three months post-operation, there was significant wound healing with no recurrences. Conclusion: Although the incidence is rare, SCC occurring in DLE is considered to be high-risk and should warrant early recognition and prompt treatment. In this paper, the authors also highlight the use of a standard wide excision in lieu of Mohs Microscopic Surgery as an alternative option where resources are limited.

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.

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.

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.

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

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

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

Incidental Focal Acantholytic Dyskeratosis in a Patient with Discoid Lupus Erythematosus: A Possible Role for SPCA1 in the Pathogenesis of the Disease

No abstract available.

Intense Pulsed Light and Q-Switched 1,064-nm Neodymium-Doped Yttrium Aluminum Garnet Laser Treatment for the Scarring Lesion of Discoid Lupus Erythematosus

Discoid lupus erythematosus (DLE) is a chronic form of cutaneous lupus that can cause permanent scarring. Treatment of DLE includes protection from sunlight and artificial sources of ultraviolet light, as well as systemic and topical medications. The first-line standard therapies are antimalarials and topical steroids. Other systemic therapies include systemic steroid, azathioprine, dapsone, and immunosuppressive agents. Topical tacrolimus and pimecrolimus have also been evaluated. Recent studies reported that several treatments, including pulsed dye laser, CO₂ laser, intense pulsed light (IPL), and 1,064-nm long-pulse neodymium-doped yttrium aluminum (Nd:YAG) have been used for the cosmetic treatment of DLE. Here, we report a case of a DLE scar that was successfully treated with a combination therapy of IPL and Q-switched 1,064-nm Nd:YAG laser.

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.

Periorbital discoid lupus: a rare localization in a patient with systemic lupus erythematosus

Abstract A 40-year-old female patient with a 5-year history of systemic lupus erythematosus was referred to our policlinic with complaints of erythema, atrophy, and telangiectasia on the upper eyelids for 8 months. No associated mucocutaneous lesion was present. Biopsy taken by our ophthalmology department revealed discoid lupus erythematosus. Topical tacrolimus was augmented to the systemic therapeutic regimen of the patient, which consisted of continuous antimalarial treatment and intermittent corticosteroid drugs. We observed no remission in spite of the 6-month supervised therapy. Periorbital discoid lupus erythematosus is very unusual and should be considered in the differential diagnosis of erythematous lesions of the periorbital area..

Association between academic performance and cognitive dysfunction in patients with juvenile systemic lupus erythematosus / Associação entre desempenho acadêmico e disfunção cognitiva em pacientes com lúpus eritematoso sistêmico juvenil

Abstract Objective To determine whether there is an association between the profile of cognitive dysfunction and academic outcomes in patients with juvenile systemic lupus erythematosus (JSLE). Methods Patients aged ≤18 years at the onset of the disease and education level at or above the fifth grade of elementary school were selected. Cognitive evaluation was performed according to the American College of Rheumatology (ACR) recommendations. Symptoms of anxiety and depression were assessed by Beck scales; disease activity was assessed by Systemic Lupus Erythematosus Disease Activity Index (SLEDAI); and cumulative damage was assessed by Systemic Lupus International Collaborating Clinics (SLICC). The presence of autoantibodies and medication use were also assessed. A significance level of 5% (p < 0.05) was adopted. Results 41 patients with a mean age of 14.5 ± 2.84 years were included. Cognitive dysfunction was noted in 17 (41.46%) patients. There was a significant worsening in mathematical performance in patients with cognitive dysfunction (p = 0.039). Anxiety symptoms were observed in 8 patients (19.51%) and were associated with visual perception (p = 0.037) and symptoms of depression were observed in 1 patient (2.43%). Conclusion Patients with JSLE concomitantly with cognitive dysfunction showed worse academic performance in mathematics compared to patients without cognitive impairment.

Resumo Objetivo Determinar se há associação entre o perfil de disfunção cognitiva e os resultados acadêmicos em pacientes com lúpus eritematoso sistêmico juvenil (LESj). Métodos Foram selecionados pacientes com idade de início da doença ≤ 18 anos e com escolaridade mínima do quinto ano do Ensino Fundamental seguidos em um hospital universitário. A avaliação cognitiva foi feita de acordo com as recomendações do Colégio Americano de Reumatologia (ACR). Os sintomas de ansiedade e depressão foram avaliados pelas escalas Beck, a atividade da doença foi avaliada pelo Systemic Lupus Erythematosus Disease Activity Index (Sledai) e o dano cumulativo pelo Systemic Lupus International Collaborating Clinics (Slicc). Também foram avaliados a presença de autoanticorpos e o uso de medicação. Adotou-se nível de significância de 5% (p < 0,05). Resultados Foram incluídos 41 pacientes com média de 14,5 ± 2,84 anos. Disfunção cognitiva foi observada em 17 (41,46%). Observou-se pioria significativa no desempenho de matemática em pacientes com disfunção cognitiva (p = 0,039). Sintomas de ansiedade foram observados em oito pacientes (19,51%) e estavam associados à percepção visual (p = 0,037) e sintomas de depressão foram observados em um paciente (2,43%). Conclusão Pacientes com LESj com disfunção cognitiva apresentam pior desempenho acadêmico em matemática em relação a pacientes sem disfunção cognitiva.

Rituximab for refractory anemia and thrombocytopenia in a patient with systemic lupus erythematosus

INTRODUCTION: Hematologic manifestations of Systemic Lupus Erythematosus (SLE) such as hemolytic anemia, leucopenia and thrombocytopenia are among the common causes of morbidity and hospitalization among patients. This is a case report of a patient presenting with refractory cytopenias.CASE: The  patient  is  a  42-year-old  female,  diagnosed with  SLE,  having  met  the  Systemic  Lupus  Erythematosus  International  Collaboration  Clinics  Criteria  for  diagnosis Persistent  serositis  (pleural  effusion  and  ascites) and worsening of anemia prompted treatment with oral corticosteroid (prednisone 1.0 mg per kg per day) which showed improvement of shortness of breath and  pleural effusion. Cytopenias persisted despite increasing IV pulse steroid to 6.0  mkd  prednisone then further to 13 mkd prednisone.Rituximab given as 1.0 g infusion once every two weeks for two doses which resulted to improvement of anemia, thrombocytopenia and serositis.  CONCLUSION: Hematologic manifestations may present as life threatening complications of lupus.Most cases are responsive  to  corticosteroid  therapy,however,in a few refractory cases,less used conventional treatment such as rituximab,may provide significant response.

Cheilitis granulomatosa associated with lupus erythematosus discoid and treated with methotrexate: report of a case

We present the rare case of a 47-year-old patient, suffering from cheilitis granulomatosa and lupus erythematosus discoid: this association is really exceptional because only once reported in English literature. In addition, the treatment of cheilitis granulomatosa is a challenge for the dermatologist: the gold standard, represented by steroids, is in fact designed as a short-time option. Our report confi rms the good efficacy of methotrexate as a steroid-sparing agent.

Teleducation about Cleft Lip and Palate: An Interdisciplinary Approach in the Promotion of Health

Introduction The Young Doctor Project (YDP) uses Telehealth and Interactive Teleducation instruments to promote the integration of different areas of health and to build knowledge. This methodology can also foster public awareness on various issues related to health. In this context, the objective of this study was to emphasize cleft lip and palate (CLP), which is one of the most common birth defects in Brazil. Objective The study aimed to apply a model of education regarding CLP, based on the dynamics of the YDP, and to evaluate the participants' knowledge acquired after participating in the YDP. Methods The participants were 41 students, 13 to 15 years of age and at the eight- and ninth-grade levels in a private elementary school in Bauru (Brazil). To analyze the performance of the participants, a questionnaire was administered before and after the completion of the training program. The training program was structured in three steps using: (1) interactive teleducation classes, (2) a cybertutor, and (3) practical activities. Results There was a statistically significant difference between the pre- and postparticipation questionnaire results. The improved performance of participants is evidenced by the increase in the rate of correct answers on all issues. Conclusion The YDP on CLP was applied in the school setting following the three steps recommended by the project, and, after the implementation of the training program, there was a significant increase in participants' knowledge of CLP. The YDP on CLP proved an effective tool in promoting health education. .

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.

Cutaneous manifestations of systemic lupus erythematosus - an experience from Bahawal-Victoria Hospital, Bahawalpur

To document the frequency of cutaneous manifestation of systemic lupus erythematosus [SLE]. It was a case-series collected from Dermatology Department and all four Medical Units of Bahawal-Victoria Teaching Hospital of Quaid-e-Azam Medical, Bahawalpur. A total of 100 patients with diagnosis of SLE and fulfilling the inclusion criteria were included in the study. All the patients were evaluated for the cutaneous manifestations of SLE. All the information was collected in a specially designed proforma and analyzed with the help of SPSS version 10. The mean age of the patients was 25.97 +/- 4.64 years. 92% patients were female and 8% were male. Out of 100 patients, 85% patient presented with cutaneous manifestations. Photosensitivity was seen in 40 [40%] patients, discoid rash in 35 [35%], malar rash in 25 [25%], and oral ulcers in 24 [24%]. SLE is predominantly seen among female patients of young age. Photosensitivity is most common presentation, followed by discoid rash

Dermoscopic Approach to a Small Round to Oval Hairless Patch on the Scalp

BACKGROUND: Various kinds of alopecia can show small round or oval hairless patch. Dermoscopy could be a simple, useful tool for making a correct diagnosis. OBJECTIVE: The aim of this study is to investigate clinical usefulness of dermoscopy for diseases with small round or oval hairless patch on the scalp. METHODS: Dermoscopic examination was performed for 148 patients with small round or oval hairless patch using DermLite(R) II pro. The type and its patient number of alopecia investigated in the study were as below: alopecia areata (n=81), trichotillomania (n=24), tinea captis (n=13), traction alopecia (n=12), lichen planopilaris (n=8), discoid lupus erythematosus (n=7), congenital triangular alopecia (n=2) and pseudopelade of Brocq (n=1). The significance of dermoscopic findings for each disease were evaluated. RESULTS: Characteristic dermoscopic findings of alopecia areata were tapering hairs and yellow dots. Those of trichotillomania and traction alopecia were broken hairs. Dermoscopic findings of tinea capitis included bent hairs, perifollicular white macules and greasy scales. Discoid lupus erythematosus and lichen planopilaris were characterized by dermoscopic findings of lack of follicular ostia. Furthermore, keratin plugs were frequently seen in discoid lupus erythematosus whereas perifollicular hyperkeratosis and erythema were frequently seen in lichen planopilaris. CONCLUSION: Dermoscopic examination for small round or oval hairless patch showed characteristic findings for each disease. Based on these results, we propose dermoscopic algorithm for small round or oval hairless patch on the scalp.