Time to progression from discoid lupus erythematosus to systemic lupus erythematosus: a retrospective cohort study.

Determining the risk of progression to systemic lupus erythematosus (SLE) among patients diagnosed with discoid lupus erythematosus (DLE), and the time frame of this risk, are important clinical questions. Past reports have demonstrated a wide time frame of progression from DLE to SLE, with mean time to progression of approximately 8 years. Using data obtained from an academic lupus centre, we identified 32 patients who progressed from DLE to SLE. In our cohort, we found that the median time to progression from DLE to SLE was 453 days, much sooner than previously reported. We believe this information can help inform clinicians on monitoring visit intervals and how best to counsel patients on SLE progression.

Early discoid lupus erythematosus protects against renal disease in patients with systemic lupus erythematosus: longitudinal data from a large Latin American cohort.

OBJECTIVES: The objective of this study was to examine whether early discoid lupus erythematosus (DLE) would be a protective factor for further lupus nephritis in patients with systemic lupus erythematosus (SLE). METHODS: We studied SLE patients from GLADEL, an inception longitudinal cohort from nine Latin American countries. The main predictor was DLE onset, which was defined as physician-documented DLE at SLE diagnosis. The outcome was time from the diagnosis of SLE to new lupus nephritis. Univariate and multivariate survival analyses were conducted to examine the association of DLE onset with time to lupus nephritis. RESULTS: Among 845 GLADEL patients, 204 (24.1%) developed lupus nephritis after SLE diagnosis. Of them, 10 (4.9%) had DLE onset, compared to 83 (12.9%) in the group of 641 patients that remained free of lupus nephritis (hazard ratio 0.39; P = 0.0033). The cumulative proportion of lupus nephritis at 1 and 5 years since SLE diagnosis was 6% and 14%, respectively, in the DLE onset group, compared to 14% and 29% in those without DLE (P = 0.0023). DLE onset was independently associated with a lower risk of lupus nephritis, after controlling for sociodemographic factors and disease severity at diagnosis (hazard ratio 0.38; 95% confidence interval 0.20-0.71). CONCLUSIONS: Our data indicate that DLE onset reduces the risk of further lupus nephritis in patients with SLE, independently of other factors such as age, ethnicity, disease activity, and organ damage. These findings have relevant prognosis implications for SLE patients and their clinicians. Further studies are warranted to unravel the biological and environmental pathways associated with the protective role of DLE against renal disease in patients with SLE.

The natural history of pediatric-onset discoid lupus erythematosus.

BACKGROUND: Pediatric discoid lupus erythematosus (DLE) is rare. The risk of progression to systemic lupus erythematosus (SLE) is uncertain. OBJECTIVE: We sought to determine the risk of progression of pediatric DLE to SLE and to characterize its phenotype. METHODS: This was a retrospective review of 40 patients with DLE. RESULTS: Six (15%) of 40 patients presented with DLE as a manifestation of concurrent SLE. Of the remaining 34, 9 (26%) eventually met SLE criteria and 15 (44%) developed laboratory abnormalities without meeting SLE criteria. Only 10 (29%) maintained skin-limited disease. The average age at progression to SLE was 11 years, with greatest risk in the first year after DLE diagnosis. Most (89%) patients with SLE met diagnostic criteria with mucocutaneous disease (discoid lesions, malar rash, oral and nasal ulcers, photosensitivity), positive antibodies, and/or cytopenia without developing end-organ damage over 5 years of median follow-up. LIMITATIONS: The study was retrospective. CONCLUSIONS: In pediatric patients, DLE carries a significant risk of progression to SLE but may predict a milder phenotype of systemic disease. All patients require careful monitoring for SLE, particularly within the first year of diagnosis.

Prevalencia de anticuerpos antifosfolípido en pacientes con lupus eritematoso cutáneo subagudo y crónico / Prevalence of Antiphospholipid Antibodies in Patients with Subacute and Chronic Cutaneous Lupus Erythematosus

Introducción y objetivos: La prevalencia de anticuerpos antifosfolípido (AcAF) en pacientes con lupus eritematoso sistémico (LES) ha sido muy estudiada, pero no en pacientes con lupus eritematoso cutáneo (LEC). Determinamos la prevalencia de AcAF entre nuestros pacientes con LEC, y analizamos sus características clínicas y serológicas. Material y métodos: Estudio retrospectivo de 182 pacientes con LEC subagudo (LECS) o crónico (LECC) que se hallaban en seguimiento en los últimos 5 años. Seleccionamos aquellos que presentaban uno o varios de los siguientes AcAF: anticoagulante lúpico (AL), anticuerpos anticardiolipina (ACA) y anticuerpos anti β2-glucoproteína i (anti-β2-GPI), en 2 determinaciones, distanciadas al menos en 12 semanas. En el caso de los ACA y los anti-β2-GPI solo se incluyeron pacientes con titulaciones iguales o superiores a 40 unidades por ml. Resultados: Obtuvimos una serie de 13 pacientes: 4 fueron clasificados como LECS y 9 como LECC. Siete cumplían criterios de LES y solo uno cumplía criterios de SAF. La prevalencia de AcAF fue del 38% entre los que cumplían criterios de LES, y del 3,65% entre los que no los cumplían. El AcAF más prevalente fue el AL, presente en 10 pacientes. Se detectaron Ac ANA en 12 pacientes y anti-dsADN en 11. Conclusiones: La prevalencia de AcAF entre nuestros pacientes con LEC que no cumplían criterios de LES fue similar a la referida para la población general. Esto, junto a la fuerte asociación de la presencia de ANA y AcAF, cuestionaría la rentabilidad de determinar los AcAF en aquellos pacientes con LEC y ANA negativo. Además destaca que entre nuestros pacientes con LEC y AcAF existe una alta prevalencia de lesiones discoides y el desarrollo de SAF es poco frecuente (AU)

Background and objectives: The prevalence of antiphospholipid antibodies (APLAs) has been extensively studied in patients with systemic lupus erythematosus (SLE) but not in those with cutaneous lupus erythematosus (CLE). We determined the prevalence of APLAs among our patients with CLE, and analyzed their clinical and serologic characteristics. Materials and methods: This retrospective study analyzed 182 patients with subacute or chronic CLE who had been in follow-up for 5 years. We selected those positive for 1 or more of the following APLAs in 2 measurements at least 12 weeks apart: lupus anticoagulant (LA), anticardiolipin antibodies (ACAs), and anti-β2-glycoprotein I (anti-β2-GPI) antibodies. In the case of ACAs and anti-β2-GPI antibodies, only patients with titers greater than or equal to 40 U/mL were selected. Results: We obtained a series of 13 patients (4 with subacute disease and 9 with chronic disease). Seven met the diagnostic criteria for SLE and only 1 met the diagnostic criteria for antiphospholipid syndrome (APS). The prevalence of APLAs was 38% among patients with SLE and 3.65% among those without SLE. The most prevalent APLA was LA, present in 10 patients. Antinuclear antibodies (ANAs) were detected in 12 patients and anti-double-stranded DNA antibodies in 11. Conclusions: The prevalence of APLAs among our patients with CLE who did not meet the diagnostic criteria for SLE was similar to that reported in the general population. This, along with the strong assocation between the presence of ANAs and the presence of APLAs, would bring into question the value of determining APLAs in patients with CLE who are negative for ANAs. We also note that there was a high prevalence of discoid lesions but a low prevalence of APS among our patients with CLE who were positive for APLAs (AU)

Cutaneous manifestations of connective tissue disease.

There is evidence that early treatment of connective tissue in adolescence improves clinical outcomes; thus, recognition of the cutaneous manifestation of CTD is critical. This review summarizes the clinical features that are unique to children and adolescents in cutaneous (CLE) and systemic lupus erythematosus (SLE), juvenile dermatomyositis (JDM), juvenile systemic scleroderma (JSS), juvenile localized scleroderma (JLS), and juvenile inflammatory arthritis (JIA).

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.

Acute lumbosacral polyradiculoneuropathy heralding transformation to systemic lupus erythematosus in a patient with discoid lupus.

Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease with which a variety of neuropathic disorders have been associated. Among these, the acute inflammatory demyelinating polyradiculoneuropathy variant of Guillain-Barré syndrome has been well established. However, acute axonal lumbosacral polyradiculoneuropathy accompanied by albuminocytological dissociation in the cerebrospinal fluid has been extremely rarely reported in SLE. We report on a 47-year-old woman with discoid lupus presenting with acute onset of flaccid paraplegia. Extensive investigations suggested the diagnoses of axonal lumbosacral polyradiculoneuropathy and SLE. Treatment with intravenous methylprednisolone and cyclophosphamide resulted in clinical recovery. Development of immune-mediated polyneuropathy in a patient with discoid lupus should forewarn the clinician regarding transformation into the systemic form of the disease.

Cutaneous manifestations and serological findings in 260 patients with systemic lupus erythematosus.

The prevalence and prognostic value of cutaneous manifestations in patients with systemic lupus erythematosus (SLE) is not clear due to a lack of distinct criteria. Our aim was to investigate the prevalence of cutaneous manifestations in SLE patients according to strict dermatological classification, compare the results with other studies and to assess differences in serological markers between patients with and without cutaneous lupus erythematosus (CLE). Secondary aims were to investigate the validity of the criteria 'malar rash' and 'photosensitivity' for SLE diagnosis. We included 260 consecutive SLE patients, and 164 with skin complaints were examined by a dermatologist. CLE was found in 23% of the 260 SLE patients. There was agreement on the presence of malar rash in only 60% of patients seen by both rheumatologists and dermatologists. A history of polymorphous light eruption (PLE) was found in 42% of patients. Raynaud's phenomenon was significantly more common in patients with CLE. In addition, four malignant melanomas were found. Based on our findings, we suggest that the American College of Rheumatology (ACR) criteria for SLE diagnosis include histopathologically confirmed CLE as one criterion, and that the criteria photosensitivity and malar rash should be re-defined. Regular examination by a dermatologist is called for in SLE patients.

Squamous cell carcinoma arising in a recent plaque of discoid lupus erythematous, in a sun-protected area.

Squamous cell carcinoma is a known complication reported to occur in chronic discoid lupus erythematosus in sun-exposed areas. We report a patient with systemic lupus erythematosus who developed a squamous cell carcinoma in a recent plaque of discoid lupus erythematosus in a sun-protected area. This article emphasizes the need for a very high index of suspicion for squamous cell carcinoma and repeated biopsies when discoid lupus erythematosus fails to respond to conventional therapy or there is unexplained exacerbation.

Discoid lupus erythematosus in a patient with scleroderma and hepatitis C virus infection.

A 49-year-old Japanese woman presented with discoid lupus erythematosus (DLE) on the face. The presence of Raynaud's phenomenon, swollen fingers, a high anti-nuclear antibody titer, and the results of a biopsy revealed limited-type systemic sclerosis (lSSc). The association of SSc with DLE is rare, although some single case reports have been published in Japan. Our patient was positive for hepatitis C virus infection. Racial predisposition and immune imbalance are proposed to have played a role in the development of these lesions in our case.

Dramatic response of scarring scalp discoid lupus erythematosus (DLE) to intravenous methylprednisolone, oral corticosteroids, and hydroxychloroquine in a 5-year-old child.

Discoid lupus erythematosus (DLE) is rare in childhood. We report the case of a 5-year-old girl who presented with erythematous scaly plaques, with scarring alopecia, involving approximately 40% of her scalp. Histopathology confirmed the diagnosis of DLE. Treatment with intravenous methylprednisolone, hydroxychloroquine, oral prednisone, topical corticosteroids, and sunscreen lead to reversal of scarring alopecia and re-growth of hair.

Early diagnosis and treatment of discoid lupus erythematosus.

Discoid lupus erythematosus is a chronic dermatological disease that can lead to scarring, hair loss, and hyperpigmentation changes in skin if it is not treated early and promptly. It has a prolonged course and can have a considerable effect on quality of life. Early recognition and treatment improves the prognosis. The diagnosis is usually made by clinical examination. In some cases histopathology may be required to confirm the diagnosis. The histology is that of an inflammatory interface dermatosis. There is insufficient evidence for which treatment is most effective. Because lesions are induced or exacerbated by ultraviolet exposure, photoprotective measures are important. Potent topical steroids and antimalarials are the mainstay of treatment. Some cases of discoid lupus erythematosus can be refractory to standard therapy; in these cases retinoids, thalidomide, and topical tacrolimus offer alternatives, as do immunosuppressives like azathioprine, cyclosporine, mycophenolate mofetil, and methotrexate.

Systemic lupus erythematosus and discoid lupus erythematosus.

The management of a patient with SLE presents the OMS with a challenging array of decisions to make in regards to treatment sequencing. Careful reviews of the patient's medical history, the presentation of the illness, and the medication profile, and an open discourse with medical colleagues are necessary to ensure the safety of patients. By becoming comfortable with the medical treatment the patient is undergoing as well as having a firm understanding of where the patient is along the cascade of disease severity (see Fig. 7), appropriate decisions can be made in regards to the patient's outcome and prognosis. Proceeding into surgical treatment without following these guidelines could potentially result in catastrophic consequences for the patient.

Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus.

The term "Acute Syndrome of Apoptotic Pan-Epidermolysis" (ASAP) designs clinical entities characterized by massive cleavage of the epidermis resulting from hyperacute epidermal basal cell apoptotic injury. It can be seen typically in classic toxic epidermal necrolysis (TEN), but occasionally occurs in non-drug-induced entities called "TEN-like" diseases (e.g., lupus erythematosus (LE), acute graft versus host disease and pseudoporphyria). We would like to highlight the difficulties of establishing differential diagnoses between "TEN-like" LE and drug reactions, especially when LE has not been previously diagnosed. We report a patient with fulminate pattern of epidermal cell injury resulting in a clinical presentation having combined features of drug-induced TEN and acute cutaneous LE with laboratory findings of systemic LE (SLE) and without systemic symptoms or high-risk drug ingestion. Although most cases of ASAP in the setting of LE are drug-induced TEN, there are reported cases of "TEN-like" LE with subacute progression, absence of systemic involvement and lack of drug ingestion. Such cases usually have a previous history of SLE and positive serologic markers. Although some authors observed that these lesions could be related to systemic severity of SLE, this is the first patient reported who progresses to discoid LE and we think it could be a marker of good prognosis.

Discoid lupus erythematosus of the eyelids associated with staphylococcal blepharitis and Meibomian gland dysfunction.

Lower eyelid involvement occurs in 6% of patients with discoid lupus erythematosus (DLE). Eyelid lesions are rarely the initial manifestation of DLE. We describe a 25-year-old woman presenting with discoid lesions of the lower eyelids, staphylococcal blepharitis and Meibomian gland dysfunction, who later developed a discoid lesion on the chin. Histopathological and immunofluorescence studies of a biopsy specimen from this lesion established the diagnosis of DLE. We are unaware of any previously reported cases of DLE presenting with discoid eyelid lesions associated with staphylococcal blepharitis and Meibomian gland dysfunction. DLE should be considered as a differential diagnosis in chronic blepharitis that persists despite usual medical management and eyelid hygiene. Misdiagnosis may lead to eyelid margin deformities, necessitate a complicated full-thickness biopsy, and delay diagnosis of systemic lupus.

Long-term thalidomide use in refractory cutaneous lesions of lupus erythematosus: a 65 series of Brazilian patients.

Thalidomide has been reported as efficacious in refractory cutaneous lupus erythematosus (LE). The most fearful side-effects are teratogenicity and neuropathy. We reported clinical efficacy of long-term low-dose use of thalidomide in 65 patients with LE, emphasizing the prevalence of adverse effects, especially of neuropathy and its related factors. Data obtained from medical records included age, sex, disease duration, and the presence of diagnostic criteria for systemic lupus erythematosus (SLE), the extent and activity of cutaneous lesions and previous treatments. Sixty-three patients (98.9%) presented complete or partial improvement with thalidomide therapy. Drowsiness occurred in 50 patients (77%). Twenty-eight patients (43.2%) presented neuropathy symptoms. Nerve conduction studies were done in 21 (75%) of them and were abnormal in 12 (57%). With the interruption of thalidomide, 24 (82.5%) had complete or partial improvement of neuropathy symptoms and 23 (82%) of them had cutaneous relapse. There were no significant differences between those who developed or not neuropathy in treatment duration, age, total dose and systemic versus cutaneous LE. In conclusion, thalidomide can be used in refractory cutaneous LE with great efficacy and relative security. Controlled studies with schemes with lower doses or intermittent usage or alternative drugs are wanted to reduce the burden of cutaneous morbidity of lupus erythematosus.