Impressive resolution of refractory hypertrophic discoid lupus erythematosus with anifrolumab.

Hypertrophic discoid lupus erythematosus is a rare variant of chronic cutaneous lupus erythematosus and is often challenging to treat. A male in his early 60s presented with diffuse erythematous, crusty, pruritic plaques on his upper and lower extremities, face, upper back, dorsal aspect of the hands and chest. He also described prolonged morning stiffness, swelling of his fingers and wrists, oral sores and Raynaud's phenomenon. He was positive for antinuclear antibody and anti-SSA antibody and had low C3 and C4 proteins. The skin biopsy was consistent with hypertrophic discoid lupus erythematosus. He was diagnosed with systemic lupus erythematosus. Skin lesions were refractory to treatment with topical corticosteroids, topical acitretin, hydroxychloroquine, azathioprine or mycophenolate. Anifrolumab infusions were initiated with a near-complete resolution of cutaneous symptoms within 3 months.

Evaluation of RNase therapy in systemic lupus erythematosus: a randomised phase 2a clinical trial of RSLV-132.

BACKGROUND: Circulating, extracellular RNA is the primary trigger of type I interferon in systemic lupus erythematosus (SLE), and interferon is known to play a central pathogenic role in the disease. RSLV-132 is a catalytically active human RNase molecule fused to human IgG1 Fc designed to digest RNA and thereby decrease the chronic inflammation associated with SLE. The drug was evaluated in a cohort of patients with SLE with moderate-severe cutaneous disease activity and the presence of RNA immune complexes. The primary objective of the study was the assessment of the impact of 13 doses of 10 mg/kg RSLV-132 over 6 months on the mean Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) score. METHODS: Sixty-five patients meeting the entry criteria of a baseline CLASI score of 10 or greater and positivity of at least one of five autoantibodies to RNA-binding proteins (SM/RNP, SSA/Ro, SSB/La, Sm, RNP) were randomly assigned (2:1) to receive 13 doses of RSLV-132 10 mg/kg or placebo, respectively. Participants received study drug for 24 weeks on days 1, 8, 15, 29, 43, 57, 71, 85, 99, 113, 127, 141 and 155 with an end-of-treatment visit on day 169 and a follow-up visit at the end of the study on day 215. The primary objective was assessed on days 85 and 169. Secondary objectives included assessment of systemic disease activity using the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K), the British Isles Lupus Assessment Group 2004 Index and the Physician's Global Assessment. Data from these instruments were used to calculate the SLE Responder Index 4 (SRI-4) and the British Isles Lupus Assessment Group-based Composite Lupus Assessment (BICLA) scores. RESULTS: The mean CLASI score change from baseline at day 169 was -5.7 (±7.0) in the placebo group and -6.2 (±8.5) in the RSLV-132 group. A subgroup of participants with moderate-severe systemic disease activity and high baseline SLEDAI scores (≥9) were analysed with respect to BICLA and SRI-4 responses. The RSLV-132 treated participants in the high SLEDAI subgroup had a greater percentage of BICLA responses (62% vs 44%) and SRI-4 responses (23% vs 11%) as compared with placebo. A second subgroup of participants with high baseline CLASI scores (≥21) were analysed with respect to BICLA and SRI-4 responses. The RSLV-132 treated participants in the high CLASI subgroup had a greater percentage of BICLA responses (28% vs 8%) and SRI-4 responses (39% vs 8%) as compared with placebo. CONCLUSIONS: Six months of RSLV-132 therapy consisting of a weekly loading dose of RSLV-132 for 1 month, followed by 5 months of biweekly administrations did not significantly improve the mean CLASI score relative to placebo in this cohort of patients with SLE. The study entry criteria selected patients with moderate-severe cutaneous disease activity and no minimum SLEDAI score, which resulted in a wide range of systemic disease activity from inactive to severe as measured by SLEDAI. When the participants with higher SLEDAI and CLASI scores were analysed, a trend towards clinical improvement favouring RSLV-132 was observed. The results warrant further evaluation of RSLV-132 in SLE and suggest that patients with more active systemic disease are most likely to benefit from RNase therapy.

Efficacy of anifrolumab in long-term intractable alopecia due to discoid lupus erythematosus.

Alopecia associated with lupus erythematosus is broadly classified into reversible nonscarring alopecia seen in the acute phase, such as worsening of systemic lupus erythematosus (SLE) and cicatricial alopecia seen in chronic cutaneous lupus erythematosus represented by discoid lupus erythematosus (DLE). In DLE-induced alopecia, early therapeutic intervention before developing scarring alopecia is important, but the condition is often resistant to conventional treatment. Anifrolumab (ANI), a novel therapeutic agent for SLE that inhibits Type I interferon activity, has been shown to be effective against acute skin lesions, including alopecia, in patients with SLE. However, there are very few reports on the effect of ANI on alopecia due to DLE. We report on a 27-year-old Japanese woman with SLE whose alopecia due to chronic DLE was refractory to topical therapy and systemic therapy with oral glucocorticoid, multiple immunosuppressants, and belimumab for ∼8 years after onset and whose alopecia improved with ANI. ANI can be considered to be an effective treatment option in lupus patients presenting with alopecia due to DLE, even in the chronic refractory stage.

Concentrated growth factor therapy as cosmetic treatment in discoid lupus erythematosus.

Discoid lupus erythematosus (DLE) is a disfigurement disease. The atrophic scar and hair loss of this disease are followed by cosmetic defects and profoundly impact psychological health. Concentrated growth factor (CGF) has been widely adopted in medical cosmetology. Here we report a 36-year-old female systemic lupus erythematosus patient with a 5-year history of alopecia in DLE, who was recommended for CGF therapy and experienced hair regrowth. We suggest that CGF may be an effective cosmetic treatment for DLE.

Hiperpigmentación de la mucosa oral y discromía ungueal inducida por cloroquina / Chloroquine-induced oral mucosal hyperpigmentation and nail dyschromia

No disponible

Síndrome de Rowell: las 2 caras de la verdad / Rowell's syndrome: the two sides of the truth

No disponible

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.

Alopecia frontal fibrosante y lupus eritematoso discoide: más allá de la coexistencia / Frontal Fibrosing Alopecia and Discoid Lupus Erythematosus: More Than a Coincidence

No disponible

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.

FR-¿Dónde posicionamos la combinación de antipalúdicos en el manejo del lupus eritematoso cutáneo refractario? / Where Should We Position Antimalarial Drug Combinations in the Management of Refractory Cutaneous Lupus Erythematosus?

No disponible

Lúpus comedônico: rara apresentação do lúpus eritematoso discoide / Comedonic lupus: a rare presentation of discoid lupus erythematosus

Lúpus eritematoso cutâneo crônico é doença autoimune, com apresentações polimorfas que podem, eventualmente, mimetizar outras condições clínicas, causando dificuldade diagnóstica. Quadro acneiforme, com comedões e lesões atróficas pontuadas (pitting scars) pode constituir apresentação atípica do lúpus eritematoso cutâneo discóide.

Chronic cutaneous lupus erythematosus is a polymorphous autoimmune disease which may mimic some other clinical conditions, causing diagnostic difficulties. Acneiform lesions, including comedones and pitting scars are occasionally atypical presentations of cutaneous discoid lupus erythematosus.

La verdadera mariposa lúpica / The real lupus butterfly

No disponible

Lúpus discóide em pálpebras: relato de caso / Discoid lupus on the eyelids: case report

O lúpus discóide caracteriza-se por lesões limitadas à pele, eritematosas e recobertas por escamas brancas aderentes, que aparecem mais nas áreas expostas ao sol. Relatamos um caso de uma paciente feminina de 26 anos, com ardência e hiperemia dos olhos há meses, em tratamento de blefarite sem melhora. Apresentava ao exame lesões palpebrais ulceradas, que através do exame de imunofluorescência direta foram diagnosticadas como lúpus discóide. Diante de lesões palpebrais crônicas refratárias a tratamento clínico, mesmo na ausência de alterações nos exames complementares, deve-se realizar a biópsia incisional para elucidação do caso, e se necessário, o exame de imunofluorescência direta.

Discoid lupus is characterized by erythematosus lesions limited to the skin, covered by adherent white scales, which rise at places more exposed to the sun. A case of a 26 year-old woman, who was suffering for months from redness and burning of the eyes, and was been treated for blepharitis without resolution is reported. On the eyelids exam, ulcerated lesions were found, that were diagnosed as discoid lupus by immunofluorescence. When faced with chronic lesions on the eyelids that do not respond to medical treatment, even if the ancillary exams are normal, the incisional biopsy must be performed to elucidate the diagnosis and, if necessary, immunofluorescence should be done as well.

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.

Reacción febril tras toma de talidomida / Fever in treatment with thalidomide

No disponible

Isotretinoína a dosis bajas en el tratamiento del lupus crónico discoide en mujeres fértiles / Low-Dose Isotretinoin For Treatment of Chronic Discoid Lupus in Women of Childbearing Age

No disponible

No disponible

Lupus discoide infantil / Infantile discoid lupus

Se describe un caso de lupus eritematoso discoide que comenzó en la infancia. El lupus eritematoso discoide es infrecuente en la edad infantil y se ha estimado que menos del 2 por ciento de los pacientes desarrollan la enfermedad antes de los 10 años. El trastorno es similar al lupus discoide del adulto, pero aparentemente presenta un riesgo elevado de progresar a lupus eritematoso sistémico

Talidomida 40 años después / Thalidomide 40 years later

La talidomida, sintetizada en Alemania como pastilla para dormir y antiemético muy popular en la década de los 50, fue retirada del mercado al ser culpada de miles de casos de focomelia. Es reintroducida a la medicina cuando Sheskin descubre sus efectos benéficos en la reacción leprosa. A partir de 1965 se inicia la rehabilitación al ser utilizado además en prurigo actínico, lupus eritematoso, transplante de médula ósea yotros padecimientos de difícil manejo. Por lo tanto este medicamento tan odiado por la humanidad merece una nueva oportunidad