RESUMO
INTRODUCTION: Terbinafine may cause subacute cutaneous lupus erythematosus (SCLE), and we aimed to analyze its clinical characteristics. METHODS: We collected literature on terbinafine-induced SCLE from 1997 to 2023 for retrospective analysis. Thirty-seven patients (33 females and 4 males) were included. RESULTS: The patients have a median age of 49.5 years (range 18-79) and onset time of 5 weeks (range 1-12). SCLE is mainly manifested as annular erythematous (83.3%), scaly erythematous (44.4%), and maculopapular erythematous (13.9%). Mainly, histopathological manifestations are lymphocytic infiltrate (55.6%), hyperkeratosis (38.9%) and keratinocyte necrosis (38.9%). Positive immunological parameters mainly include antinuclear antibody (100.0%), anti-Ro/SSA antibody (94.1%), and anti-La/SSB antibody (72.2%). Past medical history usually includes photosensitivity (33.3%), inflammatory disease (33.33%), and lupus erythematosus (12.1%). Symptoms are completely resolved within a median time of 35 days (range 7-84) after discontinuation of terbinafine and treatment with topical corticosteroids, systemic corticosteroids, hydroxychloroquine, and immunosuppressant. No recurrence was observed within 12 months (range 1.5-48) of follow-up. CONCLUSION: These results suggest that terbinafine-induced SCLE should be comprehensively diagnosed based on clinical symptoms, histopathological manifestations, immunological parameters, and past medical history. Terbinafine should be immediately discontinued when SCLE occurs, while systemic and topical corticosteroids combined with hydroxychloroquine may be an effective treatment.
RESUMO
S100 proteins comprise a family of structurally related proteins that are calcium-sensitive. S100 proteins have been found to play various roles in regulation of cell apoptosis, cell proliferation and differentiation, cell migration and invasion, energy metabolism, calcium homeostasis, protein phosphorylation, anti-microbial activity and inflammation in a variety of cell types. While the specific function of many S100 proteins remains unknown, some of the S100 proteins serve as disease biomarkers as well as possible therapeutic targets in skin diseases. Interface dermatitis (ID) is a histopathological term that covers many different skin conditions including cutaneous lupus erythematosus, lichen planus, and dermatomyositis. These pathologies share similar histological features, which include basal cell vacuolization and lymphocytic infiltration at the dermal-epidermal junction. In this review, we summarize how the S100 protein family contributes to both homeostatic and inflammatory processes in the skin. We also highlight the role of S100 proteins in neuronal signalling, describing how this might contribute to neuroimmune interactions in ID and other skin pathologies. Last, we discuss what is known about the S100 family proteins as both biomarkers and potential treatment targets in specific pathologies.
Assuntos
Homeostase , Proteínas S100 , Pele , Humanos , Proteínas S100/metabolismo , Pele/metabolismo , Pele/patologia , Dermatite/metabolismo , Dermatopatias/metabolismo , Biomarcadores/metabolismo , AnimaisRESUMO
OBJECTIVE: Skin involvement is common in systemic lupus erythematosus (SLE), but may be resistant to conventional treatment. We sought to evaluate the efficacy of anifrolumab (ANI) in refractory cutaneous manifestations of SLE. METHODS: Case series of patients with refractory cutaneous SLE from three Rheumatology Departments in Greece. Outcome measures were improvement in Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K), physician global assessment (PGA) and Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). Clinically relevant improvement in skin was defined as decrease ≥50% (CLASI50) from baseline values. RESULTS: Eighteen patients received ANI; all had active skin involvement at baseline. Mean (SD) SLEDAI and PGA at ANI initiation were 7.4 (2.7) and 1.4 (0.5), respectively, with a mean prednisone dose 4.9 (4.5) mg/day. Mean CLASI (Activity/Damage) at baseline was 13.9 (9.7)/2.9 (4.6). Patients were refractory to a mean 6.3 (1.5) immunomodulatory agents (including hydroxychloroquine and glucocorticoids) before the initiation of ANI. After a mean 8.5 (4.6) months, 89% (n = 16/18) of patients demonstrated significant improvement in general lupus and cutaneous disease activity, and glucocorticoid tapering. Mean SLEDAI and mean CLASI at last visit were 3.4 (1.9) and 2.1 (2.4)/1.4 (2.2), respectively, and mean daily prednisone dose decreased to 2.4 (2.2). Of note, in this group of highly refractory patients CLASI50 was achieved in 16/18 (89%) patients. One patient discontinued ANI after 4 infusions due to a varicella-zoster virus infection and one patient, who initially responded to treatment with ANI, experienced a skin flare due to temporary discontinuation due to Covid 19 infection. DORIS remission and LLDAS were attained in two (11.1%) and eleven (61.1%) patients, respectively. CONCLUSION: Anifrolumab is highly effective in various skin manifestations of SLE, even after prior failure to multiple treatments.
Assuntos
Dermatite Alérgica de Contato , Líquen Plano , Lúpus Eritematoso Cutâneo , Humanos , Líquen Plano/diagnóstico , Líquen Plano/epidemiologia , Dermatite Alérgica de Contato/diagnóstico , Dermatite Alérgica de Contato/epidemiologia , Dermatite Alérgica de Contato/etiologia , Feminino , Lúpus Eritematoso Cutâneo/diagnóstico , Lúpus Eritematoso Cutâneo/epidemiologia , Masculino , Pessoa de Meia-Idade , Bases de Dados Factuais/estatística & dados numéricos , Adulto , Estados Unidos/epidemiologia , Idoso , Fatores de RiscoRESUMO
Acute cutaneous lupus erythematosus (ACLE) is closely associated with systemic symptoms in systemic lupus erythematosus (SLE). This study aimed to identify potential biomarkers for ACLE and explore their association with SLE to enable early prediction of ACLE and identify potential treatment targets for the future. In total, 185 SLE-diagnosed patients were enrolled and categorized into two groups: those with ACLE and those without cutaneous involvement. After conducting logistic regression analysis of the differentiating factors, we concluded that tumor necrosis factor-alpha (TNF-α) is an independent risk factor for ACLE. Analysis of the receiver operating characteristic revealed an area under the curve of 0.716 for TNF-α. Additionally, both TNF-α and ACLE are positively correlated with disease activity. TNF-α shows promise as a biomarker for ACLE, and in SLE patients, ACLE may serve as a clear indicator of moderate-to-severe disease activity.
RESUMO
Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by diverse clinical manifestations affecting multiple organs and systems. The understanding of genetic factors underlying the various manifestations of SLE has evolved considerably in recent years. This review provides an overview of the genetic implications in some of the most prevalent manifestations of SLE, including renal involvement, neuropsychiatric, cutaneous, constitutional, musculoskeletal, and cardiovascular manifestations. We discuss the current state of knowledge regarding the genetic basis of these manifestations, highlighting key genetic variants and pathways implicated in their pathogenesis. Additionally, we explore the clinical implications of genetic findings, including their potential role in risk stratification, prognosis, and personalized treatment approaches for patients with SLE. Through a comprehensive examination of the genetic landscape of SLE manifestations, this review aims to provide insights into the underlying mechanisms driving disease heterogeneity and inform future research directions in this field.
RESUMO
Lupus erythematosus panniculitis (LEP) is a chronic inflammatory skin disease with a significant impact on the overall well-being of patients. The safety and efficacy of oral baricitinib for the treatment of LEP have not been studied. This study aimed to explore the efficacy of oral baricitinib in patients with LEP who are recalcitrant or intolerant to conventional therapies. Patients (aged ≥18 years) with active LEP (with a revised cutaneous lupus erythematosus disease area and severity index [RCLASI]-active score ≥4] were randomly assigned 2:1 to baricitinib (4 mg) or placebo (once daily for 20 weeks). The placebo group was switched to baricitinib (4 mg) at week 13, and the final evaluation was conducted at week 24. The primary endpoint was the proportion of patients with an RCLASI-A score decreased by 20% at week 12. The secondary endpoints included the changes in the Cutaneous Lupus Erythematosus Disease Area and Severity Index active-(CLASI-A) score, the Dermatology Life Quality Index (DLQI), the Physician's Global Assessment (PGA) score, and safety. Five patients were enrolled. Three patients received baricitinib (4 mg), and two patients were treated with placebo. Two patients in the baricitinib treatment group showed a significant RCLASI-A decrease at week 12 and week 24. Two patients in the placebo group had no change in RCLASI-A at week 12 and a significant decrease at week 24. No new safety events were observed. Treatment with baricitinib was effective and well tolerated in patients with LEP.
RESUMO
Tattooing, the introduction of exogenous pigments into the skin, has a rich history spanning thousands of years, with cultural, cosmetic, and medical significance. With the increasing prevalence of tattoos, understanding their potential complications and contraindications is of growing importance. The most common complications are hypersensitivity reactions, which may vary in morphology and timing. Infectious complications are often due to inadequate aseptic and hygienic practices during the tattooing process or healing period. Tattoo pigment can present diagnostic challenges, affecting cancer diagnosis and imaging. This CME article explores the history, cultural significance, epidemiology, chemistry, technique, contraindications, and complications of tattoos. Appreciating these factors can help individuals considering tattoos understand the safety and potential risks of their body art, and provide physicians with a thorough understanding of tattooing if consulted.
RESUMO
A subtype of cutaneous lupus erythematosus known as lupus erythematosus tumidus (LET) is characterized by sun-exposed areas that typically display urticaria-like papules and plaques. For LET, systemic therapy with antimalarials - particularly hydroxychloroquine (HCQ) - is the first line of treatment. Even though the safety profile of these medications appears to be high, there have been very few reports of side effects in the literature, including hemolytic anemia, retinal toxicity, maculopapular rash, gastrointestinal disturbance, and blue-gray discoloration of the skin or mucous membranes. Here, we report a unique instance of a 46-year-old LET smoker who, following HCQ treatment, developed a generalized myopathy.
RESUMO
Discoid lupus erythematosus (DLE) is an autoimmune skin condition that is typically part of the cutaneous manifestation of systemic lupus erythematosus (SLE). DLE is characterized by erythematous patches that can progress to depigmentation and alopecia, leading to scarring and permanent hair loss if left untreated. Herein, we present a unique case of localized DLE on the scalp in a 46-year-old female with no prior history of autoimmune disorders. The patient underwent several medication trials, including intralesional corticosteroids, topical calcineurin inhibitors, topical corticosteroids, and systemic hydroxychloroquine, with limited success in treating her discoid alopecia. Subsequently, a combination therapy of oral hydroxychloroquine and topical pimecrolimus significantly improved her scalp lesion. This case highlights the efficacy of combination therapy in managing localized DLE, providing valuable insights for future research focused on DLE alopecia management and optimizing treatment strategies for similar cases.
RESUMO
Subacute cutaneous lupus erythematosus (SCLE) is a variant of cutaneous lupus erythematosus (CLE) characterized by distinct skin lesions. Clinical manifestations typically include annular or psoriasiform skin lesions, often localized in sun-exposed areas such as the chest and back. The pathogenesis of SCLE is largely unknown, and contributing factors include genetics, environmental exposures, and immunological dysregulation. SCLE may be idiopathic or drug-induced, with common triggers being calcium channel blockers, thiazide diuretics, and terbinafine. Intravenous immunoglobulin (IVIG) treatment, frequently used in various autoimmune conditions, has a rare association with SCLE. We report a case in which this condition arose during IVIG treatment for chronic inflammatory demyelinating polyneuropathy (CIDP). Knowledge of this rare effect is beneficial to all providers who prescribe IVIG, including neurology, rheumatology, and dermatology.
RESUMO
Background: Cutaneous involvement is common in systemic lupus erythematosus (SLE) patients and may be essential to the disease activity. This study aimed to describe cutaneous manifestations spectrum and determine the association of cutaneous lesions with the disease activity and systemic involvement among SLE patients in Malang, Indonesia. Methods: A cross-sectional study was conducted using 54 SLE patients from rheumatology outpatient clinic at Saiful Anwar General Hospital Malang, Indonesia. Cutaneous features were classified according to Gilliam and Sontheimer classification of cutaneous lupus. Disease activity and clinical manifestations were documented according to Mexican-SLE disease activity index (Mex-SLEDAI). Results: Among 54 subjects, 50% of the patients had cutaneous manifestations. Subacute cutaneous lupus erythematosus (SCLE) was observed in 11.1% of patients, and malar rash in 20.4%. Subjects with cutaneous lesions had significantly higher Mex-SLEDAI scores, especially those who had SCLE (p<0.001), malar rash (p=0.002), alopecia (p=0.002), and photosensitivity (p=0.032). Six patients (11.1%) had skin infections with higher disease activity (9[8-11]vs.2[0-4];p<0.001). SCLE was significantly associated with malar rash (OR 11.7[1.8-76.5]), vasculitis (OR 43.0[4.1-445.6]), and fatigue (OR 15.0[2.1-108.8]). Malar rash was associated with photosensitivity (OR 8.4[1.6-44.0]), while oral or nasal ulcer was associated with fatigue (OR 8.6 [1.4-54.6]). Vasculitis (OR 5.9[1.0-35.1]) and nephritis (OR 11.7 [1.8-76.5]) were associated with the presence of skin infection. Conclusion: SCLE and malar rash are the most common cutaneous lesions among subjects. Subjects with cutaneous lesions have relatively higher disease activity. Several skin lesions are also associated with SLE patients' systemic manifestations.
RESUMO
In rare instances, patients with SLE may exhibit atypical clinical manifestations, such as Hypocomplementemic Urticarial Vasculitis, which can pose diagnostic challenges. Here, we present a case report of a 28-year-old female with a history of SLE with lupus nephritis clase IV who developed HUV-like symptoms, ultimately leading to a diagnosis of C1q Vasculitis. This case underscores the importance of considering C1q Vasculitis in SLE patients presenting with HUV-like features and highlights Rituximab as a promising therapeutic option for managing this rare condition.
Assuntos
Complemento C1q , Lúpus Eritematoso Sistêmico , Rituximab , Urticária , Vasculite , Humanos , Feminino , Adulto , Complemento C1q/deficiência , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Vasculite/diagnóstico , Vasculite/tratamento farmacológico , Urticária/diagnóstico , Rituximab/uso terapêutico , Nefrite Lúpica/diagnóstico , Nefrite Lúpica/complicações , Nefrite Lúpica/tratamento farmacológico , Diagnóstico DiferencialRESUMO
Nuclear matrix protein (NXP-2) positive amyopathic dermatomyositis (DM) may present without classic symptoms like muscle weakness, dysphagia, and edema, and mimic conditions like cutaneous lupus. Given DM's association with malignancy and interstitial lung disease, prompt and accurate diagnosis is important. Testing for myositis-specific antibodies aids diagnosis in ambiguous cases.
RESUMO
Lupus erythematosus tumidus (LET) is an uncommon but distinct photosensitive subtype of cutaneous lupus erythematosus (CLE). It differs from discoid and subacute cutaneous lupus erythematosus (SCLE) clinically and pathologically. LET is marked by extreme photosensitivity and carries a much lower risk of progression to systemic disease. The differential diagnosis of LET includes polymorphic light eruption (PMLE) and Jessner's lymphocytic infiltration of the skin (JLIS) because of subtle alterations in the histopathology and the paucity of immunopathologic markers in LET. We report herein a case of LET with positive immunoglobulin (Ig) deposits on direct immunofluorescence (DIF) testing. LET resolved completely with strict sun avoidance and treatment with topical corticosteroids, without the sequelae of atrophy, scarring, or dyspigmentation.
RESUMO
BACKGROUND: We aimed to investigate the prevalence of skin disease among patients with systemic lupus erythematosus (SLE) and determine whether LE skin disease had clinical or serologic correlates with SLE. METHODS: We reviewed records of 335 patients with SLE (seen at Mayo Clinic, Rochester, Minnesota, USA) and abstracted skin manifestations, fulfilled mucocutaneous SLE criteria, and clinical and serologic parameters. RESULTS: Of the 231 patients with skin manifestations, 57 (24.7%) had LE-specific conditions, 102 (44.2%) had LE-nonspecific conditions, and 72 (31.2%) had both. LE skin disease was associated with photosensitivity, anti-Smith antibodies, and anti-U1RNP antibodies (all P < 0.001). Patients without LE skin disease more commonly had elevated C-reactive protein levels (P = 0.01). Patients meeting 2-4 mucocutaneous American College of Rheumatology criteria less commonly had cytopenia (P = 0.004) or anti-double-stranded DNA antibodies (P = 0.004). No significant associations were observed for systemic involvement (renal, hematologic, neurologic, and arthritis) when comparing patients with or without LE skin involvement. LE skin involvement was not significantly associated with internal SLE disease flare, number of medications, or overall survival. CONCLUSIONS: LE skin disease commonly occurs in patients with SLE. The presence of LE skin disease had no mitigating impact on the severity of SLE sequelae, disease flares, number of medications, or overall survival.