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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.

Cutaneous lupus erythematosus (CLE) is a chronic inflammatory skin disease characterized by a diverse cadre of clinical presentations. CLE commonly occurs in patients with systemic lupus erythematosus (SLE), and CLE can also develop in the absence of systemic disease. Although CLE is a complex and heterogeneous disease, several studies have identified common signaling pathways, including those of type I interferons (IFNs), that play a key role in driving cutaneous inflammation across all CLE subsets. However, discriminating factors that drive different phenotypes of skin lesions remain to be determined. Thus, we sought to understand the skin-associated cellular and transcriptional differences in CLE subsets and how the different types of cutaneous inflammation relate to the presence of systemic lupus disease. In this study, we utilized two distinct cohorts comprising a total of 150 CLE lesional biopsies to compare discoid lupus erythematosus (DLE), subacute cutaneous lupus erythematosus (SCLE), and acute cutaneous lupus erythematosus (ACLE) in patients with and without associated SLE. Using an unbiased approach, we demonstrated a CLE subtype-dependent gradient of B cell enrichment in the skin, with DLE lesions harboring a more dominant skin B cell transcriptional signature and enrichment of B cells on immunostaining compared to ACLE and SCLE. Additionally, we observed a significant increase in B cell signatures in the lesional skin from patients with isolated CLE compared with similar lesions from patients with systemic lupus. This trend was driven primarily by differences in the DLE subgroup. Our work thus shows that skin-associated B cell responses distinguish CLE subtypes in patients with and without associated SLE, suggesting that B cell function in skin may be an important link between cutaneous lupus and systemic disease activity.

Lupus erythematosus (LE) is a chronic autoimmune condition with a wide spectrum of clinical presentations. Alopecias, both non-scarring and scarring, frequently occur in the context of LE and can assume several different patterns. Furthermore, alopecia occurring with LE may be considered LE-specific if LE-specific features are present on histology; otherwise, alopecia is considered non-LE-specific. Non-scarring alopecia is highly specific to systemic LE (SLE), and therefore has been regarded as a criterion for the diagnosis of SLE. Variants of cutaneous LE (CLE), including acute, subacute, and chronic forms, are also capable of causing hair loss, and chronic CLE is an important cause of primary cicatricial alopecia. Other types of hair loss not specific to LE, including telogen effluvium, alopecia areata, and anagen effluvium, may also occur in a patient with lupus. Lupus alopecia may be difficult to treat, particularly in cases that have progressed to scarring. The article summarizes the types of lupus alopecia and recent insight regarding their management. Data regarding the management of lupus alopecia are sparse and limited to case reports, and therefore, many studies including in this review report the efficacy of treatments on CLE as a broader entity. In general, for patients with non-scarring alopecia in SLE, management is aimed at controlling SLE activity with subsequent hair regrowth. Topical medications can be used to expedite recovery. Prompt treatment is crucial in the case of chronic CLE due to potential for scarring and irreversible damage. First-line therapies for CLE include topical corticosteroids and oral antimalarials, with or without oral corticosteroids as bridging therapy. Second and third-line systemic treatments for CLE include methotrexate, retinoids, dapsone, mycophenolate mofetil, and mycophenolate acid. Additional topical and systemic medications as well as physical modalities used for the treatment of lupus alopecia and CLE are discussed herein.

OBJECTIVE To evaluate the susceptibility to coronavirus disease 2019 (COVID-19) in patients with autoimmune conditions treated with antimalarials in a population-based study. METHODS All residents treated with chloroquine (CQ)/hydroxychloroquine (HCQ) from July through December 2019 and living in 3 provinces of Regione Emilia-Romagna were identified by drug prescription registries and matched with the registry containing all residents living in the same areas who have had swabs and tested positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Odds ratios (ORs) and 95% confidence intervals (95% CIs) were calculated. RESULTS A total of 4,408 patients were identified. The prevalence of patients receiving antimalarials was 0.85 per 1,000 men and 3.3 per 1,000 women. The cumulative incidence of testing during the study period was 2.7% in the general population and 3.8% among those receiving CQ or HCQ, while the cumulative incidence of testing positive was 0.55% in the general population and 0.70% among those receiving CQ/HCQ. Multivariate models showed that those receiving CQ/HCQ had a slightly higher probability of being tested compared to the general population (OR 1.09 [95% CI 0.94-1.28]), the same probability of being diagnosed as having COVID-19 (OR 0.94 [95% CI 0.66-1.34]), and a slightly lower probability of being positive once tested (OR 0.83 [95% CI 0.56-1.23]). None of the differences were significant. CONCLUSION Our findings do not support the use of antimalarials as a prophylactic treatment of COVID-19.

Systemic fluorouracil agents include not only 5-fluorouracil (5FU), but also capecitabine, tegafur, and uracil/tegafur (UFT). Systemic lupus erythematosus (SLE), subacute cutaneous lupus erythematosus (SCLE), and discoid lupus erythematosus (DLE) are subtypes of lupus erythematosus; drug-induced lupus erythematosus can also present in each of these subtypes. This report describes the case of a 65-year-old woman with systemic 5FU-induced DLE. Fluorouracil agent-induced DLE lesions occurring after initiating treatment with either systemic 5FU or its prodrugs have been described in 19 individuals (Including the woman in this report) in the literature: tegafur (10 patients), UFT (six patients), systemic 5FU (two patients), and capecitabine (one patient). The mean duration before the appearance of the DLE lesions on sun-exposed areas was 232 days after beginning the fluorouracil agent; however, the much earlier (three weeks) appearance of the DLE lesions after starting systemic 5FU in the women described in this report may have occurred since there was no delay associated with the conversion of a precursor drug to 5FU. Within two months (mean: 36 days) after stopping the fluorouracil agent, the DLE lesions resolved in 95% of the patients. Laboratory studies were only performed on some of the patients. None of the patients tested had antibodies to Ro/Sjogren's syndrome A (Ro/SSA) and La/Sjogren's syndrome B (La/SSB). The antinuclear antibody (ANA) titer was elevated in 71% of the tested individuals and decreased in all of the patients who were evaluated after the causative drug was discontinued. The pathogenesis for fluorouracil agent drug-induced DLE remains to be definitively established.

Discoid menisci on both the medial and lateral sides are rare, and there are very few reports on cases involving both sides. We report a case of a 52-year-old female with medial and lateral discoid menisci in both knees. Arthroscopy revealed the lateral menisci of both knees were complete discoid menisci, and partial meniscectomy was performed. The medial menisci were incomplete discoid menisci, but there were no findings of abnormal mobility or injury; therefore, the medial menisci were observed without treatment. At six months postoperatively, her pain and range of motion restrictions disappeared.

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.

This longitudinal retrospective study aims at describing the safety profile and the reasons for discontinuation of antimalarials in patients with systemic lupus erythematosus (SLE) and discoid lupus erythematosus (DLE), focusing on ocular toxicity. We analyzed the clinical data of 845 SLE and DLE patients; 59% of them were taking antimalarials: 1.4% chloroquine (CQ), 88.5% hydroxychloroquine (HCQ) and 10.1% both. The mean therapy duration was 82.5 ± 77.4 months. At least one side effect was reported by 19.4% of patients, leading to temporary or permanent withdrawal in 9.1% and 10.3% of cases, respectively; 19.3% of patients experienced side effects with HCQ and 8.6% with CQ. In 55.1% of cases, the adverse event was mild or moderate. Ophthalmological alterations were reported by 8.5% but were confirmed by the ophthalmological examination in 5.5% of cases. Retinal alterations were associated with age, disease duration and duration of the antimalarial therapy, but not to drug dose and comorbidities or lupus nephritis. This is the largest monocentric longitudinal study confirming the good safety profile of antimalarials in DLE and SLE patients. The main adverse events during the therapy were mild or moderate, but maculopathy—reported in a low percentage of patients—remains the main cause of treatment withdrawal.

Familial multiple trichodiscomas is a condition characterized by multiple asymptomatic skin papules. The inheritance pattern has not been established. The skin lesions usually appear in childhood. The diagnosis of the cutaneous papules is established by pathologic evaluation. Birt-Hogg-Dube syndrome is excluded by not detecting any aberration in the folliculin gene locus. Including our patient, 15 index individuals and their families are described. There is no systemic organ involvement or associated malignancies in individuals with this condition.

A 22-year-old woman with a 3-year history of discoid lupus erythematosus presented with two circumscribed patches of non-scarring alopecia, clinically simulating alopecia areata. Histopathological analysis of scalp lesions revealed discoid lupus erythematosus. Based on the clinical history, physical examination, and histological and immunological findings, we distinguished our case from a true combination of alopecia areata and typical chronic discoid lupus erythematosus.

Purpose: To introduce and evaluate the clinical results of a new arthroscopic technique for partial meniscectomy of symptomatic lateral discoid meniscus using a knife. Materials and Methods: From March 2005 to October 2010, 60 knees of 58 patients underwent arthroscopic partial meniscectomies for lateral discoid meniscus. The average age was 28.9 years (range, 12 to 63 years), and average follow-up was 26 months (range, 8 to 72 years). In this procedure, using a No. 11 knife holder inserted through the high far anteromedial portal, a stab incision on the anterior meniscal horn and following piecemeal meniscal excision were made. Clinical results were assessed using the scale of Ikeuchi and Lysholm score. Results: Meniscus shape was complete in 32 knees (53.3%) and incomplete in 28 knees (46.6%). The shape of tears in complete type lesions was horizontal cleavage in 17 knees (53.1%), flap or complex degenerated tears in 10 knees (31.2%) and radial tears in 5 knees (15.6%). Clinical results assessed using the scale of Ikeuchi were excellent in 38 (63.3%), good in 13 (21.6%), fair in 8 (13.3%) and poor in 1 knee (1.6%). The average Lysholm score was improved from 82.8 preoperatively to 95.4 postoperatively. Conclusions: Our new arthroscopic technique in lateral discoid partial meniscectomy suggests convenient methods and successful clinical results.

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. Relatar o caso de uma paciente com diagnóstico e tratamento para lúpus eritematoso discóide (LED), que se tratava de hanseníase.

Purpose: The aim of the study was to investigate and analyze bilateral incidence and morphology of complete discoid lateral meniscus (DLM) with possible relation to tears and symptoms in knee joints. Materials and Methods: Thirty-eight consecutive patients with symptomatic or already-torn complete DLM on magnetic resonance imaging in a unilateral knee underwent diagnostic arthroscopy on both knee joints upon agreement. The presence and shape of complete DLM as well as presence and pattern of tear were recorded accordingly. Results: In total, 89.5% (34 of 38 patients) showed bilateral complete DLM, and 84.2% yielded bilateralism with identical shape. Cape-slab was the most frequent shape, comprising 68.8% of patients with identically-shaped bilateral DLM overall. Tear patterns were more frequent, in the order of longitudinal, simple horizontal, radial, and degenerative; however, the morphological shape of complete DLM was not significantly related to tear incidence or pattern. Meniscus tears and knee symptoms occurred in the contralateral knee with incidences of 32.4% and 26.5% in patients with bilateral complete DLM, respectively. Conclusion: Based on these findings, more aggressive warning on the presence of discoid pathology and the need for evaluation on the contralateral knee should be considered during consultation with patients with symptomatic complete DLM in a unilateral knee in the outpatient clinic.

To verify (1) how many patients with cutaneous lupus erythematosus (CLE) fulfill 4 or more American Rheumatism Association (ARA) and European Academy of Dermatology and Venereology (EADV) criteria for classification of systemic lupus erythematosus (SLE); (2) which criteria are mostly fulfilled; (3) the severity of the disease in patients fulfilling criteria; (4) how many patients with systemic involvement fail to fulfill 4 ARA and EADV criteria.We studied 207 patients with chronic and subacute CLE, classified according to ARA and EADV criteria.Twenty-four patients with localized discoid (L-DLE; 21.8%), 22 with disseminated discoid (D-DLE; 30.5%) and 7 with subacute CLE (SCLE; 28%) had 4 or more ARA criteria. With EADV criteria, these figures fell to 7 (6.4%), 7 (9.7%) and 6 (24%), respectively. Only 3 L-DLE (2.7%), 5 D-DLE (6.9%) and 3 SCLE cases (12%) defined as SLE by ARA criteria and 1, 3 and 3, respectively, by EADV criteria had a renal or neurological disorder, hemolytic anemia and/or thrombocytopenia, vasculitis or serositis. ARA criteria did not classify 7 patients with a similar visceral involvement, while EADV criteria failed in 11 patients.In our patients, ARA criteria showed a sensitivity of 88%, a specificity of 79%, a positive predictive value of 56% and a negative predictive value of 96%. EADV criteria showed a sensitivity of only 64%, but a specificity of 93%, a positive predictive value of 61% and a negative predictive value of 94%. ARA criteria should not be used in CLE patients as they are too sensitive, poorly specific and altogether misleading. EADV criteria are more specific, but less sensitive.

A 55-year-old patient, who had undergone excisional biopsy of upper lip two years ago and diagnosed to have squamous cell carcinoma, was referred to us for evaluation with Fluorine-18 fluorodeoxyglucose (F-18 FDG) positron emission tomography (PET)/CT (F-18 FDG-PET/CT) scan. F-18 FDG-PET/CT scan was performed and the maximum intensity projection images (MIP) showed unusual FDG uptake at both ears. Histopathological examination of the biopsy specimen obtained from the ears revealed discoid lupus erythematosus (DLE). Conflict of interest:None declared.

ObjectiveSystemic lupus erythematosus is a clinically heterogeneous autoimmune disease. A number of genetic loci that increase lupus susceptibility have been established. This study examines if these genetic loci also contribute to the clinical heterogeneity in lupus.Materials and methods4001 European-derived, 1547 Hispanic, 1590 African-American and 1191 Asian lupus patients were genotyped for 16 confirmed lupus susceptibility loci. Ancestry informative markers were genotyped to calculate and adjust for admixture. The association between the risk allele in each locus was determined and compared in patients with and without the various clinical manifestations included in the ACR criteria.ResultsRenal disorder was significantly correlated with the lupus risk allele in ITGAM (p=5.0×10−6, OR 1.25, 95% CI 1.12 to 1.35) and in TNFSF4 (p=0.0013, OR 1.14, 95% CI 1.07 to 1.25). Other significant findings include the association between risk alleles in FCGR2A and malar rash (p=0.0031, OR 1.11, 95% CI 1.17 to 1.33), ITGAM and discoid rash (p=0.0020, OR 1.20, 95% CI 1.06 to 1.33), STAT4 and protection from oral ulcers (p=0.0027, OR 0.89, 95% CI 0.83 to 0.96) and IL21 and haematological disorder (p=0.0027, OR 1.13, 95% CI 1.04 to 1.22). All these associations are significant with a false discovery rate of ConclusionSignifi cant associations were found between clinical manifestations and the FCGR2A, ITGAM, STAT4, TNSF4 and IL21 genes. The findings suggest that genetic profiling might be a useful tool to predict disease manifestations in lupus patients in the future.

Purpose It was hypothesised that the coding variant (R77H), rs1143679, within ITGAM could predict specific clinical manifestations associated with systemic lupus erythematosus (SLE). Method To assess genetic association, 2366 patients with SLE and 2931 unaffected controls with European ancestry were analysed. The patients with SLE were coded by the presence or absence of individual American College of Rheumatology criteria. Logistic regression and Pearson χ 2 tests were used to assess statistical significance. Results For overall case-control analysis, a highly significant association was detected (p=2.22×10−21, OR 1.73). Using case-only analysis, a significant association was detected with renal criteria (p=0.0003), discoid rash (p=0.02) and immunological criteria (p=0.04). When patients with SLE were compared with healthy controls, the association became stronger for renal (p=4.69×10−22, OR 2.15), discoid (p=1.77×10−14, OR 2.03) and immunological (p=3.49×10−22, OR 1.86) criteria. Risk allele frequency increased from 10.6% (controls) to 17.0% (SLE), 20.4% (renal), 18.1% (immunological) and 19.5% (discoid). Conclusion These results show a strong association between the risk allele (A) at rs1143679 and renal disease, discoid rash and immunological manifestations of SLE.

Two patients presented with gradually enlarging solitary red plaques on the cheek; one was a 50-year-old man who had had a plaque for 6 months’ duration (Fig 1, A), and the other was a 52-year-old man who had had a plaque for 8 months’ duration (Fig 1, B). Clinical examination of both lesions revealed an erythematous, slightly infiltrated plaque, without scaling.

FUNDAMENTO: Lúpus eritematoso cutâneo crônico é uma doença inflamatória crônica relativamente freqüente, mas pouco estudada entre nós. OBJETIVO: Caracterizar epidemiologia e clínica de pacientes com lúpus eritematoso cutâneo crônico, visando comparar dados obtidos com literatura mundial. PACIENTES E MÉTODOS: Foram estudados retrospectivamente 290 pacientes com lúpus eritematoso cutâneo crônico no período de 1982 a 1996, na Clínica de Dermatologia da Santa Casa de São Paulo. RESULTADOS: A média de idade da instalação da doença foi de 32,3 anos, houve predomínio do sexo feminino em relação ao masculino (3,4:1), a maior parte dos pacientes teve lesões localizadas no segmento cefálico (58,3%). Quanto às variedades clínicas, houve predomínio da placa discóide típica em 90,4% dos casos, seguida das variantes verrucosa ou hipertrófica (7,9%), lúpus eritematso pérnio (1,4%), e túmida (0,3%). Lesões em mucosas ou epitélios de transição ocorreram em 27,2% dos pacientes. CONCLUSÕES: Lúpus eritematoso cutâneo crônico é doença mais comum em mulher adulta, sendo a placa discóide típica a lesão mais comum. Lesões mucosas ocorreram em aproximadamente em um quarto dos casos. BACKGROUND: Chronic cutaneous lupus erythematosus is a chronic inflammatory disease, which albeit relatively frequent, has been the object of few studies. OBJECTIVE: To characterize the epidemiological and clinical aspects of patients with chronic cutaneous lupus erythematosus, with a view to comparing the data obtained with the world literature. PATIENTS AND METHODS: A retrospective study was done on 290 patients with chronic cutaneous lupus erythematosus from 1982 to 1996, attended at the Dermatology Clinic of Hospital Santa Casa de Sao Paulo. RESULTS: The mean age at onset of the disease was 32.3 years, there was a female prevalence in relation to males (3.4:1), most of the patients had lesions located in the cephalic segment (58.3%). Regarding the clinical types, there was a prevalence of the typical discoid plaque in 90.4% of cases, followed by the verrucous or hypertrophic forms (7.9%), erythematous lupus pernio (1.4%) and tumid (0.3%). Lesions in the mucous membranes or transition epithelia occurred in 27.2% of the patients. CONCLUSIONS: Chronic cutaneous lupus erythematosus is a disease more frequent in adult women and the most common lesion is the typical discoid plaque. Mucous lesions occurred in approximately one fourth of the cases.

The Systemic Lupus Activity Measure (SLAM) is a system proposed by rheumatologists to measure disease activity in their patients with systemic lupus erythematosus (LE). It involves scoring a group of clinical symptoms and laboratory findings, the maximum possible score being 84. In systemic LE, the mid-point is between 9 and 12. We applied SLAM to 176 patients with cutaneous LE. Ninety-seven had localized discoid LE (L-DLE), 59 had disseminated discoid LE (D-DLE) and 20 had subacute cutaneous LE (SCLE). Eighty-five patients had low activity disease (0–4 points), 72 mildly active disease (5–9 points), 15 moderately active disease (10–14 points) and only four had very active disease (≥ 15 points). The most frequent lesions in patients who scored more than 10 points were photosensitivity, cicatricial alopecia, Raynaud’s phenomenon and oral ulcers. Fifty patients were followed up for more than 5 years (mean follow-up 9 years). Nine of these had an increased SLAM score. Seven had L-DLE, one D-DLE and one SCLE. Seven of the 50 patients had photosensitivity, five cicatricial alopecia, five non-cicatricial alopecia, two Raynaud’s phenomenon and two oral ulcers. Three patients who started with L-DLE evolved to D-DLE. The SLAM system is useful in the monitoring of disease activity in patients with cutaneous LE. Over time, even L-DLE patients may develop active disease. Photosensitivity, alopecia, oral ulcers and Raynaud’s phenomenon seem to herald a worse prognosis.

Discoid lupus erythematosus of palms and soles is a distinct rarity. A patient is described who had such manifestations along with signs of minor systemic involvement.