Cutaneous lupus erythematosus: a review of etiopathogenic, clinical, diagnostic and therapeutic aspects

Abstract Cutaneous lupus erythematosus is an autoimmune disease of varied clinical expression, which may present as an exclusively cutaneous disease or be one of the multiple manifestations of systemic lupus erythematosus. Its classification includes acute, subacute, intermittent, chronic and bullous subtypes, which are usually identified based on clinical features and histopathological and laboratory findings. Other non-specific cutaneous manifestations may be associated with systemic lupus erythematosus and are usually related to disease activity. Environmental, genetic and immunological factors play a role in the pathogenesis of skin lesions in lupus erythematosus. Recently, considerable progress has been made in elucidating the mechanisms involved in their development, which allows for foreseeing future targets for more effective treatments. This review proposes to discuss the main etiopathogenic, clinical, diagnostic and therapeutic aspects of cutaneous lupus erythematosus, aiming to update internists and specialists from different areas.

Cutaneous lupus erythematosus: a review of etiopathogenic, clinical, diagnostic and therapeutic aspects.

Cutaneous lupus erythematosus is an autoimmune disease of varied clinical expression, which may present as an exclusively cutaneous disease or be one of the multiple manifestations of systemic lupus erythematosus. Its classification includes acute, subacute, intermittent, chronic and bullous subtypes, which are usually identified based on clinical features and histopathological and laboratory findings. Other non-specific cutaneous manifestations may be associated with systemic lupus erythematosus and are usually related to disease activity. Environmental, genetic and immunological factors play a role in the pathogenesis of skin lesions in lupus erythematosus. Recently, considerable progress has been made in elucidating the mechanisms involved in their development, which allows for foreseeing future targets for more effective treatments. This review proposes to discuss the main etiopathogenic, clinical, diagnostic and therapeutic aspects of cutaneous lupus erythematosus, aiming to update internists and specialists from different areas.

Comedogenic lupus: a rare variant of chronic cutaneous lupus erythematosus - case series.

BACKGROUND: Comedogenic lupus is an uncommon variant of cutaneous lupus, clinically characterized by the presence of comedones, papules and erythematous-infiltrated plaques, cysts and scars in photo-exposed areas, mimicking acne vulgaris and acneiform eruptions. OBJECTIVES: To report clinicopathological characteristics of patients with comedogenic lupus in a tertiary dermatology service over a 15-year period and review cases described in the literature. METHODS: Retrospective study of patients with clinical and histopathological diagnoses of comedogenic lupus between the years 2006 and 2021. The literature search was carried out in the PubMed and VHL Regional Portal databases, using the terms: "comedogenic lupus" and "acneiform lupus" in Portuguese and English. RESULTS: Five patients were diagnosed during the described period, all female, with a mean age of 56.6 years. Smoking was observed in three cases, as well as pruritus. The most affected site was the face, especially the pre-auricular, malar and chin regions. Follicular plugs, epidermal thinning and liquefaction degeneration of the basal layer were predominant histopathological findings. Hydroxychloroquine was used as the first-line treatment; however, other medications were used, such as dapsone, methotrexate, tretinoin cream, and topical corticosteroids. The literature search identified 17 cases, with a mean age of 38.9 years, 82% of which were women. Only 23% had a diagnosis of systemic lupus erythematosus. Hydroxychloroquine was the most recommended systemic medication. STUDY LIMITATIONS: Retrospective, single-center study. The literature search was carried out in two databases. CONCLUSIONS: Dermatologists should be aware of acneiform conditions with poor response to the usual treatment. Early diagnosis and treatment reduce the risk of unaesthetic scars.

Lupus eritematoso cutáneo subagudo psoriasiforme. Reporte de caso / Subacute psoriasiform cutaneous lupus erythematosus. Case report

El Lupus eritematoso sistemico (LES) subagudo representa el 10% del total de los casos y en su variedad psoriasiforme resulta poco frecuente. Presentamos el caso de una paciente femenina de 22 años con el diagnóstico previo de LES y nefropatía lúpica hace 2 años, que refiere cuadro clínico de +/- aproximadamente 2 meses de evolución posterior a la suspensión de micofenolato de mofetilo. Ccuadro caracterizado por la aparición progresiva de lesiones dérmicas discoides, costrosas y descamativas que comprometen aproximadamente el 80% de la superficie corporal acompañado de orina espumosa. Examen físico: lesiones corporales respetando palmas y plantas, dolorosas a la digitopresión. Llenado capilar > a 2 segundos. Laboratorios: leucocitos 5930 y Granulocitos 90%. Examen general de orina: infeccioso. Se trata de LES cutáneo subagudo psoriasiforme. El tratamiento fue antibiótico, inmunosupresor y antihipertensivo. Se otorgó el Aalta hospitalaria con micofenolato y ciprofloxacino. El seguimiento debe realizarse por personal médico especializado en reumatología, nefrología y dermatología.

Subacute systemic lupus erythematosus (SLE) represents 10% of all cases and is rare in its psoriasiform variety. We present the case of a 22-year-old female patient with a previous diagnosis of SLE and lupus nephropathy 2 years ago, who reported a clinical picture of +/- 2 months of evolution after the suspension of mycophenolate mofetil. Table characterized by the progressive appearance of discoid, crusty and scaly dermal lesions that involve approximately 80% of the body surface accompanied by foamy urine. Physical examination: bodily injuries respecting palms and soles, painful on acupressure. Capillary filling> 2 seconds. Laboratories: leukocytes 5930 and Granulocytes 90%. General urine test: infectious. This is subacute psoriasiform cutaneous SLE. The treatment was antibiotic, immunosuppressive and antihypertensive. Hospital discharge with mycophenolate and ciprofloxacin. Follow-up should be by medical personnel specialized in rheumatology, nephrology, and dermatology.

Lupus eritematoso sistémico ampolloso en un paciente varón de 16 años / Bullous systemic lupus erythematosus in a 16-year-old male patient

RESUMEN El lupus eritematoso sistémico ampolloso (LESA) es una enfermedad vesículo-ampollosa mediada por autoanticuerpos en pacientes con lupus eritematoso sistémico (LES). Se observan vesículas y ampollas tensas sobre una piel edematosa, eritematosa y, en ocasiones, normal en cualquier región del cuerpo, incluyendo áreas mucosas y que no han sido fotoexpuestas. Se presenta el caso de un paciente varón de 16 años de edad con nefritis lúpica, que al séptimo día de hospitalización presenta múltiples ampollas serosas y hemorrágicas sobre el rostro, el tronco, el abdomen y las extremidades superiores. El estudio histológico mostró una dermatosis ampollar subepidérmica con numerosos neutrófilos.

ABSTRACT Bullous systemic lupus erythematosus (BSLE) is a vesiculobullous disease mediated by autoantibodies in patients with systemic lupus erythematosus (SLE). Tense vesicles and bullae are seen on an edematous, erythematous and sometimes normal skin in any body region, including mucous membranes and non-photoexposed areas. This is the case of a 16-year-old male patient with lupus nephritis who, on the seventh day of hospitalization, presented multiple serous and hemorrhagic blisters on the face, trunk, abdomen and upper extremities. The histological study showed a subepidermal bullous dermatosis with numerous neutrophils.

Pseudomonas aeruginosa as an uncommon agent of infectious panniculitis.

Pseudomonas aeruginosa is a Gram-negative bacillus that frequently causes septicemia, abscesses and infections in skin wounds. Panniculitis caused by this microorganism is unusual and there are few well-documented cases, none of them in a patient with systemic lupus erythematosus. The present report describes an immunosuppressed patient with systemic lupus erythematosus who developed panniculitis caused by Pseudomonas aeruginosa, with a review of the literature on this rare presentation.

Pseudomonas aeruginosa as an uncommon agent of infectious panniculitis

Abstract Pseudomonas aeruginosa is a Gram-negative bacillus that frequently causes septicemia, abscesses and infections in skin wounds. Panniculitis caused by this microorganism is unusual and there are few well-documented cases, none of them in a patient with systemic lupus erythematosus. The present report describes an immunosuppressed patient with systemic lupus erythematosus who developed panniculitis caused by Pseudomonas aeruginosa, with a review of the literature on this rare presentation.

Lupus eritematoso asociado a eritema multiforme: Síndrome de Rowell. Reporte del primer caso en Chile

Rowell's syndrome is characterized by an association of lupus erythematosus and erythema multiforme, with distinctive laboratory findings. Its treatment is similar to lupus. We report a 16-year-old female presenting with skin lesions, laboratory and histology consistent with this entity. Because of the spread of cutaneous involvement and development of epidermal detachment, she required therapy with intravenous corticosteroids and gamma globulin.

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.

Rowell's syndrome: 7 cases and literature review / 中华风湿病学杂志

Objective To leam the clinical features and management of Rowell's syndrome (RS).Methods The clinical information of patients with RS who were admitted to the First Affiliated Hospital of Zhengzhou University from December 2010 to December 2016 was retrospectively analyzed.Results Seven cases,6 women,1 man;with the average age at onset of (40±11) years were reported.Only one patient had clear history of seafood intake.Skin lesions presented as generalized targeted lesions,coupling with some papules.Histopathological examination revealed hyperkeratinization,partial dyskeratocytes,focal areas of basal cell liquefaction,and perivascular infiltration of lymphocytes in the upper dermis.Six of them were treated with prednisone at a dose of 0.7-1.1 mg ·kg-1 ·d-1,one was treated with methylprednisolone at a dose of 80 mg/d while in hospital and at a dose of 1.2 mg ·kg-1 ·d-1 after discharge.During the process of glucocorticoid tapering,hydroxychloroquine was administered at a dose of 0.2-0.4 g/d in 4 patients,and 1 was treated with cyclophosphamide at 0.4 g per week.Glucocorticoid could be tapered once the symptoms were relieved,4 patients without relapse while others relapsed.Conclusion RS is a rare clinical syndrome with relative special immunological presentations.No specific therapy strategy has developed for this disease.Glucocorticosteroids tapering should be slower than in those who with lupus erythematosus,and the prognosis is variable.

Lupus tumidus: a report of two cases

Abstract Lupus tumidus is considered a rare subtype of chronic cutaneous lupus erythematosus, characterized by erythema and bright urticarial erythematous-violaceous lesions that leave no scars after regression. Histopathology reveals perivascular and periannexal lymphohistiocytic infiltrates in the papillary and reticular dermis and interstitial mucin deposition. Treatment is based on photoprotection, topical corticosteroids and antimalarials. We report two cases of lupus tumidus, which deserve attention for their low frequency in the literature, in addition to their relevance as a differential diagnosis among dermatologic disorders.

Linear cutaneous lupus erythematosus following the lines of Blaschko - Case report

Abstract: Chronic cutaneous lupus erythematosus in a linear configuration is rare, particularly in children, demonstrating similar incidence in both genders, no photo-sensitivity and lower probability of progression to systemic disease. We describe the case of a 9-year-old girl who presented erythematous papules with central atrophy on the upper and lower right limbs, asymptomatic and following the lines of Blaschko, since age four. Histological examination showed atrophy of the epidermis with aggression from epidermal-dermal interface and periadnexal and perivascular lymphocytic inflammatory infiltrate. Laboratory tests showed ANA in a titer of 1:320, in a dense and fine speckled pattern. Due to the rarity of presentation and location of the disease, this case is reported here.

Lupus eritematoso neonatal: reporte de un caso / Neonatal lupus erythematosus: case report

El lupus eritematoso neonatal (LEN) es una enfermedad muy poco frecuente que se presenta en el recién nacido, dada por el pasaje transplacentario de autoanticuerpos anti Ro/SSA, anti La/SSB y U1-RNP. Las principales manifestaciones clínicas son dermatológicas y cardíacas, pudiendo además presentar manifestaciones hepáticas, hematológicas y/o neurológicas. Presentamos un caso clínico de un lactante de 3 meses al que se le realizó diagnóstico de LEN en base a la presencia de lesiones cutáneas y hallazgos histopatológicos e inmunológicos compatibles, no se acompañó de manifestaciones sistémicas y presentó una muy buena evolución posterior. El interés del caso es mostrar una patología poco frecuente que suele presentarse con lesiones cutáneas características, destacando que el pronóstico estará determinado por el compromiso cardíaco y que permite en muchos casos realizar diagnóstico materno de una enfermedad autoinmune asintomática.

Neonatal lupus erythematosus (LEN) is a rare disease that occurs in the newborn given by the transplacental passage of maternal Anti-Ro/SSA, Anti-La/SSB and Anti-U1-RNP autoantibodies. The main clinical manifestations are dermatologic and cardiac, it may also have hepatic, hematologic or/and neurological ones. The study reports a case of a three month infant who is diagnosed with LEN, based on the presence of consistent findings of skin lesions and histopathology and immunology compatible findings. In this case, it was not accompanied by systemic manifestations evolution was a favorable one. The case is relevant since it presents an unusual condition that is usually accompanied by characteristic skin lesions, where prognosis is determined by cardiac involvement. In many cases, this enables maternal diagnosis of an asymptomatic autoimmune disease.

Clinical characteristics of 1 006 Chinese patients with lupus erythematosus: results from the Lupus Erythematosus Multicenter Case-control Study in Chinese populations (LEMCSC) / 中华皮肤科杂志

Objective To explore clinical characteristics of Chinese patients with lupus erythematosus (LE).Methods Data were obtained from the Lupus Erythematosus Multicenter Case-control Study in Chinese populations (LEMCSC).A unified standard was used to recruit patients and collect clinical information.The EpiData 3.1 and SPSS 18 softwares were utilized to input and analyze data respectively.Results One thousand and six patients (87.6％ female) with lupus erythematosus (LE) were included in this analysis,of whom,887 (89.9％ female) had systemic LE (SLE),and 119 (70.6％ female) had isolated cutaneous LE (CLE).The most common involved system in SLE patients was skin (72.7％),followed by joints (69.2％),hematological system (60.8％),kidney (48.5％),serosa (18.2％),and nervous system (5.7％).The appearance of LE-specific skin manifestations was associated with an increased risk of arthritis (odds ratio [OR] =1.612,95％ confidence interval [CI]:1.181-2.200),but with a decreased risk of nephritis (OR =0.218,95％ CI:0.157-0.303) and serositis (OR =0.311,95％ CI:0.218-0.443).The presence of acute CLE (ACLE) lesions was a risk factor for systemic involvement (OR =4.931,95％ CI:3.232-7.524),while that of chronic CLE (CCLE) lesions was a protective factor for systemic involvement (OR =0.355,95％ CI:0.234-0.541).The appearance of LE-nonspecific skin manifestations was closely correlated with the involvement of internal organs in patients with LE.Conclusion This study revealed main characteristics of LE patients in China and the relationship between LE-related skin lesions and internal organ involvement.

Possibly drug-induced palpable migratory arciform erythema

Palpable migratory arciform erythema is an entity of unknown etiology, with few published cases in the literature. The clinical and histopathological features of this disease are difficult to be distinguished from those of Jessner’s lymphocytic infiltration of the skin, lupus erythematous tumidus and the deep erythema annulare centrifugum. We describe here the first two Brazilian cases of palpable migratory arciform erythema. The patients presented with infiltrated annular plaques and erythematous arcs without scales. These showed centrifugal growth before disappearing without scarring or residual lesions after a few days. They had a chronic course with repeated episodes for years. In addition, these cases provide evidence of a drug-induced etiology.

Anti-TNF-α and hydralazine drug-induced lupus

Drug-induced lupus is a rare drug reaction featuring the same symptoms as idiopathic lupus erythematosus. Recently, with the introduction of new medicines in clinical practice, an increase in the number of illness-triggering implicated drugs has been reported, with special emphasis on anti-TNF-α drugs. In the up-to-date list, almost one hundred medications have been associated with the occurrence of drug-induced lupus. The authors present two case reports of the illness induced respectively by hydralazine and infliximab, addressing the clinical and laboratorial characteristics, diagnosis, and treatment.

Hereditary angioedema and lupus: A French retrospective study and literature review.

Hereditary angioedema (HAE) is a rare genetic disorder that is primarily caused by a defect in the C1 inhibitor (C1-INH). The recurrent symptoms are subcutaneous edema and abdominal pain. Laryngeal edema, which can also occur, is life threatening if it goes untreated. HAE can be associated with some inflammatory and autoimmune disorders, particularly lupus. The aim of this study was to describe cases of lupus among HAE patients in France and to perform a literature review of lupus and HAE studies. Case detection and data collection (a standardized form) were performed, thanks to the French Reference Center for Kinin-related angioedema. Data were collected from 6 patients with type 1 HAE and lupus in France; no cases of systemic lupus erythematosus were reported. In the literature review, 32 cases of lupus combined with HAE were identified, including 26 female patients. The median patient age at the time of first reported HAE symptoms and at diagnosis were 17.5 years (range, 9-41 years) and 19 years (range, 9-64 years), respectively for our 6 patients and 14 years (range, 3-30 years) and 17 years (range, 7-48 years), respectively, for the literature review. The clinical manifestations of HAE were mainly abdominal pain (83% in our patients vs 47% in the literature) and edema of the limbs (83% vs 38%). The C4 levels were low (for 100% of our cases vs 93% in the literature). Eighteen patients in the literature demonstrated HAE symptoms prior to the lupus onset vs 5 for our patients. The mean patient age at lupus onset was 20 years (range, 13-76 years) for our patients and 19.5 years (range, 1-78 years) in the literature, respectively. In the literature, 81% of the patients had skin manifestations, 25% had renal involvement and 28% received systemic steroids to treat lupus. Treatment with danazol did not modify the clinical expression of lupus. The association between lupus and HAE is a rare but not unanticipated event. Patients are often symptomatic for HAE before developing lupus. Lupus cases associated with HAE share some characteristics of lupus cases related to other complement deficiencies, such as the absence of severity and the predominance of cutaneous symptoms.