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.

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

Abstract 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.

Periorbital discoid lupus: a rare localization in a patient with systemic lupus erythematosus

Abstract A 40-year-old female patient with a 5-year history of systemic lupus erythematosus was referred to our policlinic with complaints of erythema, atrophy, and telangiectasia on the upper eyelids for 8 months. No associated mucocutaneous lesion was present. Biopsy taken by our ophthalmology department revealed discoid lupus erythematosus. Topical tacrolimus was augmented to the systemic therapeutic regimen of the patient, which consisted of continuous antimalarial treatment and intermittent corticosteroid drugs. We observed no remission in spite of the 6-month supervised therapy. Periorbital discoid lupus erythematosus is very unusual and should be considered in the differential diagnosis of erythematous lesions of the periorbital area..

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.

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.

The study on serum level and clinical significance of anti-ribosomal protein P0 antibody in DLE and SLE patients / 实用医学杂志

Objectives To study the serum level and the clinical significance of anti-ribosomal protein P0 antibody in discoid lupus erythematosus(DLE) and systemic lupus erythematosus(SLE) patients. Methods Serum anti-RPLP0 IgG antibody of 18 DLE patients and 23 SLE patients were tested by Enzyme-Linked Immunosorbent Assay (ELISA). Direct immunofluoreseence (DIF) was used to examined the immunoreaetants from skin lesion. Serum antibody and complement C3 were detected by conventional methods. Results Anti-ribosomal P0 antibody was higher in SLE patients (1.23 ± 0.62. mean ± SD) than in patients with DLE (0.53 ± 0.18, P<0.001) and healthy controls (0.72 ± 0.16, P<0.001), but was no difference in the later two groups (P=0.5). Among SLE patients , anti-ribosomal P0 protein antibody were much higher in patients with arthritis , nephritis and specific skin lesion than in those without these disorders (P<0.05). Anti-ribosomal P0 antibody was not associated with SLEDAI and CLASI(P=0.012). Conclusions There is no difference of serum anti-ribosomal P0 antibodies between healthy controls and DLE patients. SLE patients have higher level of serum anti-ribosomal P0 antibody , specially in those with specific skin lesion.

Prognosis of patients with systemic lupus erythematosus and discoid lesions / Prognostico de pacientes com lupus eritematoso sistemico e lesoes discoides

BACKGROUND: It has been observed that patients with systemic lupus erythematosus and discoid lesions have a milder systemic disease. OBJECTIVE: To compare the clinical, demographic and autoantibody profile of systemic lupus erythematosus patients with and without discoid lesions. METHODS: We carried out a retrospective study involving 288 systemic lupus erythematosus patients who met at least four classification criteria of the American College of Rheumatology for systemic lupus erythematosus, comparing the clinical, serological and demographic factors between patients with and without discoid manifestations. RESULTS: Of the 288 patients, 13.8% had discoid lesions. Univariate analysis found no differences in the prevalence of malar rash, photosensitivity, arthritis, serositis, leukopenia, lymphopenia and hemolytic anemia or anemia of the central nervous system (p = ns). Renal lesions were more common in those without discoid lesions (p =0.016), and hemolysis (p<0.0001) was more common in those with discoid lesions. Regarding the profile of autoantibodies, only the anti-RNP antibody was more common in those with discoid events (p =0.04). In a logistic regression study, only the renal lesions and anti-RNP maintained their associations with discoid manifestations. CONCLUSION: Patients with lesions of systemic lupus erythematosus and discoid lesions have lower prevalence of renal involvement and a greater presence of anti RNP. .

FUNDAMENTOS: Existe a observação de que pacientes com lúpus eritematoso sistêmico e lesões discoides têm uma doença sistêmica mais branda. OBJETIVO: Comparar o perfil clínico, demográfico e de autoanticorpos de pacientes com lúpus eritematoso sistêmico com e sem lesões discoides. MÉTODOS: Estudo retrospectivo de 288 pacientes com lúpus eritematoso sistêmico que satisfizeram pelo menos 4 critérios classificatórios do Colégio Americano de Reumatologia para o diagnóstico de lúpus eritematoso sistêmico, comparando-se os achados clínicos, sorológicos e demográficos entre pacientes com e sem manifestações discoides. RESULTADOS: Dos 288 pacientes, 13,8% tinham lesões discoides. Na análise univariada não se encontraram diferenças quanto à prevalência de eritema malar, fotossensibilidade, artrite, serosite, leucopenia, linfopenia e anemia hemolítica ou de sistema nervoso central (p=ns). Lesões renais foram mais comuns naqueles sem lesão discoide (p =0,016), e a hemólise (p<0.0001) foi mais comum nos com lesão discoide. No que se refere ao perfil de autoanticorpos apenas o anticorpo anti-RNP foi mais comum naqueles com manifestações discoides (p=0,04). Em estudo por regressão logística, só as lesões renais e o anticorpo anti-RNP mantiveram suas associações com manifestações discoides. CONCLUSÃO: Pacientes de lúpus eritematoso sistêmico com lesões discoides têm menor prevalência de envolvimento renal e uma maior presença do anticorpo anti RNP. .

Follicular red dots: a normal trichoscopy feature in patients with pigmentary disorders? / Pontos vermelhos foliculares: um achado tricoscópico normal em pacientes com distúrbios da pigmentação?

Follicular red dots have been described as a trichoscopic feature of active discoid lupus erythematosus of the scalp and its presence associated with a better prognosis. We report five patients with pigmentary disorders in whom follicular red dots were detected during scalp examination. We suggest that this pattern is probably related to the rich vasculature that naturally envelops the normal hair follicle. The possible implications of such proposition in cases of discoid lupus erythematosus and other scalp disorders are also discussed.

Pontos vermelhos foliculares foram descritos como achado tricoscópico de lupus eritematoso discóide do couro cabeludo em atividade e a presença destes associado a melhor prognóstico. Relatamos cinco pacientes com distúrbios da pigmentação nos quais pontos vermelhos foliculares foram detectados ao exame do couro cabeludo. Sugerimos que este padrão está provavelmente relacionado à rica vascularização que naturalmente envolve o folículo piloso normal. As possíveis implicações desta suposição nos casos de lupus eritematoso discóide e outras doenças do couro cabeludo também são discutidas.

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.

Lúpus eritematoso crônico discoide nas linhas de Blaschko / Linear chronic discoid lupus erythematosus following the lines of Blaschko

O lúpus eritematoso crônico discoide linear é manifestação rara da doença lúpica cutânea, em que lesões eritêmato-atrófico-discrômicas dispõem-se nas linhas de Blaschko. Descrevemos o caso de um menino de 15 anos, com dois anos de história de lesões discoides eritêmato-atróficas, hipo e hiperpigmentadas, dispostas nas linhas de Blaschko do membro superior direito. O exame histopatológico revelou atrofia da epiderme, hiperqueratose, rolhas córneas, espessamento da zona da membrana basal, infiltrado inflamatório crônico perianexial e perivascular superficial e profundo, depósito de mucina na derme, confirmando o diagnóstico. Há, no total, 14 casos descritos dessa variante que se inicia frequentemente na infância e que não apresenta predomínio quanto ao sexo. As lesões ocorrem preferencialmente na face. Não há relatos de associação com doença sistêmica.

Linear chronic discoid lupus erythematosus is a rare manifestation of cutaneous lupus in which erythematous, atrophic, dyschromic lesions are located along the lines of Blaschko. This report describes the case of a 15-year old boy with a 2-year history of discoid, erythematous, hyper and hypopigmented lesions with central atrophy, situated along the lines of Blaschko on his right arm. Histopathology showed epidermal atrophy, hyperkeratosis, follicular plugging, thickening of the basement membrane zone, and superficial and deep chronic perivascular and periadnexal inflammatory infiltrate, with dermal mucin deposit, thus confirming diagnosis. A total of 14 cases have been described of this variant, the onset of which is often in childhood. There is no difference in incidence between genders. Lesions most commonly develop on the face. There have been no reports of any association with systemic disease.

Padrão dermatoscópico das alopecias cicatriciais causadas por lúpus eritematoso discoide e líquen plano pilar / Dermoscopy patterns of cicatricial alopecia resulting from discoid lupus erythematosus and lichen planopilaris

FUNDAMENTOS: A dermatoscopia é método importante de diagnóstico de doenças melanocíticas benignas e malignas. Recentemente, o uso desse método tem demonstrado grande ajuda também no diagnóstico e acompanhamento das alopecias. OBJETIVO: Descrever e demonstrar os achados dermatoscópicos de pacientes com quadros clínicos e histopatológicos de alopecia cicatricial. MÉTODOS: Estudo transversal descritivo em que foram selecionados, pelos achados clínicos e histopatológicos do couro cabeludo, 14 pacientes com alopecia cicatricial, sendo quatro casos de líquen plano pilar clássico, cinco casos de alopecia fibrosante frontal e cinco com lúpus eritematoso discoide. Os pacientes foram avaliados com videodermatoscópio e dermatoscópio manual por três examinadores diferentes, de forma independente. Os aumentos variaram de 10 a 70 vezes. RESULTADOS: Foram achados predominantes no lúpus eritematoso discoide placas brancas, capilares arboriformes, tampões ceratósicos e áreas com diminuição dos óstios foliculares; no líquen plano pilar clássico, escamas perifoliculares, pontos brancos e diminuição de óstios foliculares; na alopecia fibrosante frontal, diminuição de óstios foliculares, escama e eritema perifolicular, além de capilares arboriformes. As estruturas azul-acinzentadas, demonstradas neste estudo, não foram descritas na literatura. CONCLUSÕES: O uso da dermatoscopia na avaliação clínica das alopecias ajudou a estabelecer elementos semióticos, melhorou a capacidade de diagnóstico em relação à simples inspeção e revelou novas características das alopecias cicatriciais.

BACKGROUND: Dermoscopy is an important tool for the diagnosis of benign and malignant melanocytic diseases. Recently, this method has also been found to be extremely useful in the diagnosis and follow-up of alopecias. OBJECTIVE: The objective of this study was to describe dermoscopic findings in patients with clinical and histopathological characteristics of cicatricial alopecia. METHODS: A descriptive cross-sectional study was conducted in which 14 patients with cicatricial alopecia were selected based on clinical and histopathological evaluation of the scalp. The underlying cause was classic lichen planopilaris in four cases, frontal fibrosing alopecia in five and discoid lupus erythematosus in the remaining five. The patients were evaluated using videodermoscopy and conventional dermoscopy (with a handheld dermoscope), performed independently by three different examiners. Magnification ranged from 10x to 70x. RESULTS: Principal findings in cases of discoid lupus erythematosus were: white patches, branching capillaries, keratin plugs and areas of reduced follicular ostia; in classic lichen planopilaris: perifollicular scales, white dots and reduced follicular ostia; and in frontal fibrosing alopecia: reduced follicular ostia, perifollicular scales, perifollicular erythema and branching capillaries. The blue-grey dots described in this paper were a novel feature in scalp dermoscopy. CONCLUSIONS: The use of dermoscopy for the clinical evaluation of the scalp in cases of cicatricial alopecia improves diagnostic capacity beyond simple clinical inspection and reveals novel features of the disease.

Grupos sanguíneos e lúpus eritematoso crônico discoide / Blood groups and discoid lupus erythematosus

FUNDAMENTOS: Lesão discoide é a manifestação cutânea mais comum do lúpus eritematoso, e formas cutâneas crônicas apresentam características imunológicas próprias, direcionadas ao polo Th1. Diversas doenças possuem associação com grupos sanguíneos, o que não foi ainda estudado no lúpus discoide. OBJETIVO: Investigar a associação entre tipos sanguíneos (ABO e Rh) e lúpus eritematoso discoide. MÉTODOS: Estudo prospectivo tipo transversal envolvendo tipagem sanguínea ABO e Rh, inquérito de dados clínicos e dosagem de FAN e C4 de portadores de lúpus discoide sem critérios de doença sistêmica, atendidos em hospital universitário. RESULTADOS: Foram incluídos no estudo 69 pacientes, sendo 71,0 por cento do sexo feminino (p 1:160, em 31,9 por cento; e níveis baixos de C4, em 8,7 por cento. Não houve diferença significativa entre as frequências dos grupos sanguíneos dos pacientes e da população local; entretanto, o grupo A foi associado às formas disseminadas da doença (OR 4,1 e p < 0,05). CONCLUSÕES: Grupos sanguíneos de pacientes com lúpus discoide apresentam frequência semelhante à da população; porém, formas clínicas disseminadas foram mais prevalentes entre portadores do grupo A.

Background: Discoid lesion is the commonest cutaneous finding in lupus erythematosus and chronic types have their own immunological features, with Th1 inflammation profile. Although many diseases have association with blood-group systems, this fact was not enlightened in discoid lupus erythematosus. Objective: To investigate the association between blood groups (ABO and Rh) and discoid lupus erythematosus. Methods: A prospective cross-sectional study assessing clinical information, blood group systems (ABO and Rh), FAN and C4 serum levels from discoid lupus patients without characteristics of systemic disease, was carried out at a clinic from a Brazilian university hospital. Results: Sixty-nine patients were enrolled in the study, 71.0 percent were females (p1:160 in 31.9 percent, and low levels of C4 in 8.7 percent. There was no significant difference between the frequency of blood groups from discoid lupus patients and local population, however, blood group A was associated to disseminate forms of the disease (OR 4.1 and p < 0.05). Conclusions: Discoid lupus erythematosus patients with ABO and Rh blood groups exhibit similar frequencies as in the general population; nevertheless, disseminate clinical forms were more prevalent among group A patients.

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.

Study on the expression of high mobility group box chromosomal protein 1 in skin lesions in patients with lupus erythematosus / 中华风湿病学杂志

Objective To study the expression of high mobility group box chromosomal protein 1(HMGB-1) in the skin lesions of patients with lupus erythematosus and investigate the role of HMGB-1 in the pathogenesis of lupus erythematosus. Methods Immunohistochemical assay and Western-blot were used to test the expression of HMGB-1 in skin lesions from 20 discoid lupus erythematosus (DLE) patients, 25 systemic lupus erythematosus (SLE) patients and 20 healthy controls. Results In healthy controls, H MGB-1 was mainly expressed in the nucleus of keratinocytes. In skin lesions of DLE patients, HMGB-1 was mainly expressed in mononuclear cells of dermis, but the percentage of positive keratinocytes in epidermis of lesion area was decreased than that of healthy controls (t=11.315, P<0.01). In skins that were not involved,HMGB-1 was also expressed in the nucleus of keratinocytes and there was no difference in the percentage of positive keratinocytes between DLE and healthy controls (P>0.05). By Western-blot, there was no stati-stical significance in total protein between DLE and healthy controls (t=0.681, P>0.05). In SLE, besides the mononuclear cells, HMGB-1 could be detected both in the cytoplasmic and extracellular space in dermis,while the HMGB-1 nuclear expressions in keratinocytes'of epidermis were decreased than those of DLE (t=6.821, P<0.01), and in un-involved skin, HMGB-1 was also expressed in the nucleus of keratinocytes and there was no difference in the percentage of positive keratinocytes with healthy controls (P>0.05). The total protein was increased in SLE than that of healthy controls and DLE patients (t=15.494, P<0.01 ; t=13.221, P<0.01, respectively). The intensity of HMGB-1 was con'elated with SLEDAI and proteinuria (r=0.565, P<0.01,OR=1.027, P<0.05, respectively). Conclusion Compared with healthy controls, there is translocation and alteration of HMGB-1 expression in patients with lupus erythematosus, which indicates that HMGB-1 may be involved in the inflammation of lupus erythematosus.

Lupus eritematoso discóide na infância: [revisão] / Discoid lupus erythematosus in childhood: [review]

OBJETIVO: Realizar revisão da literatura sobre o lupus eritematoso discóide (LED) na infância. FONTES DE DADOS: Livros-texto e artigos de revistas indexadas pelo Medline e SciELO nos últimos dez anos, usando as seguintes palavras-chave: "discoid lupus erythematosus", "chronic cutaneous lupus erythematosus", "lupus erythematosus in childhood", "lupus erythematosus in children", "discoid lupus erythematosus in childhood", "discoid lupus erythematosus in children". SÍNTESEDOS DADOS: A idade de início da doença ocorre predominantemente entre cinco e dez anos e a história familiar de lupus eritematoso está presente em 11 a 35 por cento dos casos. A relação gênero feminino/masculino varia de 1/1 a 2,4/1. Por sua vez, 24 a 27 por cento dos pacientes com LED desenvolvem lupus eritematoso sistêmico (LES). Lesões discóides localizadas (que acometem cabeça e pescoço) são observadas em 56 a 75 por cento dos pacientes. A face é o local mais acometido. O LED localizado e o generalizado apresentam evolução semelhante. Os achados histológicos são típicos, mostrando dermatite de interface. IgM e IgG são os depósitos mais freqüentes na zona da membrana basal da epiderme. Os tratamentos geralmente utilizados são: fotoproteção, corticosteróides tópicos e antimaláricos. Imunossupressores, talidomida, dapsona e retinóides podem ser usados nos casos refratários. CONCLUSÕES: O LED da infância parece ter pequeno predomínio no gênero feminino, alta prevalência de história familiar de lupus eritematoso e elevada proporção que evolui para a forma sistêmica da doença, comparada ao LED do adulto. O LED localizado e o generalizado apresentam prognósticos semelhante. Os achados histológicos não foram diferentes daqueles descritos no LED do adulto.

OBJECTIVE: To review the literature about discoid lupus erythematosus (DLE) in childhood. DATA SOURCES: Textbooks and journals indexed for Medline and SciELO in the last ten years. The following key-words were searched: "discoid lupus erythematosus", "chronic cutaneous lupus erythematosus", "lupus erythematosus in childhood", "lupus erythematosus in children", "discoid lupus erythematosus in childhood", "discoid lupus erythematosus in children". DATA SYNTHESIS: Disease onset occurs predominantly between five and ten years old. Family history of lupus erythematosus is present in 11-35 percent of cases. The female/male ratio varies from 1/1 to 2.4/1. Development of systemic lupus erythematosus (SLE) is present in 24-27 percent of patients. Localized discoid lesions are observed in 56-75 percent of patients. The face is the most frequent site of involvement. Localized and generalized DLE have a similar course. The histological findings are typical, with interface dermatitis. IgM and IgG are the most frequent deposits in the basement membrane zone. The treatments usually used are: sunscreens, topical corticosteroids and antimalarials. Immunosuppressive agents, thalidomide, dapsone and retinoids can be used in refractory cases. CONCLUSIONS: Childhood DLE shows slight female predominance, high prevalence of familiar history of lupus erythematosus, and it evolves to systemic lupus erythematosus in a higher proportion than adult DLE. Disseminated DLE and localized DLE have similar prognosis. The histological findings did not differ from those described in adults.