ABSTRACT
Objective To summarize the clinical features,treatment and prognosis of bullous systemic lupus erythematosus (BSLE).Methods Clinical data were collected from 6 cases of BSLE between May 2009 and September 2015,and were analyzed retrospectively.Results Among the 6 patients,2 were male and 4 were female.The mean age of onset was 34 years.All the 6 patients presented with tense blisters and bullae arising on an erythematous base or normal skin,which were arranged in an annular pattern in 3 patients.The SLE Disease Activity Index (SLEDAI) score was ＞ 4 in all the 6 patients.Histopathological examination showed subepidermal blisters or fissures in all the patients.Direct immunofluorescence (DIF) revealed continuous linear deposition of immunoglobulin G (IgG),IgM and IgA in all the patients,linear deposition of C3 in 4 patients,and linear deposition of Clq in 2 patients at the basement membrane zone (BMZ).All the 6 patients were treated with oral glucocorticoids and hydroxychloroquine,and 2 patients were additionally treated with cyclophosphamide.During the follow-up period,adverse reactions to different extents were observed in the 6 patients.Conclusions BSLE mainly occurs in young and middle-aged people.Histopathologically,subepidermal blisters or fissures can be observed with linear deposition of IgG,IgM,IgA,or C3 along the basement membrane zone on DIF.
ABSTRACT
Bullous systemic lupus erythematosus (SLE) is a kind of LE-non-specific bullous skin disease that is rarely induced by a medication. We describe the first case of bullous SLE to develop after administration of methimazole. A 31-yr-old woman presented with generalized erythematous patches, multiple bullae, arthralgia, fever, conjunctivitis, and hemolytic anemia. Biopsy of her bulla showed linear deposition of lgG, lgA, C3, fibrinogen, and C1q at dermo-epidermal junction. She was diagnosed as bullous SLE and treated with prednisolone, dapsone, hydroxychloroquine, and methotrexate. Our experience suggests that SLE should be considered as a differential diagnosis when bullous skin lesions develop in patients being treated for hyperthyroidism.
Subject(s)
Adult , Female , Humans , Anti-Inflammatory Agents/therapeutic use , Antirheumatic Agents/therapeutic use , Antithyroid Agents/adverse effects , Blister/chemically induced , Drug Therapy, Combination , Graves Disease/diagnosis , Hydroxychloroquine/therapeutic use , Immunosuppressive Agents/therapeutic use , Lupus Erythematosus, Systemic/chemically induced , Lupus Nephritis/diagnosis , Methimazole/adverse effects , Mycophenolic Acid/analogs & derivatives , Prednisolone/therapeutic use , Skin/pathologyABSTRACT
Bullous systemic lupus erythematosus (bullous SLE) is uncommon; distinctive clinical variant of SLE that is characterized by tense vesicles and bullae filled with fluid that occurs on either erythematous or normal skin. Although bullous SLE can accompany erythematous plaque with annular configuration, urticarial papules, and erythema multiforme (EM)-like lesions initially, there is no report of EM-like lesions as an initial sign of bullous SLE in the Korean literatures. Herein, we describe 31-year-old women with bullous SLE that showed EM-like skin lesions as the initial sign.
Subject(s)
Adult , Female , Humans , Blister , Erythema , Erythema Multiforme , Lupus Erythematosus, Systemic , Skin , Transcutaneous Electric Nerve StimulationABSTRACT
Bullous systemic lupus erythematosus (bullous SLE) is uncommon; distinctive clinical variant of SLE that is characterized by tense vesicles and bullae filled with fluid that occurs on either erythematous or normal skin. Although bullous SLE can accompany erythematous plaque with annular configuration, urticarial papules, and erythema multiforme (EM)-like lesions initially, there is no report of EM-like lesions as an initial sign of bullous SLE in the Korean literatures. Herein, we describe 31-year-old women with bullous SLE that showed EM-like skin lesions as the initial sign.
Subject(s)
Adult , Female , Humans , Blister , Erythema , Erythema Multiforme , Lupus Erythematosus, Systemic , Skin , Transcutaneous Electric Nerve StimulationABSTRACT
El lupus ampolloso es una manifestación poco frecuente del lupus eritematoso sistémico (LES). Otras enfermedades ampollosas tales como el penfigoide ampolloso, epidermólisis ampollosa adquirida, dermatosis ampollosa Ig A lineal y dermatitis herpetiforme también han sido informadas en LES. Describimos un paciente que desarrolló lesiones ampollosas 14 días luego de iniciar terapia con altas dosis de glucocorticoides y ciclofosfamida para manifestaciones severas del LES (nefritis y hemorragia alveolar). Se confirmó el diagnóstico de lupus ampolloso. La respuesta al tratamiento con dapsona fue notable a las 48 horas. En este artículo revisamos la epidemiología, hallazgos clínicos, histopatológicos e inmunopatológicos; el diagnóstico diferencial y el tratamiento del LES ampolloso.
Bullous systemic lupus erythematosus (BSLE) is an unusual finding in systemic lupus erythematosus (SLE). Other bullous disorders such as bullous pemphigoid, epirdermolysis bullosa acquisita, linear IgA bullous dermatosis and dermatitis herpetiformis has also been reported in association with SLE. We described a patient who developed severe bullous lesions 14 days after high-dose systemic glucocorticoids and cyclophosphide therapies were initiated for severe SLE manifestations (nephritis and alveolar hemorrhage). A diagnosis of bullous SLE was made. Therapy with dapsone resulted in a marked clinical improvement of the bullous eruption within 48 hours. This article also discusses the epidemiology, clinical, histopathologic and immunopathologic features, differential diagnosis and the treatment of BSLE.
Subject(s)
Humans , Male , Adult , Therapeutics , Dapsone , Lupus Erythematosus, Systemic , Epidemiology , Cyclophosphamide , Diagnosis , GlucocorticoidsABSTRACT
O objetivo deste relato é alertar a comunidade médica sobre o raro desdobramento do lúpus eritematoso sistêmico em lúpus eritematoso sistêmico bolhoso como manifestação inicial do lúpus e associado à nefrite lúpica classe V da Organização Mundial da Saúde. Descreve-se o caso de uma paciente de 16 anos de idade que procurou o Centro de Pediatria do Hospital Universitário de Brasília com queixa de febre, edema palpebral matutino, exantema maculopapulovesicular nas regiões palmares e plantares e manifestações de artrite. Iniciada pulsoterapia com metilprednisolona, a doente apresentou exantema vesicobolhoso nas mucosas e em áreas difusas da pele. Houve melhora excelente do quadro após administração de dapsona para o tratamento de lúpus eritematoso sistêmico bolhoso.
The objective of this case report is to alert the medical community to the rare development from systemic lupus erythematosus to bullous systemic lupus erythematosus in the first display of lupus added to lupus nephritis level V from World Health Organization. We report the case of a 16 year-old female adolescent admitted to the Pediatric emergency unit of the Hospital Universitário de Brasília reporting fever, morning lid edema, symptoms of arthritis, macule-papule-vesicle exanthema in hands and feet. After the methylprednisolone pulse the patient started to present vesicobullous exanthema in mucosae and at diffuse areas of the skin, markedly improving when, after suspecting of bullous systemic lupus erythematosus, dapsone was administered.
Subject(s)
Humans , Female , Adolescent , Exanthema , Lupus Erythematosus, Systemic/diagnosis , Lupus Nephritis , Connective Tissue , BlisterABSTRACT
Bullous systemic lupus erythematosus (bullous SLE) is an uncommon, distinctive clinical variant of systemic lupus erythematosus (SLE), histopathologically characterized by subepidermal blisters with neutrophil infiltration and immunopathologically linear IgG depositon at the dermoepidermal junction. A blistering eruption may occur during flares of SLE such as lupus nephritis. We report two cases of bullous SLE, confirmed by the clinical, histopathological and immunopathological features. In one patient who has been diagnosed as SLE with diffuse proliferative lupus nephritis (WHO class IV), a blistering eruption occurred during the course of treatment of SLE. In the other patient, the bullous eruption was the initial manifestation of SLE and then she was diagnosed as SLE. We review the recent literature and describe the distinctive features of this rare disorder.
Subject(s)
Humans , Blister , Immunoglobulin G , Lupus Erythematosus, Systemic , Lupus Nephritis , Neutrophil InfiltrationABSTRACT
We describe two female patients with bullous eruption of systemic lupus erythematosus(SLE). In these patients several vesiculobullous lesions occurred on the extensor surface of the hands and feet; in one patient, additional lesions of erythematous patches and vesicles were observed along the belt-line on her waist. The occurrences of mechanobullous lesions at the areas of trauma or pressure without having any spontaneous blister in these cases may be considered to be an unusual clinical finding in bullous SLE.
Subject(s)
Female , Humans , Blister , Foot , Hand , Lupus Erythematosus, SystemicABSTRACT
A 21-year-old woman, who had a one-year history of pancytopenia with histiocytic necrotizing lymphadenitis and hepatosplenomegaly, presented with a 5 day history of tense bullae, which were localized on the face. These clusters of tense bullae occurred on clinically normal skin, she did not have other skin lesions. A diagnosis of bullous systemic lupus erythematosus (BSLE) was established based on clinical, laboratory, histological, and immunological findings. The bullae showed good responses to dapsone (100mg, daily) and resolved within 10 days with-out scaring. Bullous lesions of SLE may be the first cutaneous manifestation in some patients with SLE and should be considered in the differential diagnosis of the other subepidermal bullous disorders.
Subject(s)
Female , Humans , Young Adult , Blister , Dapsone , Diagnosis , Diagnosis, Differential , Histiocytic Necrotizing Lymphadenitis , Lupus Erythematosus, Systemic , Pancytopenia , Skin , Transcutaneous Electric Nerve StimulationABSTRACT
We describe a 19-year-old girl showing typical laboratory and clinical features of bullous systemic lupus erythematosus(BSLE). Clinically she has suffered from recurrent vesiculobullous skin eruptions for 7 years. The bullous eruption has not been known as the initial manifestation of SLE until she was diagnosed as SLE. These cutaneous lesions have waxed and waned with oral prednisolone with or without dapsone. Bullous SLE should be considered in the differential diagnosis of children presenting with generalized bullous eruptions.