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1/8. Severe subacute cutaneous lupus erythematosus presenting with generalized erythroderma and bullae.

    A 31-year-old woman presented with progressive generalized erythroderma and bullae. Histologic evaluation revealed dyskeratosis and interface dermatitis with a paucity of infiltrate. Serologic evaluation revealed markedly elevated titer of Ro/SS-A and La/SS-B antibodies. Further workup revealed leukopenia. The generalized eruption cleared with prednisone. The patient later had the classic discrete lesions of subacute cutaneous lupus erythematosus develop. The erythrodermic and bullous presentation of subacute cutaneous lupus erythematosus is rare and requires a high index of suspicion.
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ranking = 1
keywords = lupus erythematosus, erythematosus, lupus
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2/8. Bullous subacute cutaneous lupus erythematosus.

    We describe a 59-year-old woman, with a history of autoimmune disease and disseminated uterine leiomyosarcoma, who developed a photoaggravated, blistering skin eruption. An initial rash, at the outset of treatment with chemo- and radiotherapy, resembled erythema multiforme. review of the original skin biopsy showed it to be subacute cutaneous lupus erythematosus. There were no systemic symptoms or signs to suggest systemic lupus erythematosus. The much later photoaggravated rash consisted mainly of bullae and eventual epidermal denuding which resembled toxic epidermal necrolysis. We propose that the clinical and histological diagnosis is one of bullous subacute cutaneous lupus erythematosus in a patient with no other features of systemic lupus erythematosus.
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ranking = 1.3653316719555
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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3/8. Photoaging and phototoxicity from long-term voriconazole treatment in a 15-year-old girl.

    Voriconazole is a second-generation triazole that was approved by the food and Drug Administration in May 2002 for treatment of severe fungal infections. In clinical trials it demonstrated superior efficacy in addition to a survival benefit when compared with the then current treatment standard, amphotericin b, for primary treatment of invasive aspergillosis. Voriconazole is a highly selective inhibitor of fungal cytochrome P450 enzymes. Adverse cutaneous reactions have been reported, namely cheilitis, erythema, discoid lupus erythematosus, stevens-johnson syndrome, toxic epidermal necrolysis, erythema multiforme, and photosensitivity reactions. We report a case of photoaging caused by voriconazole therapy. A 15-year-old patient developed cheilitis and erythema over the sun-exposed areas of her body 5 weeks after beginning voriconazole for a severe fungal infection. The lesions showed a mild transient improvement before subsequent photodamage occurred to the back of her forearms, back of her hands, and face. Voriconazole was discontinued once the fungal infection had completely resolved. The patient's blisters, erythema, and cheilitis resolved after discontinuation of voriconazole. However, she was left with solar elastotic changes, multiple lentigines, and ephelides of sun-exposed areas. These cutaneous manifestations may represent a unique adverse event caused by a new second-generation triazole.
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ranking = 0.16666666666667
keywords = lupus erythematosus, erythematosus, lupus
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4/8. Bullous systemic lupus erythematosus: an unusual clinical course and detectable circulating autoantibodies to the epidermolysis bullosa acquisita antigen.

    Bullous systemic lupus erythematosus is a newly recognized form of systemic lupus erythematosus characterized by a skin eruption clinically and histologically resembling dermatitis herpetiformis and responsive to dapsone. We report on a patient with bullous systemic lupus erythematosus who initially presented with lesions clinically resembling erythema multiforme, experienced exacerbation of her disease with dapsone, and had detectable circulating autoantibodies to the epidermolysis bullosa acquisita antigen.
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ranking = 1.2786608518442
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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5/8. Bullous retinal detachment associated with renal failure: case report.

    A case of bilateral bullous retinal detachment in a patient with long-standing disseminated lupus erythematosus is presented. Although several authors have reported the presence of bullous retinal detachment in association with chronic renal disease, in no other case have the ocular findings preceded the onset of frank renal failure. The etiology of nonrhegmatogenous detachment and uveal effusion in association with chronic renal disease was discussed and the possible contributory role of hypertension, electrolyte imbalance, and renal glomerular malfunction was investigated. The additional finding of late onset bilateral keratoconus is, in all probability, unrelated to the patient's retinal findings, though it is a major factor in her present visual morbidity. The purpose of this paper is twofold. First-ly, to alert the ophthalmologist to the possible development of bullous retinal detachment in patients with chronic renal disease, and secondly, to alert the internist to the possibility of impending renal failure in patients with such bilateral nonrhegmatogenous detachments.
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ranking = 0.16666666666667
keywords = lupus erythematosus, erythematosus, lupus
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6/8. Evidence supporting a role for immune complex-mediated inflammation in the pathogenesis of bullous lesions of systemic lupus erythematosus.

    Evidence supporting an immune complex pathogenesis of bullous lesions in systemic lupus erythematosus includes immune deposits, acute inflammation, and blister formation at the cutaneous basement membrane zone. Since cutaneous immune deposits are a general feature of lupus, an attempt has been made to determine whether deposits in lupus patients with blisters are functionally different from those in patients without blisters. skin was obtained from 4 consecutive patients with blisters and 14 controls. The groups were matched for clinical and serologic features, duration and activity of disease, and treatment. skin was examined by direct immunofluorescence for immune deposits and by the leukocyte attachment assay for quantification of complement-activating immune complexes. Clinically normal, viable skin from 1 patient with blisters and 1 patient without blisters was incubated in organ culture with normal human leukocytes and serum complement. All patients in both groups had immune deposits at the basement membrane zone with an equivalent incidence of the major Ig classes. Deposits in patients with blisters were slightly more intense and a linear pattern of fluorescence seen in 75% of these patients was not seen in controls. The leukocyte attachment assay showed significantly greater (p less than .02) cell attachment in patients with blisters (mean = 167) than in patients without blisters (mean = 64) and greater cell attachment in peribullous than normal skin from the same patient. Organ culture showed complement-dependent migration of leukocytes and histologic features similar to those in spontaneous lesions in skin from the patient with blisters but not in skin from the patient without blisters. These results provide evidence supporting immune complex and complement-dependent inflammation in the pathogenesis of bullous lesions in systemic lupus erythematosus.
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ranking = 1.1124866370838
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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7/8. Cutaneous bullae following acute steroid withdrawal in systemic lupus erythematosus.

    A patient with systemic lupus erythematosus developed severe widespread bullae following sudden steroid withdrawal. Histologically the lesion was suggestive of leukocytoclastic vasculitis with a regenerating subepidermal bulla, but immunofluorescence on non-lesional skin was typical of lupus erythematosus. Treatment led to resolution of the bullous eruption, and despite exacerbations of the patient's lupus nephropathy, the blisters have not recurred. The differential diagnosis of bullous LE is discussed.
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ranking = 1.0882416571641
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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8/8. Localized linear bullous eruption of systemic lupus erythematosus in a child.

    A 9-year-old girl newly diagnosed with systemic lupus erythematosus (SLE) developed a localized linear papulovesicular eruption over the right dorsal hand and ulnar forearm. The skin findings were clinically suggestive of herpes zoster, lichen striatus, or lichen planus-lupus erythematosus overlap. However, histologic, immunofluorescent, immunoelectron microscopic, and immunoblot studies revealed findings compatible with bullous SLE. Our patient is noteworthy because she is the first one reported with bullous SLE presenting in a localized linear pattern. She is also the second-youngest reported patient with bullous SLE.
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ranking = 1.0799958465554
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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