Bullous lesions can occur in systemic lupus erythematosus sle as a subepidermal blistering disease 1 or when severe edema and hydropic. Dapsone was initiated while awaiting the result of skin biopsies. Pdf bullous systemic lupus erythematosus bsle is a rare manifestation of systemic lupus erythematosus sle, characterized histologically by. Bullous systemic lupus erythematosus sle is a transient autoimmune bullous disease that occurs in. Topical and systemic corticosteroids are used initially. Bullous systemic lupus erythematosus a case report ncbi nih. A bullous lupus associated with cutaneous leishmaniasis. In a large cohort of sera taken from patients with immunobullous disorders, 12% were identified as bullous sle 1,2. In a large cohort of sera taken from patients with immunobullous disorders, 12% were identified as bullous sle 1,2 like sle, bullous sle has been reported most commonly in women of african descent in their thirties. Because of the association of egr with malignancy, abbreviations used. We report a case of bullous systemic lupus erythematosus sle. Bullous pemphigoid is an autoimmune pruritic skin disease preferentially in older people, aged over 60, that may involve the formation of blisters in the space between the epidermal and dermal skin layers.
An erythema gyratum repens variant of bullous lupus. Vesicles form not only on the le erythema but also on the normal looking skin. The second is vasculitis with subepidermal blister and pustule formation. Bullous lupus erythematosus as a cutaneous manifestation. Nov 21, 2007 bullous systemic lupus erythematosus sle is a rare, distinctive subepidermal blistering disorder that occurs in systemic lupus erythematosus. Bullous systemic lupus erythematosus bsle is a rare cutaneous presentation of systemic lupus erythematosus sle. Methimazoleinduced bullous systemic lupus erythematosus. Bullous systemic lupus erythematosus bsle is an uncommon. The histology may be identical with discrimination only possible where linear igg and igm is found at the basement membrane. Review article treatment of bullous systemic lupus. They encompass many specific disorders, including the bullous pemphigoid bp, pemphigoid gestationis pg, linear iga bullous dermatosis lad, dermatitis herpetiformis dh, epidermolysis bullosa acquisita eba, bullous lupus erythematosus ble, lichen planus pemphigoides lpp, and bullous. Multiple blistering eruption on face, trunk and oral mucosa for 1.
It has characteristical clinic, pathologic, and immunologic findings including antibodies to type vii collagen, laminin 332, laminin 331, and bullous pemphigoid antigen 230. In 1981, gilliam and sontheimer described a morphological classification scheme to organize cutaneous le as lupus specific or lupus aspecific based on characteristic findings of interface dermatitis on histopathology in the former group. As its name indicates, pemphigoid is similar in general appearance to pemphigus, but, unlike pemphigus, pemphigoid does not feature acantholysis, a loss of connections between skin cells pemphigoid is more common than pemphigus, and is slightly more common in women than in men. Full text vesiculobullous diseases in relation to lupus. Bullous systemic lupus erythematosus sciencedirect. Apr 20, 2017 given the evolution of her skin lesions despite steroids, the differential diagnosis included infectious vs. Sep, 2011 all these presentations represent specific lupus disease occurring on the vermilion. Dec 30, 2020 bullous systemic lupus erythematosus bsle is a rare autoimmune blistering disorder that typically manifests as an acute vesiculobullous eruption in a patient it seems to us that you have your javascript disabled on your browser. Bullous systemic lupus erythematosus bsle is a rare autoimmune blistering disease in patients with a known diagnosis of systemic lupus erythematosus sle. It is characterized clinically by a pemphigoidlike eruption with tense fluidfilled vesicles and bullae, often with a background of maculopapular or urticated erythema. Although cutaneous manifestations are common in lupus 5985%, bsle is rare, affecting bullous dermatosis which resembles pct but normal levels of porphyrins in serum, urine and feces drug induced pathology in patients with chronic renal failure undergoing hemodialysis therapyinduced bullous photosensitivity histology.
Autoimmunity in bsle is characterized by the presence of circulating antitype vii collagen antibodies. It is a heterogeneous disease that is caused by autoantibodies to the dermoepidermal junction, mainly type vii collagen. Review article treatment of bullous systemic lupus erythematosus lihuaduan, 1 liyingchen, 1 shanzhong, 2 yingwang, 1 yanhuang, 1 yanhe, 1 jiechen, 3 andguixiushi 1 department of rheumatology and clinical immunology,e first hospital of xiamen university, xiamen, china. Bullous lupus erythematosus bsle is a rare subset of systemic lupus erythematosus sle, often associated with autoimmunity to type vii collagen. Mar 01, 2004 bullous systemic lupus erythematosus with autoantibodies recognizing multiple skin basement membrane components, bullous pemphigoid antigen 1, laminin5, laminin6, and type vii collagen arch dermatol, 5 1999, pp.
Routine histopathology and immunoflorescence studies. Alerts and notices synopsis bullous systemic lupus erythematosus bsle is a rare autoimmune blistering disease in patients with a known diagnosis of systemic lupus erythematosus sle. First description of bullous lupus associated with. Pdf bullous systemic lupus erythematosus bsle is an autoantibodymediated vesiculobullous disease in patients with sle. Abstract bullous systemic lupus erythematosus bsle is a rare cutaneous complication of systemic lupus erythematosus sle. Pdf treatment of bullous systemic lupus erythematosus. While some valued the completeness of skin lupus symptoms, others found the list much too long. Bullous systemic lupus erythematosus is a rare distinctive subepidermal bullous disease seen in patients with systemic lupus erythematosus sle. Bullous pemphigoid is a chronic autoimmune skin disorder resulting in generalized, pruritic, bullous lesions in older patients. Article english pdf article in xml format how to cite this article scielo analytics curriculum scienti automatic translation.
Bullous systemic lupus erythematosus successfully treated. Bullous systemic lupus erythematosus bsle is a rare subtype of lupus erythematosus sle that is quite uncommon during infancy, childhood and adolescence. The incidence of bullous sle was estimated to be 0. Autoimmune subepidermal bullous diseases are a heterogeneous group of disorders. Bullous systemic lupus erythematosus bsle is an autoantibodymediated vesiculobullous disease in patients with sle. Bullous sle is a heterogeneous disease without pathognomonic clinical features, making the diagnosis and differentiation from other. Although most general approaches to the treatment and diagnosis of these entitie. Bsle patients often present with multiple, tense, clear fluidfilled vesicles and bullae overlying erythematous edematous plaques. Bullous systemic lupus erythematosus and cicatricial. In addition, blisters can occur due to the association of sle with other autoimmune blistering diseases e. Second, inflammatory cell component should be evaluated, which could vary due to age of the lesion.
The cutaneous pathology of lupus erythematosus wiley online. May 25, 2017 autoimmune bullous diseases mainly present with vesiculobullous reaction pattern. Clinical presentation combined with histopathology, immunological testing. Go back to the top to order, visit the purchasing page for details. Histomorphologic and direct immunofluorescence findings of. Pdf bullous systemic lupus erythematosus bsle is a rare manifestation of systemic lupus erythematosus sle, characterized.
Bullous systemic lupus erythematosus grover c, khurana a. Bullous systemic lupus erythematosus bsle is a rare autoimmune blistering disorder that typically manifests as an acute vesiculobullous eruption in a patient it seems to us that you have your javascript disabled on your browser. Epidermolysis bullosa acquisita eba is histopathologically and immunopathologically identical since both are mediated by circulating antibodies against type vii collagen. Similarities in histology and immunopathology exist between bsle and other primary. Oral lesions are believed to occur in 30% of patients with bullous le 6. First description of bullous lupus associated with cutaneous. Histopathology of cutaneous lupus erythematosus sciencedirect. The patient suddenly presented with a widespread blistering. Phototoxic bullous dermatosis which resembles pct but normal levels of porphyrins in serum, urine and feces drug induced pathology in patients with chronic renal failure undergoing hemodialysis therapyinduced bullous photosensitivity histology. Given the concentric morphology of the eruption, a diagnosis of an egr variant of bullous lupus erythematosus was made. Interestingly, histology also revealed the presence of numerous corpuscles of leishmania in the cytoplasm of histiocytes fig. An erythema gyratum repens variant of bullous lupus erythematosus. Comparison of histopathology, immunofluorescence, and.
Bullous systemic lupus erythematosus sle is a rare blistering disease with a distinctive combination of clinical, histologic and immunopathologic features that. Sera and frozen biopsy specimens taken for immunofluorescence can be of diagnostic value in studies of vesiculobullous eruptions and connective tissue diseases. Bullous systemic lupus erythematosus and cicatricial pemphigoid. Patients with bsle should be worked up for systemic involvement. Recent advances in our understanding of the pathophysiological basis of le bring hope for novel therapeutic approaches. The parasitological culture of the skin biopsy was positive, and mass spectrometry identi. Treatment of bullous systemic lupus erythematosus lihuaduan, 1 liyingchen, 1 shanzhong, 2 yingwang, 1 yanhuang, 1 yanhe, 1 jiechen, 3 andguixiushi 1. Pdf histopathological study of vesiculobullous lesions of. Bullous systemic lupus erythematosus bsle is a rare complication of systemic lupus erythematosus sle characterized by cutaneous vesicles and bullae with a primarily neutrophilic infiltrate on histopathology. Lupus erythematosus le is an autoimmune connective tissue disease whose precise aetiological basis is at present uncertain, in which the clinical presentations range from a skin rash unaccompanied by extracutaneous stigmata to one comprising progressive multisystem disease. Bullous cie epidermolytic hyperkeratosis incontinentia pigmenti autoimmune linear iga bullous dermatosis of childhood dermatitis herpetiformis bullous systemic lupus erythematosus epidermolysis bullosa acquisita bullous pemphigoid bp pemphigus folicaeous, vulgaris, drug induced pnp, iga herpes gestionis.
The clinical presentations range from a skin rash unaccompanied by extracutaneous. Cutaneous histopathology of lupus erythematosus diagnostic. Bullous systemic lupus erythematosus bsle is a rare complication of. Bullous systemic lupus erythematosus bioline international. Diagnosis of bullous pemphigoid includes clinical assessment, skin biopsy for histopathology and direct immunofluorescence, indirect immunofluorescence and elisa test. Blistering eruptions are rare cutaneous manifestations of lupus erythematosus le that may be caused by different mechanisms. Pemphigoid is a group of rare autoimmune blistering diseases of the skin, and mucous membranes. Immunoreactive on dermal side of salt split skin positive lupus serology neutrophil rich infiltrate dermatitis herpetiformis. In cases of pemphigus and pemphigoid, findings of typical antibodies in sera and. Apr 18, 2018 since the first description of discoid lupus erythematosus le in two dogs in 1979, the spectrum of canine cutaneous lupus erythematosus cle variants has expanded markedly. Bullous lesions as a manifestation of systemic lupus.
Uses for immunofluorescence tests of skin and sera. Bullous lesions as a manifestation of systemic lupus erythematosus. We present a case of a 17yearold adolescent girl who presented with. Multiple blisters along the lip vermilion are a clue to. Most of these diseases are associated with substantial morbidity, and a few may result in death. Bullous systemic lupus erythematosus sle is an uncommon manifestation of sle and is caused by autoantibodies against type vii collagen. Bullous systemic lupus erythematosus bsle is a rare cutaneous autoimmune disorder characterized by rapid, widespread vesiculobullous lesions in patients with systemic lupus erythematosus sle. The commonest lesion overall was bullous pemphigoid followed by pemphigus vulgaris. Lupus erythematosus le is an autoimmune disease with a wide range of clinical manifestations, from limited skin disease to multiorgan systemic involvement.
Jul 08, 2010 bullous lesions in sle due to photosensitivity, acute lupus or drugs can be differentiated by both histopathology and immunopathology. It is classified as a type ii hypersensitivity reaction, with the formation of antihemidesmosome antibodies. A case report bullous systemic lupus erythematosus sle is a kind of lenonspecific bullous skin disease that is rarely induced by a medication. When antibody capture is utilized, this is referred to as sandwich elisa because the target antigen is bound between the immobilizing antibody and the primary antibody. It can be the presenting symptom of sle in patients who do not carry a diagnosis. Painful erythematous or edematous nodules on acral skin following exposure to cold clinical context variable. Pdf histopathological study of vesiculobullous lesions. Generally, patients with bsle meet the criteria for sle as defined by the american college of rheumatology. Subepidermal clefting with frank vesiculation may occur in early lesions of chronic, subacute, and acutecutaneous le due to a severe vacuolar alteration of the dermoepidermal junction dej, dermal edema, and lekocytoclastic vasculitis. Pdf bullous systemic lupus erythematosus a case report. Bullous systemic lupus erythematosus bsle is a rare cutaneous.
In this case report, we present a case of a 22yearold african american woman with bsle and impaired renal. We describe the first case of bullous sle to develop after administration of methimazole. Bullous systemic lupus erythematosus differential diagnosis. The first is striking basal layer vacuolization with subsequent blister formation. In the present study of 74 cases, clinical diagnosis correlated with the histological diagnosis in 64. Bullous systemic lupus erythematosus bsle is a rare autoimmune blistering disorder that typically manifests as an acute vesiculobullous. First algorithm to approach skin biopsy of autoimmune vesiculobullous disease should be localization of the anatomic level of the split, which could be either intraepidermal or subepidermal. Indeed, most of these items have a very low prevalence in sle. Review article treatment of bullous systemic lupus erythematosus. Three histologic patterns have been identified in such lesions. Diagnosis is by skin biopsy and immunofluorescence testing of skin and serum. Bullous systemic lupus erythematosus clinics in dermatology. Papillary neutrophilic microabscesses immunofluorescence granular iga in dermal papillae linear iga bullous disease. Bullous systemic lupus erythematosus bsle is a blistering skin condition occurring in lupus patients.
Bullous dermatoses are a variety of autoimmune skin diseases that are characterized by the presence of bullae or blisters. Treatment of bullous systemic lupus erythematosus hindawi. Bullous pemphigoid dermatologic disorders merck manuals. Pdf skin involvement in systemic lupus erythematosus sle occurs in more than 75% of patients with this condition. Cynthia magro, department of pathology, atoid le, lupus erythematosus profundus, drug. Pdf bullous systemic lupus erythematosus researchgate. In contrast, bullous systemic lupus erythematosus bsle is a distinctive bullous eruption occurring with sle, presenting with typical clinical and. Subepidermal blister with neutrophil predominant inflammation harrisstith et al. Among all, direct immunofluorescence is the gold standard for diagnosis of bullous pemphigoid. Bsle can present as the initial manifestation of sle and may be a marker of severe disease. Bullous systemic lupus erythematosus bsle is a rare manifestation of systemic lupus erythematosus sle, characterized histologically by subepidermal bullae with predominantly neutrophilic infiltrate and immunoglobulin igg, iga, igm and c3 deposition at the basement membrane zone. Jul 01, 1985 4 histopdthology of cutaneous lupus erythematosus antoinette f.
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