Given the concentric morphology of the eruption, a diagnosis of an egr variant of bullous lupus erythematosus was made. Histomorphologic and direct immunofluorescence findings of. 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. Review article treatment of bullous systemic lupus erythematosus. The incidence of bullous sle was estimated to be 0. Apr 20, 2017 given the evolution of her skin lesions despite steroids, the differential diagnosis included infectious vs. 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 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.
The histology may be identical with discrimination only possible where linear igg and igm is found at the basement membrane. Oral lesions are believed to occur in 30% of patients with bullous le 6. First description of bullous lupus associated with. Indeed, most of these items have a very low prevalence in sle.
Bullous systemic lupus erythematosus bsle is a rare autoimmune blistering disease in patients with a known diagnosis of systemic lupus erythematosus sle. Bullous systemic lupus erythematosus bsle is an autoantibodymediated vesiculobullous disease in patients with sle. Bullous systemic lupus erythematosus bsle is an uncommon. Jul 08, 2010 bullous lesions in sle due to photosensitivity, acute lupus or drugs can be differentiated by both histopathology and immunopathology. 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.
Full text vesiculobullous diseases in relation to lupus. An erythema gyratum repens variant of bullous lupus erythematosus. Pdf bullous systemic lupus erythematosus bsle is an autoantibodymediated vesiculobullous disease in patients with sle. Blistering eruptions are rare cutaneous manifestations of lupus erythematosus le that may be caused by different mechanisms. Autoimmune subepidermal bullous diseases are a heterogeneous group of disorders. Multiple blisters along the lip vermilion are a clue to. Second, inflammatory cell component should be evaluated, which could vary due to age of the lesion. May 25, 2017 autoimmune bullous diseases mainly present with vesiculobullous reaction pattern. The patient suddenly presented with a widespread blistering. Among all, direct immunofluorescence is the gold standard for diagnosis of bullous pemphigoid. Generally, patients with bsle meet the criteria for sle as defined by the american college of rheumatology.
Bullous lupus erythematosus as a cutaneous manifestation. Abstract bullous systemic lupus erythematosus bsle is a rare cutaneous complication of systemic lupus erythematosus sle. Bullous pemphigoid is a chronic autoimmune skin disorder resulting in generalized, pruritic, bullous lesions in older patients. 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. Bullous systemic lupus erythematosus successfully treated. Interestingly, histology also revealed the presence of numerous corpuscles of leishmania in the cytoplasm of histiocytes fig. Uses for immunofluorescence tests of skin and sera. Although most general approaches to the treatment and diagnosis of these entitie. 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.
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. Recent advances in our understanding of the pathophysiological basis of le bring hope for novel therapeutic approaches. A bullous lupus associated with cutaneous leishmaniasis. Bullous systemic lupus erythematosus sle is an uncommon manifestation of sle and is caused by autoantibodies against type vii collagen. Clinical presentation combined with histopathology, immunological testing. Lupus erythematosus le is an autoimmune disease with a wide range of clinical manifestations, from limited skin disease to multiorgan systemic involvement. 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 first is striking basal layer vacuolization with subsequent blister formation. Patients with bsle should be worked up for systemic involvement. Pdf bullous systemic lupus erythematosus researchgate. First description of bullous lupus associated with cutaneous. Bullous systemic lupus erythematosus sciencedirect.
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. Most of these diseases are associated with substantial morbidity, and a few may result in death. 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 systemic lupus erythematosus bsle is a rare subtype of lupus erythematosus sle that is quite uncommon during infancy, childhood and adolescence.
Bullous systemic lupus erythematosus sle is a transient autoimmune bullous disease that occurs in. It can be the presenting symptom of sle in patients who do not carry a diagnosis. Methimazoleinduced bullous systemic lupus erythematosus. 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. Sep, 2011 all these presentations represent specific lupus disease occurring on the vermilion. The cutaneous pathology of lupus erythematosus wiley online. Treatment of bullous systemic lupus erythematosus hindawi. Pdf histopathological study of vesiculobullous lesions. An erythema gyratum repens variant of bullous lupus. We report a case of bullous systemic lupus erythematosus sle. Pdf bullous systemic lupus erythematosus bsle is a rare manifestation of systemic lupus erythematosus sle, characterized. Multiple blistering eruption on face, trunk and oral mucosa for 1. Bullous systemic lupus erythematosus bsle is a rare complication of. 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.
The second is vasculitis with subepidermal blister and pustule formation. In the present study of 74 cases, clinical diagnosis correlated with the histological diagnosis in 64. Bullous systemic lupus erythematosus is a rare distinctive subepidermal bullous disease seen in patients with systemic lupus erythematosus sle. Papillary neutrophilic microabscesses immunofluorescence granular iga in dermal papillae linear iga bullous disease. Go back to the top to order, visit the purchasing page for details. Immunoreactive on dermal side of salt split skin positive lupus serology neutrophil rich infiltrate dermatitis herpetiformis. Frequently the dermal papillary neutrophilic infiltrate is seen to extend into the surrounding interstitium. The parasitological culture of the skin biopsy was positive, and mass spectrometry identi.
In addition, blisters can occur due to the association of sle with other autoimmune blistering diseases e. Nov 21, 2007 bullous systemic lupus erythematosus sle is a rare, distinctive subepidermal blistering disorder that occurs in systemic lupus erythematosus. Epidermolysis bullosa acquisita eba is histopathologically and immunopathologically identical since both are mediated by circulating antibodies against type vii collagen. Cutaneous histopathology of lupus erythematosus diagnostic. Bsle patients often present with multiple, tense, clear fluidfilled vesicles and bullae overlying erythematous edematous plaques. Pdf treatment of bullous systemic lupus erythematosus. 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. Bullous systemic lupus erythematosus differential diagnosis. Bullous systemic lupus erythematosus bioline international.
Pdf bullous systemic lupus erythematosus bsle is a rare manifestation of systemic lupus erythematosus sle, characterized histologically by. Bullous systemic lupus erythematosus clinics in dermatology. Similarities in histology and immunopathology exist between bsle and other primary. Bullous pemphigoid dermatologic disorders merck manuals. While some valued the completeness of skin lupus symptoms, others found the list much too long. Pemphigoid is a group of rare autoimmune blistering diseases of the skin, and mucous membranes. Bullous lesions as a manifestation of systemic lupus. The clinical presentations range from a skin rash unaccompanied by extracutaneous. Histopathology of cutaneous lupus erythematosus sciencedirect. We describe the first case of bullous sle to develop after administration of methimazole. Vesicles form not only on the le erythema but also on the normal looking skin.
Article english pdf article in xml format how to cite this article scielo analytics curriculum scienti automatic translation. 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. Bullous lupus erythematosus bsle is a rare subset of systemic lupus erythematosus sle, often associated with autoimmunity to type vii collagen. Painful erythematous or edematous nodules on acral skin following exposure to cold clinical context variable. 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 systemic lupus erythematosus grover c, khurana a. Gane fromthe departmentofpathology, godfrey hugginsschoolofmedicine, university ofrhodesia, salisbury, rhodesia synopsis asimple sequential chart for identifying bullous skin lesions is presented based on the site andsize ofthe bulla andhelpful changes in the bulla, epidermis, anddermis. It is a heterogeneous disease that is caused by autoantibodies to the dermoepidermal junction, mainly type vii collagen.
In a large cohort of sera taken from patients with immunobullous disorders, 12% were identified as bullous sle 1,2. Topical and systemic corticosteroids are used initially. 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. Bullous lesions as a manifestation of systemic lupus erythematosus.
Review article treatment of bullous systemic lupus. Bullous systemic lupus erythematosus bsle is a blistering skin condition occurring in lupus patients. Pdf bullous systemic lupus erythematosus a case report. Three histologic patterns have been identified in such lesions. 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. 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. 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. Bullous systemic lupus erythematosus a case report ncbi nih.
Bullous systemic lupus erythematosus and cicatricial pemphigoid. Cynthia magro, department of pathology, atoid le, lupus erythematosus profundus, drug. Bullous lesions can occur in systemic lupus erythematosus sle as a subepidermal blistering disease 1 or when severe edema and hydropic. Pdf skin involvement in systemic lupus erythematosus sle occurs in more than 75% of patients with this condition. It has characteristical clinic, pathologic, and immunologic findings including antibodies to type vii collagen, laminin 332, laminin 331, and bullous pemphigoid antigen 230. Bullous sle is a heterogeneous disease without pathognomonic clinical features, making the diagnosis and differentiation from other.
Because of the association of egr with malignancy, abbreviations used. 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. Pdf histopathological study of vesiculobullous lesions of. Subepidermal blister with neutrophil predominant inflammation harrisstith et al. Autoimmunity in bsle is characterized by the presence of circulating antitype vii collagen antibodies. Dapsone was initiated while awaiting the result of skin biopsies. Bullous dermatoses are a variety of autoimmune skin diseases that are characterized by the presence of bullae or blisters. 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. Routine histopathology and immunoflorescence studies. Jul 01, 1985 4 histopdthology of cutaneous lupus erythematosus antoinette f. Bullous systemic lupus erythematosus sle is a rare blistering disease with a distinctive combination of clinical, histologic and immunopathologic features that. 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.
It is characterized clinically by a pemphigoidlike eruption with tense fluidfilled vesicles and bullae, often with a background of maculopapular or urticated erythema. Diagnosis of bullous pemphigoid includes clinical assessment, skin biopsy for histopathology and direct immunofluorescence, indirect immunofluorescence and elisa test. In cases of pemphigus and pemphigoid, findings of typical antibodies in sera and. Bullous systemic lupus erythematosus bsle is a rare cutaneous autoimmune disorder characterized by rapid, widespread vesiculobullous lesions in patients with systemic lupus erythematosus sle. We present a case of a 17yearold adolescent girl who presented with.
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