scholarly journals Detection of Type VII Collagen Autoantibodies Before the Onset of Bullous Systemic Lupus Erythematosus

2015 ◽  
Vol 151 (5) ◽  
pp. 539 ◽  
Author(s):  
Daniel A. Grabell ◽  
Loderick A. Matthews ◽  
Kim B. Yancey ◽  
Benjamin F. Chong
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Type Vii Collagen

scholarly journals Diffuse Bullous Eruptions in an Elderly Woman: Late-Onset Bullous Systemic Lupus Erythematosus

2016 ◽  
Vol 8 (3) ◽  
pp. 278-282 ◽  
Author(s):  
Prajwal Boddu ◽  
Mojtaba Nadiri ◽  
Owais Malik
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Late Onset ◽  
Clinical History ◽  
The Elderly ◽  
Direct Immunofluorescence ◽  
Systemic Lupus ◽  
Diverse Range ◽  
American College Of Rheumatology ◽  
Type Vii Collagen

Vesiculobullous eruptions in the elderly represent a diverse range of varying pathophysiologies and can present a significant clinical dilemma to the diagnostician. Diagnosis requires a careful review of clinical history, attention to detail on physical and histomorphological examination, and appropriate immunofluorescence testing. We describe the case of a 73-year-old female who presented to our hospital with a painful blistering skin rash developed over 2 days. Examination of the skin was remarkable for numerous flaccid hemorrhagic bullae on a normal-appearing nonerythematous skin involving both the upper and lower extremities. Histopathology of the biopsy lesion showed interface change at the epidermo-dermal region with subepidermal blister formation, mild dermal fibrosis, and sparse interstitial neutrophilic infiltrate. Immunohistological analysis was significant for positive IgG basement membrane zone antibodies with a dermal pattern of localization on direct immunofluorescence and positive IgG antinuclear antibodies on indirect immunofluorescence. Evidence of antibodies to type VII collagen suggested the diagnosis of epidermolysis bullosa acquisita versus bullous systemic lupus erythematosus (BSLE). A diagnosis of BSLE was made based on positive American College of Rheumatology criteria, acquired vesiculo-bullous eruptions with compatible histopathological and immunofluorescence findings. This case illustrates one of many difficulties a physician encounters while arriving at a diagnosis from a myriad of immunobullous dermatoses. Also, it is important for internists and dermatologists alike to be aware of and differentiate this uncommon and nonspecific cutaneous SLE manifestation from a myriad of disorders presenting with vesiculobullous skin eruptions in the elderly.

scholarly journals Bullous Systemic Lupus Erythematosus

2018 ◽  
Vol 19 (2) ◽  
pp. 123-125
Author(s):  
Sk Jakaria Been Sayeed ◽  
Md Mujibur Rahman ◽  
AKM Humayon Kabir ◽  
Md Moniruzzaman ◽  
Uzzal Mallik ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Bullous Pemphigoid ◽  
Epidermolysis Bullosa ◽  
Lupus Erythematosus ◽  
Dermatitis Herpetiformis ◽  
Epidermolysis Bullosa Acquisita ◽  
Systemic Lupus ◽  
Type Vii Collagen ◽  
Linear Iga Disease ◽  
Distinct Disease

Bullous systemic lupus erythematosus (BSLE) is extremely rare but distinct disease, characterized by vesicobullous skin eruptions in systemic lupus erythematosus (SLE). It can develop either before or after a diagnosis of SLE has been established. BSLE is characterized by a dermatitis herpetiformis-like histology and an autoimmunity to type VII collagen. It must be differentiated from other autoimmune vesicobullous diseases such as epidermolysis bullosa acquisita, dermatitis herpetiformis, linear IgA disease, and bullous pemphigoid. Its important to combine clinical, histological, and immunofluorescence findings to establish a diagnosis of BSLE. We report a case of BSLE to illustrate and emphasize the need for an integrative diagnostic approach.J MEDICINE JUL 2018; 19 (2) : 123-125

scholarly journals A Case of Systemic Lupus Erythematosus with Cutaneous Leukocytoclastic Vasculitis Mimicking Bullous SLE

2021 ◽  
pp. 464-469
Author(s):  
Fumi Miyagawa ◽  
Kohei Ogawa ◽  
Takashi Hashimoto ◽  
Hideo Asada
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Unusual Case ◽  
Leukocytoclastic Vasculitis ◽  
Oral Prednisolone ◽  
Systemic Lupus ◽  
Prednisolone Dose ◽  
Type Vii Collagen ◽  
Clinical Presentations ◽  
Collagen Antibodies

Rarely, patients with systemic lupus erythematosus (SLE) develop bullous eruptions, a disease called bullous SLE in a narrow sense that has autoantibodies against type VII collagen. We describe an unusual case in which a patient with SLE developed extensive bullae on her lower extremities. Histologically, the bullous lesions were suggestive of leukocytoclastic vasculitis with deposition of C3 within blood vessel walls. Immunoblot analyses and enzyme-linked immunosorbent assays were negative for anti-type VII collagen antibodies. We initially considered bullous SLE, but eventually made a diagnosis of secondary vasculitis in SLE. The oral prednisolone dose was increased, and the vesiculobullous lesions resolved. The clinical presentations of cutaneous vasculitis in SLE include palpable purpura, petechiae, papulonodular lesions, and livedo reticularis. Bullous lesions seem to be uncommon. Physicians need to be aware that extensive bullae can occur as a result of secondary vasculitis in SLE, even if the patient does not exhibit high disease activity.

scholarly journals Bullous systemic lupus erythematosus – a case report

2013 ◽  
Vol 22 (1) ◽  
pp. 90-92
Author(s):  
Chowdhury Mohammad Ali ◽  
SM Bakhtiar Kamal ◽  
Md Mosaraf Hossain Khan
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Epidermolysis Bullosa ◽  
Lupus Erythematosus ◽  
Dermatitis Herpetiformis ◽  
Systemic Lupus ◽  
Medical College ◽  
Type Vii Collagen ◽  
Linear Iga Disease ◽  
Distinct Disease

Bullous systemic lupus erythematosus (BSLE) is a rare but distinct disease, characterized by vesicobullous skin eruptions in systemic lupus erythematosus (SLE). It can arise either before or after a diagnosis of SLE has been established. BSLE is characterized by a dermatitis herpetiformis-like histology and an autoimmunity to type VII collagen. It must be differentiated from other autoimmune vesicobullous diseases such as epidermolysis bullosa acquisita, dermatitis herpetiformis, linear IgA disease, and bullous pemphigoid. A combination of clinical, histological, and immunofluorescence findings are necessary to establish a diagnosis of BSLE. We present a case of BSLE to illustrate and emphasize the need for an integrative diagnostic approach. DOI: http://dx.doi.org/10.3329/jdmc.v22i1.15691 J Dhaka Medical College, Vol. 22, No. 1, April, 2013, Page 90-92

Bullous systemic lupus erythematosus: Detection of antibodies against noncollagenous domain of type VII collagen

1998 ◽  
Vol 38 (5) ◽  
pp. 844-848 ◽  
Author(s):  
Shigeho Shirahama ◽  
Fukumi Furukawa ◽  
Hiroaki Yagi ◽  
Toshihiro Tanaka ◽  
Takashi Hashimoto ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Type Vii Collagen

scholarly journals Treatment of Bullous Systemic Lupus Erythematosus

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Lihua Duan ◽  
Liying Chen ◽  
Shan Zhong ◽  
Ying Wang ◽  
Yan Huang ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Marked Improvement ◽  
Skin Condition ◽  
Basement Membrane Zone ◽  
Systemic Lupus ◽  
Clear Fluid ◽  
Comprehensive Review ◽  
Type Vii Collagen ◽  
Collagen Antibodies

Bullous systemic lupus erythematosus (BSLE) is an autoantibody-mediated vesiculobullous disease in patients with SLE. Autoimmunity in BSLE is characterized by the presence of circulating anti-type VII collagen antibodies. BSLE patients often present with multiple, tense, clear fluid-filled vesicles and bullae overlying erythematous edematous plaques. Skin biopsy from BSLE patients shows subepidermal bullae with numerous neutrophils and only occasional eosinophils. Furthermore, immunofluorescence examination showed linear deposition of lgG, lgA, C3, and C1q along the basement membrane zone. BSLE patients with corticosteroids treatment constantly do not receive a marked improvement, while dapsone generally dramatically improved the skin condition. Recently, it has been reported that quite a few cases of BSLE were successfully treated with other immune suppressive drugs. Therefore, a comprehensive review of the treatment of BSLE would be beneficial to cure the disease.

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