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bullous systemic lupus erythematosus ( sle ) is a clinicopathologically distinctive immunobullous eruption occurring during the course of sle . circulating antibodies to type vii collagen ( nc1 domain )
subepidermal blister formation in the course of severe systemic lupus erythematosus ( sle ) can occur due to extensive interface inflammation and basal cell vacuolation , presenting as polycyclic erosions with advancing blistering border predominantly on sun - exposed areas .
in contrast , bullous systemic lupus erythematosus ( bsle ) is a distinctive bullous eruption occurring with sle , presenting with typical clinical and pathological findings including circulating antibodies to type vii collagen ( nc1 [ noncollagenous domain 1 ] domain ) .
occasionally other antigens such as laminin 5 , laminin 6 , and bp230 ( bullous pemphigoid antigen ) have also been described .
a 39-year - old lady , a homemaker and mother of three , presented with largely asymptomatic vesicobullous lesions ( 2 months duration ) , involving head and neck , upper limbs , and trunk over both photo - exposed and unexposed areas . the blisters would rupture over 2 - 3 days ; yielding clear ( sometimes hemorrhagic ) fluid ; heal over 2 - 3 weeks ; leaving behind pigmentation and occasional scarring .
concurrent painful oral ulcers were reported ; though , other mucosae were uninvolved . a 6-month history suggestive of raynaud 's phenomenon ; arthralgias involving small joints of hands and feet ;
the patient reported three previous , spontaneous abortions , ( 5months gestation ) the last one being 6 years back . on examination , there were multiple , tense , clear as well as hemorrhagic , vesicles and bullae , arising over an erythematous base and predominantly involving upper body [ figure 1 ] .
in addition , superficial erosions , areas of postinflammatory hyperpigmentation , and small superficial scars were noted .
superficial erosions were also noted over the buccal mucosa , hard palate , and lips .
presence of tense vesicles ( marked with an arrow ) filled with cleat fluid along with superficial erosions with polycyclic margins .
lesions are present on covered as well as exposed areas a provisional diagnosis of bsle was considered and she was thoroughly investigated . she had a significant anemia ( 8.8 gm / dl ) with evidence of iron deficiency on peripheral smear and iron studies .
extensive serological workup revealed antinuclear antibody positivity of 1:640 ( using hep-2 cells ) ; positive anti - dsdna , anti - ro , and anti - la antibodies ; low levels of c3 and c4 with a normal ch50 component ; negative antiphospholipid antibody , rheumatoid factor , and c - reactive protein .
x - rays of chest and involved joints were normal . a lesional biopsy revealed subepidermal split with neutrophilic infiltrate in the papillary dermis [ figure 2 ] .
direct immunofluorescence ( both perilesional and distant - covered skin ) revealed igm and c3 deposition along the basement membrane zone ( bmz ) as well as perivascular location . due to resource constraints , indirect immunofluorescence or salt - split skin
based on clinical , histopathological , and serological evidence , a diagnosis of bsle was confirmed .
biopsy taken from vesicular lesion revealed a subepidermal split , containing numerous red blood cells .
a marked neutrophilic infiltrate is seen in the papillary dermis ( h and e , 250 ) .
inset shows a positive lupus band ( igg ) at the basement membrane zone ) with a normal glucose 6-phosphate dehydrogenase ( g6pd ) activity , we planned to start her on dapsone ; however , her low hemoglobin prevented this .
she was started on prednisolone ( 1 mg / kg / day as a single morning dose ) ; hydroxychloroquine ( 200 mg twice a day ) ; along with complete sunprotection and topical care .
there was only partial improvement with new lesions developing even after 4 weeks ; wherein , she was started on dapsone .
she responded quite favorably with no new lesions and satisfactory healing of pre - existing ones [ figure 3 ] . over the next 6 months , steroids were completely withdrawn , and dapsone and hydroxychloroquine continued for further 1 year .
the patient has remained in remission without any systemic manifestations as well , over the last 1 year .
cutaneous lesions are reported during the course of sle in 76% patients ; however , vesicobullous lesions account for less than 1% of these .
it generally affects young adult females and involves both sun - exposed and nonexposed areas .
clinically , it is characterized by subepidermal blisters ; appearing on erythematous or urticated base ; with a predilection for face , upper trunk , and proximal extremities .
lesions can mimic bullous pemphigoid ( bp ) , inflammatory variant of epidermolysis bullosa acquisita ( eba ) , and linear iga disease .
our understanding of the pathology of this interesting and unique dermatologic condition has evolved with improved diagnostic criteria and clinicohistologic profiling .
gammon and briggaman suggested six diagnostic criteria : fulfillment of american rheumatism association ( ara ) criteria for sle ; acquired vesicobullous eruption on ( but not limited to ) sun - exposed skin ; histologic evidence of subepidermal blister with neutrophilic infiltrate ; presence of igg , igm , iga , and c3 at the bmz ; evidence of antibodies to type vii collagen ; and codistribution of immunoglobulin deposits with anchoring fibrils / type vii collagen by immunoelectron microscopy .
on the basis of these , bsle can be of two distinctive types : type i fulfilling all six criteria , while type ii ( undetermined antigen or dermal antigen other than type vii collagen ) satisfies criteria 1 - 4 only .
as seen in our case , bsle developing with or prior to features of sle can present a diagnostic dilemma ; hence , other subepidermal vesicobullous disorders need to be ruled out .
histopathologically , a predominant neutrophilic infiltrate may suggest dermatitis herpetiformis ( dh ) and dramatic response to dapsone is also common to both diseases .
eba is histopathologically and immunopathologically identical , as it is also mediated by antibodies to type vii collagen ; however , a dramatic response to dapsone , as in our case , helps differentiate the two as eba is notoriously resistant to treatment . the major antigenic epitopes for both bsle and eba reside within the fibronectin type iii homology region ( fn3 ) of nc1 domain of type vii collagen .
this region is important in mediating the interaction between anchoring fibrils and other matrix proteins .
blister formation occurs as a result of antibody - induced interference with normal interactions between type vii collagen and its extracellular matrix ligands ; destabilization of anchoring fibrils due to impaired antiparallel dimer formation of type vii collagen ; or complement mediated tissue damage . whether or not bsle is associated with systemic activity is a hotly debated topic .
some authors argue against this ; though , others have reported a clinical and/or serological flare with the eruption . in our patient ,
symptoms of sle developed shortly before the eruption , though no significant systemic activity was recorded ; however , a meticulous search for systemic involvement is highly suggested .
most reported cases have dramatically responded to dapsone , with cessation of new lesions in 1 - 2 days and healing within several days .
the same was seen in our case with dapsone ( 2 mg / kg / day ) .
though , it may be difficult to introduce the drug initially , as in our case ; every attempt should be made to use it , as relatively low doses ( 25 - 50 mg ) have also been shown to be efficacious . a relative resistance to steroids and immunosuppressives
our patient showed only modest improvement on steroids and antimalarials , complete response being seen only with dapsone .
the case is presented for its typical and distinctive features which should prompt an astute physician to suspect and investigate underlying systemic manifestations of sle .
also , the dramatic response to dapsone is highlighted which remains the drug of choice in the management of this disorder .
dapsone should be considered the drug of choice for bullous systemic lupus erythematosus as treatment response to other options is suboptimal .
even if one is not able to initiate dapsone due to relative contraindications , as in our case , every effort should be made to use it as early as possible .
the course of the disease is benign with remission reported in most of the cases within a year , including ours .
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 . 
 often associated is a heterogeneous profile of autoimmunity to components of type vii collagen . 
 it needs to be differentiated from other subepidermal bullous diseases in view of potentially associated systemic manifestations of sle . 
 we report a 39-year - old lady who presented with an extensive vesicobullous eruption on face , neck , trunk , and mucosa . she was also found to have photosensitivity , significant anemia , and high antibody titers confirming sle . 
 routine histopathology and immunoflorescence studies suggested bsle and she responded favorably to dapsone . the importance of clinical and histopathological findings in confirming a diagnosis of bsle along with the very encouraging response to dapsone therapy is highlighted .