we report a case of iga pemphigus with iga antibodies to desmoglein 1 ( dsg1 ) and desmoglein 3 ( dsg3 ) .
we report the case of an 60-year - old man with intraepidermal neutrophilic iga pemphigus with iga antibodies to dsg1 and dsg3 .
the disease was not effectively controlled by conventional therapeutic regimens ( colchicine , dapsone ) .
systemic treatment with isotretinoin 25 mg / d and prednisone 20 mg / d achieved only a slight effect after six months .
our case confirmed the recalcitrant nature of iga pemphigus in response to distinct therapies , indicating that further research focusing on therapeutic approaches for this type of pemphigus is needed .
an otherwise healthy 60-year - old man was referred to our department with a one - year history of recurrent pruritic vesiculo - pustular lesions on both axillae and groin .
the lesions improved after topical application of steroids but reappeared and gradually spread to trunk and extremities .
examination revealed a symmetric eruption on the proximal extremities and trunk with prominent involvement of the axillae and groin .
it was composed of grouped vesiculo - pustular lesions mostly on well - circumscribed erythematous patches ( figure 1 ) .
direct immunofluorescence microscopy ( dif ) of perilesional skin detected intercellular deposits of iga throughout the epidermis ( figure 3 ) .
igg - elisa for desmogleins ( dsg ) showed no igg antibodies to either dsg1 or dsg3 .
iga - elisa for dsgs was then performed and the results indicated that the patient s serum was positive for iga anti - dsg1 and anti - dsg3 antibodies .
colchicine , 0.5 mg 3 times daily was begun , but this had no effect after one month so it was substituted by dapsone 100 mg / d , which also failed to produce any improvement and his disease remained active .
subsequently , isotretinoin 25 mg / d and prednisone 20 mg / d were initiated achieving only a slight effect after six months .
based on pathology and dif findings , iga pemphigus can be further divided into two subtypes , namely , subcorneal pustular dermatosis ( spd ) and intraepidermal neutrophilic dermatosis ( ien ) types .
clinically , as seen in our patient , both subtypes of iga pemphigus present with small blisters and pustules overlaying well - circumscribed erythemas . a herpetiform appearance has also been reported .
the whole body can be involved , with a predilection for flexures , such as axilla , groin and submammary area .
while spd - type iga pemphigus shows subcorneal pustules , the ien type is characterized by pustule formation throughout the entire epidermis . in dif ,
spd - type iga pemphigus involves cell surface iga binding only in the upper epidermis , where as ien - type iga pemphigus shows binding throughout the epidermis .
although the histological features of our case are consistent with those of the spd type of iga pemphigus , ien - type iga pemphigus was diagnosed in the present patient because of iga deposits throughout the epidermis .
in contrast , in the ien type no reactivity of auto antibodies with desmocollin 1 , 2 , and 3 has been found , whereas desmoglein 1 and 3 were suggested as putative target antigens of ien type in single case reports .
the result of immunoelectron microscopic study revealed that the antigen of ien type may not be a desmosomal component . in our patient , iga - elisa for dsgs showed reactivity with dsg1 and dsg3 , suggesting that his pemphigus most likely belongs to the ien type .
iep should be performed because iga pemphigus has been reported to be associated with monoclonal iga gammapathy .
treatments of iga pemphigus are performed based on the disease pathomechanism and on anecdotal reports .
dapsone is commonly the drug of choice due to its effect in suppressing neutrophilic infiltration .
recently , adalimumab and mycophenolate mofetil , have also been reported to be useful in treating iga pemphigus .
our case confirmed the recalcitrant nature of iga pemphigus in response to distinct therapies , indicating that further research focusing on therapeutic approaches for this type of pemphigus is needed .
 backgroundiga pemphigus is a rare autoimmune vesiculo - pustular skin disease . 
 only approximately 70 cases have been reported to date . 
 we report a case of iga pemphigus with iga antibodies to desmoglein 1 ( dsg1 ) and desmoglein 3 ( dsg3).case reportwe report the case of an 60-year - old man with intraepidermal neutrophilic iga pemphigus with iga antibodies to dsg1 and dsg3 . 
 histologic examination revealed subcorneal neutrophilic pustules with few acantholytic cells . 
 the disease was not effectively controlled by conventional therapeutic regimens ( colchicine , dapsone ) . 
 systemic treatment with isotretinoin 25 mg / d and prednisone 20 mg / d achieved only a slight effect after six months.conclusionsour case confirmed the recalcitrant nature of iga pemphigus in response to distinct therapies , indicating that further research focusing on therapeutic approaches for this type of pemphigus is needed . 
 physicians should keep iga pemphigus in mind when approaching patients with bullous eruption . 