the pemphigus diseases , which include some of the most severe bullous autoimmune skin reactions , are seen predominantly in middle - aged and elderly individuals .
all nonendemic pemphigus diseases , including paraneoplastic pemphigus , have been reported to occur in adolescents and even very rarely in children younger than 10 years .
pemphigus herpetiformis ( ph ) is considered a variant of pemphigus displaying clinical features similar to dermatitis herpetiformis and a diverse histopathologic pattern with intraepidermal and subcorneal microabscesses , eosinophilic spongiosis , or superficial bullae with usually scant acantholytic cells [ 1 , 2 ] .
the diagnosis is based on detection of ig g antikeratinocyte cell surface antibodies , both bound in vivo and in circulation . in the literature , few cases of pemphigus are reported in children .
this rarity in childhood may be only apparent as a result of its difficult diagnosis .
an 12-year - old boy was seen at our department in march , 2007 , with erosive plaques , vesicles , bulls , and crusted lesions associated with severe itching persisting for six months .
the boy 's family history and personal history were unremarkable , and his growth and development had proceeded normally . on examination , we observed vesicular , bullous lesions , some having clear contents and some being purulent with erosive arciform plaques and crusted lesions ( figures 1(a ) and 1(b ) ) .
they were seen especially over the back , buttocks , chest , abdomen legs , and arms .
histologic examination of one of the lesions showed an intraepidermal bulla containing rare acantholytic epidermal cells , eosinophil and neurophil cells .
the superticia1 and reticular dermis had an inflammatory infiltrate of eosinophile and neutrophile around some of the blood vessels ( figures 2(a ) and 2(b ) ) .
iga intercellular deposits on epidermis were not seen ( figures 3(a ) and 3(b ) ) .
a diagnosis of ph was made , and treatment with dapsone 2  mg / kg per day resulted in total clinical remission .
however , two months later , new vesicles reappeared and treatment was begun with prednisone at a dose of 2  mg / kg daily .
there was a very good response ; lesions regressed slowly , until they completely disappeared after 4 weeks of treatment .
the daily dose of the prednisone was lowered gradually according to the clinical improvement , and , at the time of this report , one year after onset of the disease the skin involvement was being kept under control on a low maintenance dose of 10  mg of prednisone daily .
autoimmune blistering diseases are extremely rare in children . a review from a paediatric dermatology referral center for a population of 4 million people identified only 23 cases of immunobullous disease in patients less than 18 years of age over a 16-year period .
a recent review of pemphigus vulgaris ( pv ) in children found only 46 cases reported in the literature .
pemphigus herpetiformis ( ph ) was first introduced by jablonska and colleagues   as a variant of pemphigus .
various terms have been used to describe this condition , such as acantholytic herpetiform dermatitis , pemphigus controlled by sulfapyridine , and mixed bullous disease .
clinical manifestations consist of erythematous , urticarial plaques , and vesicles that present in herpetiform arrangement .
histological findings of hp are variable and include eosinophilic spongiosis and subcorneal pustules with minimal or no apparent acantholysis [ 9 , 10 ] .
immunofluorescence findings show that most patients with ph have igg antibodies against keratinocyte cell surfaces .
dapsone is the first choice of drug in the ph treatment given as monotherapy or combined with systemic corticosteroids . in most
reported cases , the use of systemic corticosteroids was necessary to achieve clinical remission . in some cases , however , the use of immunosuppressive drugs such as azathioprine and cyclophosphamide becomes necessary to induce clinical remission or to help in reducing steroid dose so that its collateral effects are minimized .
based on its clinical features , some researchers reported the disease as a distinct entity and consider it to be different from classic pemphigus because of its clinical peculiarity and benign course .
however , others described it as a variant of pemphigus foliaceus ( pf ) or pv .
we believe the boy presents a pemphigus herpetiformis variant because of the clinical features of dermatitis herpetiformis ( vesicles , bulls , and itching ) and the immunologic ( igg and c3 deposit on intercellular epidermal ) and histological features ( intraepidermal bulls and acantholysis ) of pemphigus with an eosinophilic spongiosis .   various types of pemphigus including vulgaris , foliaceus , erythematosus , vegetans , and paraneoplastic pemphigus can be seen in childhood .
the disease must not be forgotten as a possible cause of erosive mucocutaneous disease in children .
it is important that antibody titers , management plans , and outcomes for this disease be reported so that greater knowledge of the effects of various treatment regimens can be obtained .
 pemphigus herpetiformis ( ph ) is one of the less common forms of pemphigus . 
 ph in children is unreported . 
 we describe a case of a child who developed ph . 
 observation . 
 a 12-year - old boy was seen at our department with erosive plaques , vesicles , and crusted cutaneous lesions associated with severe itching persisting for six months . 
 histologic examination showed an intraepidermal bulla containing rare acantholytic epidermal cells with eosinophilic spongiosis . 
 direct immunofluorescence demonstrated intercellular ig g and c3 deposit . 
 the serum titer of antibodies against intercellular epidermal was 1/200  
 ui / l . 
 diagnosis of ph was made , and treatment with dapsone 2  mg / kg per day resulted in total clinical remission . 
 however , two months later , new vesicles reappeared and treatment was begun with prednisone at a dose of 2  mg / kg daily . 
 there was a very good response . 
 
 discussion . 
 childhood pemphigus herpetiformis is a rare disease , often initially misdiagnosed . 
 it must not be forgotten that the disease is a possible cause of erosive mucocutaneous disease in children . 
1. Introduction
2. Observation
3. Discussion