kaposi 's varicelliform eruption ( kve ) , also known as eczema herpeticum , is a rare and potentially fatal viral infection , caused mainly by reactivation of herpes simplex virus ( hsv ) , predominantly type i. other viruses such as coxsackie a 16 , vaccinia , and varicella zoster have also been implicated in its pathogenesis .
it concomitantly occurs with preexisting skin conditions , mostly atopic dermatitis ( ad ) , with a higher prevalence in childhood , although it can occur at any age .
it is characterized by widespread clusters of umbilicated vesicopustules and eroded vesicles , alternating with punched - out ulcers covered by hematic crusts usually located over the head , neck , and trunk .
antiviral treatment proved to be effective and should be instituted with no delay to prevent significant morbidity and mortality .
we conducted a retrospective observational study that included 19 patients [ table 1 ] between 0 and 55 years who were diagnosed with kve between 01 march 2008 and 01 march 2014 .
fifteen were children and only four were adults , with a similar distribution in both sexes ( 9 females and 10 males ) .
clinical features of patients with kaposi 's varicelliform eruption fourteen patients had a history of ad , one had psoriasis , one pemphigus foliaceus , one szary syndrome , and two previously healthy patients developed their debut of ad immediately before kve .
fifteen cases were sporadic , whereas four were familial ( two cousins on one side and two siblings on the other ) .
the most severe forms of kve were seen in patients with ad ( three of them had widespread lesions ) .
widespread clusters of vesicles and erosions covered by hemorrhagic crusts clinical diagnosis was performed in all cases , supported by the positivity of cytodiagnosis of tzanck in all patients in whom it was performed . in five patients a skin biopsy
two patients showed oral involvement with feeding difficulties , two had significant scarring on their face [ figure 2 ] , whereas two patients had severe involvement of conjunctival and genital [ figure 3 ] mucosa .
all patients were treated with oral or parenteral acyclovir ( with a dose ranging from 800 to 1600 mg per day until complete resolution ) , whereas in 10 patients , systemic antibiotic was added .
five patients required acyclovir suppressive therapy due to relapses ( acyclovir 800 mg per day for 612 months ) .
( b ) significant scars after the acute period kaposi 's varicelliform eruption affecting conjunctival mucosa and periocular area
kve was first described by moritz kaposi in the 19 century , who assumed it was a fungal infection , whereas juliysber argued that it was caused by bacterias . in
it has also been reported in association with psoriasis , pityriasis rubra pilaris , darier s , grover s , and hailey 
hailey diseases , and contact dermatitis ( both irritant and allergic ) , among others . of note ,
one of our patients developed kve while being treated with prednisone 60 mg per day and cyclophosphamide 150 mg per day for pemphigus foliaceus [ figure 4 ] .
kaposi 's varicelliform eruption over pemphigus foliaceus . in a clockwise direction : erosions covered by hematic crusts on ( a ) right ear , ( b ) abdomen , and ( c ) face ; ( d ) clusters of vesicles on right index finger although the pathogenesis of kve is not completely elucidated , it is well recognized that the patients with diseases with underlying skin barrier damage and those with immune deficiencies are the most susceptible .
it is believed to be due to humoral and cellular immunity dysfunction . in a study that assessed the growth of hsv
i in cultured skin of patients with atopic dermatitis and psoriasis , faster replication than in normal skin was observed .
decreased production of some cytokines , such as interferon or cxcl 10/ip b 10 , or elevated levels of serum ige have also been associated with a higher risk of developing kve .
atopic patients would have an impaired immunity against the virus mediated by t cells , a defect in the specific antibody to the virus and a decrease in nk cells and il-2 receptors .
they would also show inhibition of the th1 response due to increased level of il4 , decreased levels of cathelicidins and plasmacytoid dendritic cells producing interferon - gamma , a cytokine with known antiviral effects .
the source of kve would be hsv i infection in family members or close contacts and/or endogenous recurrent infection .
it has been speculated that hsv may enter due to the overexpression of adhesion molecules in the affected skin of kve .
it is more common in children , mainly because of its relationship with ad , although cases in healthy adults have also been reported .
most cases are sporadic , although an outbreak in a dermatology ward has been recently described . in our study
we included four contemporary cases within two families : two were siblings with severe ad , both under treatment with cyclosporine and two were previously healthy cousins who made their debut of ad immediately before their kve . there has been a significant increase in the communications of kve cases since 1980 due to the drastic increase in hsv infection in the world .
this phenomenon took particular effect in japan in 2007 , with yet no known cause .
some authors relate this increase to the use of corticosteroids and topical tacrolimus in ad . lately
, kve has been related to the use of methotrexate and retinoids in patients with psoriasis , darier , and hailey  hailey .
our series included a 15 year old girl with a previous diagnosis of psoriasis with lesions located only on the buttocks .
recently , mathes et al . proposed the term eczema coxsackium to refer to a kve caused by coxsackie virus a 16 .
kve is characterized by vesicopustules , some umbilicated and other eroded , and extended in clusters .
the lesions are painful and they are often associated with fever , malaise , and regional lymphadenopathy .
the average disease duration is 16 days and most of the lesions heal without scarring within 26 weeks .
the tzanck smear allows a diagnostic approach , that is rapid and economical , though not specific .
it is and its positivity rules out smallpox and its vacccine , booming because of bioterrorism .
detection of viral dna by pcr , electron microscopy , and immunofluorescence as well as sampling of vesicular fluid for culture and viral serology and histopathological biopsy could also be performed .
differential diagnoses are chickenpox , impetigo , contact dermatitis , and smallpox vaccine could also be done .
the most common complications are both bacterial infection that can lead to sepsis and viremia , with involvement of other organs . therefore , prophylactic antibiotic therapy is recommended .
viremia could affect lungs , liver , brain , gastrointestinal tract , and adrenal glands .
the bacterial agent most commonly found in secondary infected erosions is staphylococcus aureus , although beta - hemolytic streptococci and pseudomonas may also be isolated .
disseminated cutaneous herpes simplex in childhood , often seen in association with ad may be associated with bone marrow suppression and disseminated intravascular coagulation , presumably caused by viremia .
when general health is affected , a complete laboratory study including blood count , metabolic profile , and liver function studies should be made to rule out systemic involvement . at the present time , there is no standard therapeutic regimen for kve .
patients treated with acyclovir ( 1015 mg / kg / day ) , intravenous or oral , resolved the lesions faster than patients treated with placebo . for outpatients
this treatment is very effective and should be instituted without delay to prevent significant morbidity and mortality .
some authors add vidarabine ointment to the oral acyclovir therapy . in recalcitrant cases , foscarnet is the agent of choice . after healing , suppressive antiherpes treatment is recommended .
the highlights of our case series is the low frequency of the disease and the unusual presentation of cases in the same family .
we believe it is important to recognize this entity and to establish early treatment in order to avoid complications .
the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s )
has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal .
the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed .
the authors declare that there are no conflicts of interest , that the manuscript has been read and approved by all the authors , that the requirements for authorship have been met , and that each author believes that the manuscript represents honest work .
the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s )
has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal .
the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed .
the authors declare that there are no conflicts of interest , that the manuscript has been read and approved by all the authors , that the requirements for authorship have been met , and that each author believes that the manuscript represents honest work .
 kaposi 's varicelliform eruption is a rare and potentially fatal viral infection caused mainly by reactivation of herpes simplex virus . 
 it concomitantly occurs with pre - existing skin conditions , mostly atopic dermatitis , so it is predominately found in children . 
 we present a case series that includes four adults , familial cases , and previously healthy patients . 
 we also highlight clinical features , associations and therapeutic options . 
INTRODUCTION
CASE REPORTS
DISCUSSION
Declaration of patient consent
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Conflicts of interest