acute generalised exanthematous pustulosis ( agep ) is a significant adverse cutaneous reaction , most often provoked by drugs and acute infections [ 1 , 2 ] . the clinical hallmark is the presence of multiple disseminated sterile non - follicular pustules on an erythematous background , predominantly in the folds and/or the face . the disease is characterised by an acute onset and typically a spontaneous resolution within 2 weeks . a 43-year - old man presented to a rural hospital in australia for lethargy , fever , arthralgia , chills , peri - orbital oedema and an erythematous pustular rash increasing in size over his hands , back , chest and face for approximately 48 h. he was a fly - in fly - out worker in the catchment area of the hospital . he mentioned holidaying in bali , indonesia , for 9 days to visit a female partner with whom he had unprotected sexual intercourse . he reported to have contracted genital herpes from this partner on his first visit and had sought treatment with antivirals ( acyclovir ) upon his return to australia and had remained on them ever since . he also mentioned that both he and his partner had been monogamous since the day they met each other . furthermore , he described some recent coryzal symptoms towards the end of his holiday , for which he bought some local cold and flu medication , the name and ingredients of which being unknown but being somewhat like sudafed. he also took some nurofen tablets ( ibuprofen ) in bali and upon returning to australia . apart from cystic acne 25 years ago and intermittently taking ibuprofen , the patient reported no significant past medical history . at this rural hospital , he continued to decline systemically ( fever > 39.5c , heart rate > 125 bpm ) over 36 h and was flown to a tertiary referral hospital in brisbane . on arrival , he had widespread pustules on his face , neck , back , chest , upper and lower limbs ( fig . 1 , fig he described only a mild itch and denied any genitourinary symptoms . some inguinal and cervical lymphadenopathy and minimal oral involvement were observed . he had a raised white blood cell count ( 13.2 ) , neutrophilia ( 12.17 ) , a mild lymphocytosis ( 0.56 ) , a raised c - reactive protein level ( 273 ) , and was started immediately on intravenous piperacillin / tazobactam three times daily , intravenous vancomycin twice daily and intravenous acyclovir three times daily with a suspected but yet unknown infection . he had negative serologies over the initial week for the following infections : streptococcus , syphilis , ross river virus , barmah forest virus , alphavirus , rickettsia rickettsii , measles , mumps , leptospirosis , parvovirus b19 , enterovirus , varicella and hiv . a biopsy of the hand showed evidence of spongiotic dermatitis with eosinophilic dermal infiltrates and occasional intra - epidermal vesicles harbouring langerhans cells . these results were considered inconsistent with the clinical impression of agep held at that time . further biopsies were then taken from his chest and left thigh , which showed spongiform subcorneal pustules accompanied by dermal inflammatory infiltrates and perivascular infiltrates with occasional eosinophils reported as acute neutrophilic folliculitis. acanthosis and papillomatosis were not observed . a biopsy of the right foot favoured a spongiotic hypersensitivity reaction with a vasculitic component . clinically , his symptoms appeared to indicate agep at this stage , and given that serologies up until this point were negative , he was started on oral prednisone 0.5 mg / kg and topical betamethasone dipropionate 0.05% ointment twice daily , with a very good effect over the proceeding 5 days . it was only on day 6 of this treatment that , with the help of the infectious diseases team , a positive scrub typhus ( orientia tsutsugamushi ) igtotal serology was confirmed , with a titre of 1:128 . this was not considered a primary infection , and paired tests 1 and 4 weeks later showed that the titre was stable and that this was likely an old rickettsial infection or a cross - reaction . this was also paired with scrub typhus and spotted fever pcr testing , which was also negative . prednisone was weaned down to half the dose over the following 5 days , and he was discharged home on day 13 of admission ( significantly improved on emollients and weaning dose of prednisone ) . an outpatient review 12 days following discharge revealed a slight flare - up again at home after taking ibuprofen tablets for 48 h for aches and pain . however , this resolved promptly with the cessation of the non - steroidal anti - inflammatory drug . clinically , this patient had all the hallmark symptoms of agep as described in the literature [ 1 , 2 , 3 ] but was confused initially with having an acute infectious disease given his preceding history . a wide variety of other cutaneous diseases or reactions can cause pustular eruptions , and most of them can be easily differentiated from agep . the primary clinical differential diagnosis of agep remains pustular psoriasis ( von zumbusch type ) , and often presents with a positive history of psoriasis , more generalised rash distribution , longer durations of pustules and fever , lack of a history of drug reactions or recent drug administration , more arthritis , and papillomatosis plus acanthosis on histology . sidoroff et al .   developed a sophisticated agep validation scoring system based on the euroscar study group . we noted that our patient scored 10 out of a possible 12 on this scale , which they have denoted as  definitely agep. his cutaneous symptoms included an acute - onset rash on the face and flexures , and oedematous erythema , which then developed into a widespread rash consisting of hundreds of small ( < 5 mm ) non - follicular sterile pustules . this was accompanied by the classic symptoms of fever > 38c , leucocytosis mostly due to neutrophilia > 7  10/l and lymphadenopathy . whilst his recent sexual history remains a red flag , we acknowledge that 90% of cases are attributable to drugs , although the causative agent in this case is not confirmed . the patient 's cold and flu medication in bali remains unknown but likely contained a sympathomimetic agent such as pseudoephedrine and anti - pyretic / analgesic agent such as paracetamol , both of which have been known to cause agep . however , we assume that this presentation could more likely be caused by the use of ibuprofen premorbidly , and his flare - up after his discharge , when he consumed more ibuprofen , somewhat confirms this . the patient 's initial histopathology was not conclusive and did not fit with the clinical impression of the treating team , which may relate to either the disease process evolving , sampling error , or the lack of specific guidance to look for agep features by the pathologist . however , the second set of biopsies did show the typical features of agep including : spongiform subcorneal and/or intra - epidermal pustules , an often marked oedema of the papillary dermis , and perivascular infiltrates with neutrophils and exocytosis of some eosinophils . vasculitis and/or some single - cell necroses of keratinocytes may be present   ( acute vasculitis demonstrated in foot biopsy ) . we presented this case to demonstrate that the combination of a diffuse pustular rash in combination with high fever and leucocytosis is often misinterpreted as an acute infectious disease . it is important to diagnose agep early to avoid unnecessary and/or expensive investigations and treatments . it also demonstrates that agep is largely a clinical diagnosis and clinicians may wish to use the agep validation score   to assist in their diagnosis .
we present the case of a 43-year - old man with a clinical diagnosis of acute generalised exanthematous pustulosis in the setting of a recent 9-day holiday to bali , indonesia , to visit a female partner whom he had visited five times this year for unprotected sexual intercourse and from whom he had contracted a sexually transmitted infection . 
 he also reports having taken some indonesian cold and flu medications and ibuprofen .