Makis et al. Journal of Medical Case Reports 2010, 4:281 http://www.jmedicalcasereports.com/content/4/1/281 and an elevated erythrocyte sedimentation rate (ESR) (57 mm/1st hour) and C-reactive protein (CRP) (100 mg/l) (Table 1). Tests for infections were negative except for the detection of a rotavirus antigen (immuno- chromatographic test). The cultures from skin lesions were negative. On day one of hospitalization a papulo- nodular rash with lesions of up to 2 cm in diameter, some of them with central ulceration, appeared on the trunk and extremities. The child remained febrile and in the following days new crops of similar skin lesions erupted, while blood neutrophilia and elevated ESR and CRP persisted. On day six a restriction of the sponta- neous movements of the left hip, indicative of underly- ing arthritis was added to his clinical picture, however, without corresponding pathological findings in X-ray and ultrasound imaging. Because of the clinical suspi- cion of a staphylococcal infection the child was initially treated with intravenous amoxycilline-clavulanic acid, which was later changed to acyclovir, vancomycin and ceftazidime, considering the possibility of disseminated herpes virus infection with secondary bacterial super- infection of the skin lesions, although the relevant cul- tures were negative. On the 11th day of hospitalization, and having shown no substantial improvement, he was transferred to our Page 2 of 6 hospital. On admission he presented with a fever of 39°C, movement restriction of the left hip and multiple, polymorphous skin lesions: deep ulcers, up to about 1 cm in diameter on the buttocks, bullous lesions on the face and extremities, purulent ulcerations on the knees and elbows as well as numerous crusted erosions on the face, extremities, the perianal area and the oropharynx (Figures 1, 2, 3). Blood leukocytosis (25,050/μl) with neutrophilia (71%) and elevated ESR (100 mm/1st hour) and CRP (30 mg/l) were the main laboratory findings (Table 1). Remarkable was the pathergy-like eruption of cutaneous lesions at sites of minimal skin trauma, like intravenous catheter placement. Initial clinical differen- tial diagnoses included Sweet’s syndrome as well as a superficial bullous variant of pyoderma gangrenosum, Behcet’s disease and bacterial superinfection of varicella lesions. Thus the antibiotic and antiviral treatment was continued and the child was re-evaluated by extensive serological tests for infectious agents and an immunol- ogy profile, Tzank smear, microbiological cultures of lesional tissue samples and skin lesion biopsy. On the second day in our department stridor suddenly appeared, an X-ray revealed an airway constriction and intravenous dexamethasone (0.6 mg/kg body weight) was immediately administered. The child responded Table 1 Laboratory findings during the course of the syndrome Laboratory 5th day of illness 15th day of illness 17th day of illness parameters (admission to another (admission to our (initiation of hospital) hospital) prednisone) 25,050 22,770 20th day of illness (3rd day on prednisone) 15,550 889,000 59 54 32 9.5 12 14 0.4 3.5 135 27 22 57th day of illness (termination of prednisone) 13,140 19 73 14.2 491,000 4 3 21 0.5 4.5 138 42 15 Leukocytes 26,500 (/μl) Neutrophils (%) 47 Lymphocytes 40 (%) Hemoglobin 11.9 (g/dl) Platelets (/μl) 559,000 ESR (mm/1st 57 hour) CRP (mg/l) 100 Blood urea 22 nitrogen (mg/ dl) Serum 0.5 creatinine (mg/ dl) Serum 4.8 potassium (meq/l) Serum sodium 137 (meq/l) AST (IU/l) 37 ALT (IU/l) 11 986,000 100 71 21 9.8 30 11 0.4 4.3 135 46 17 807,000 102 74 18 9.2 60 13 0.4 3.6 135 34 21