Fever with hemorrhagic and pustular blebs in 13 year old boy

Prepared by... Virat Sirisanthana, M.D.
Department of Pediatrics, Chiang Mai University
		Patient: A 13 year old Thai boy. Address: Chiang Mai Chief complaint: Fever and multiple skin lesions 2 days prior to admission

Present illness:
>		10 days prior to admission,
			the patient was admitted at a private hospital because of high continuous fever. He had no nausea, emesis or any URI symptoms at that time. The final diagnosis was Dengue hemorrhagic fever. He was treated by 4000cc intravenous fluid and was discharged after four days. The platelet count had normalized before the discharge.
>		5 days prior to admission,
			he has low grade fever every afternoon. The parents gave him antipyretics for the symptoms.
>		3 days prior to admission,
			the patient complained of pain in his left index finger and right second toe. He was still able to walk and perform his daily activities. His parents took him to a private clinic, where he was given some analgesics and antibiotics. The fever somehow subsided in these few days.
>		2 days prior to admission,
			multiple skin lesions appeared on his body. They were vesicles, bullae and varying size of purpuric spots which occurred simultaneously.
>		1 day prior to admission,
			the lesions expanded rapidly and progressed over the patient’s face and extremities. They also became painful. The patient felt excruciating pain in multiple joints, including both elbows, both knees and the previously affected digits. He was lethargic so his parents decided to take him to the hospital.

Past history:
		He is the only child. He was born by Cesarean section at a private hospital. He was fully vaccinated and has no history of any serious medical illness. He took no medication before this illness. He has not been out of the community in the past few months. His residence is in the country where there are paddy fields and orchards. He used to catch fish in the fields 3 months ago.

Physical examinations:
		Vital signs: Temperature 39.6 oC Blood pressure 90/50 mmHg, Respiratory rate 32 breaths/min Pulse rate 130 beats/min
		General appearance: A boy, febrile, good consciousness
		HEENT: not pale, no icteric sclera, no injected pharynx, tonsils: not enlarged, normal tympanic membranes
		Lymph nodes: right cervical lymph nodes 1.5 cm in diameter
		Heart: regular rhythm, normal S1, S2, no murmur
		Lungs: equal breath sounds, bilateral crepitation, no wheezing
		Abdomen: soft, no point of tenderness, no abnormal mass. Liver, spleen: not palpable
		Extremities: marked tender at both knee and elbow joints. Gangrene at left index finger and right second toe
		Skin: generalized, multiple hemorrhagic and pustular blebs and purpura scattered over face, body, extremities, palms and soles, spared mucous membrane.

Laboratory investigations
		CBC: Hb 11.5g% Hct 35% WBC 13,500/mm3 (N93%, L7%), platelet count: 216,000/mm3
		BUN 56 mg% Cr 1.9mg% Na 126 K 4.7 Cl 94 CO2 18 mEq/L
		PT 14.6 (control 11.5min) PTT 32.7(control 32.3min) INR 1.28
		LP: pressure 10 cmH2O, WBC 10 /mm3, all mononuclear cells, 100RBC/mm3 no organisms on Gram stain, Protein 38 mg%, sugar 82mg% in blood sugar 166mg%
		LFT: TP 6.1g% Alb 2.8g% Glob 3.3g% Chol l54g% AlkP 189u/L SGOT 135U/L SGPT 104U/L TB 2.67mg% DB 2.16mg%
		CXR : as shown

Fluid&Pus from blebs: Many Gram negative bacilli, many PMN

Course in the hospital
	After admission the patient developed shock and did not responded to supportive and antibiotic (cotrimoxazole , amikacin and cetazidime) therapy. He died 22 hours after admission.
Final Diagnosis :
	Hemoculture and pus culture: Chromobacterium violaceum

Purple-black colonies on blood agar plate	Violet colonies on Mueller-Hinton agar plate
Copy from Principal and Practice of Infectious Diseases 3rd ed. Mandell.Page 1785-6

Chromobacterium vioraceum is a well-known organism in the southeastern United States where it occasionally is involved in human infection. The organism is a soil and water inhabitant, especially in tropical areas; cases have been reported as well from the northeastern United States, Southeast Asia, and South America." Some strains produce proteases that may be involved in the pathogenesis of human illness. C. violaceum now is the only important species causing human disease;

Chromobacterium typhiflavium now has been reclassified as a Ve1 strain of Pseudomonas, and other species are not known to be pathogenic for humans. Most strains of this organism produce violacein, a pigment insoluble in water (as opposed to the water-soluble pyocyanin of Psetidomonas) that imparts a violetblack color to the colonies on solid media. The color may be lost on subculture or after therapy is begun. The organisms produce extracellular products that enhance the activity of beta-lactam inhibitors against gram-negative organisms.

Infected patients usually acquire the organism as a result of trauma leading to contamination with soil or water or by ingesting contaminated water. Resulting infection involves urinary tract, gut, or blood stream invasion, with or without an obvious primary focus. An underlying defect of host defenses, especially of the neutrophils, seems to predispose to infection, but a number of cases are described in patients with no known host factor dysfunction. Patients with chronic granulomatous disease appear susceptible, regardless of geographic location. A deficiency of polymorphonuclear leukocyte glucose-6-phosphate dehydrogenase and neutrophil dysfunction also were present in a 3-year-old patient who died with Chromobacterium violaceum sepsis. Fever is often high, and spread through the blood stream often leads to secondary sites of infection such as osteomyelitis and abscess in the lung, liver, or abdomen. Reported skin findings include pustular dermatitis, cellulitis, or ulceration.

Diagnosis is made by culture of blood, abscess fluid, or skin exudate; serologic tests are not available. Gram stain appearance of the organisms is that of gram-negative long bacilli; occasionally, the organisms will have a slight curve, which makes confusion with vibrios possible. The organisms are facultatively anaerobic and grow readily in 18-24 hours on media containing tryptophan, which include common laboratory media such as sheep blood agar, chocolate agar, Mueller-Hinton agar, trypticase soy broth, and MacConkey agar. Incubation at 30-35'C usually is effective, although growth is enhanced if incubation occurs at 25'C. The organism produces colonies with a violet pigment (hence the species name). The organisms produce hydrogen cyanide, so a faint cyanide smell may be present; the urease and citrate reactions are variable. The oxidase reaction is usually positive but hard to detect in pigmented strainssometimes demonstrating oxidase can be enhanced by incubating the culture anaerobically, which inhibits pigment formation. Indole reactions are weak and catalase reactions usu ally are negative.' A few strains do not produce pigment, so they may be confused with Plesiomonas or Aeroniotias unless results of indole production are assessed.

Chromobacterium violaceum strains usually are susceptible in the laboratory to chloramphenicol, tetracycline, trimethoprim-sulfamethoxazole, and gentamicin. They are variably susceptible to penicillins and the other aminoglycosides but resistant to most cephalosporins. Erythromycin appears ineffective regardless of the susceptibility testing results." Relapse has occurred more than 2 weeks after completion of therapy and apparent cure. Despite therapy, more than half of the reported patients have died, which may reflect the underlying defects in host defenses in most of the victims or late recognition of the etiology.