Pediatric Annals

A 10-year-old Boy With Skin Lesions, Fever, and Hip Pain

Melissa L Abrams, MD; Ross M Levy, MD; Sarah L Chamlin, MD

Abstract

DIAGNOSIS

Osier's nodes and Janeway lesions, with skin manifestations of bacterial endocarditis

DISCUSSION

In addition to the above observations, an echocardiogram revealed two large intracardiac vegetations, one mobile pedunculated mass in association with a conal ventrical septal defect, the other nonmobile attached to the pulmonary valve leaflet. Spiral computed tomography showed a pulmonary embolus in the right main pulmonary artery, and a consolidation with nodules in the left lobe of the right lung. Computed tomography of the head and renal ultrasound were normal. The patient required surgical removal of the intracardiac vegetations.

Bacterial endocarditis is associated with skin lesions including petechiae, subungal (splinter) hemorrhages, Osier's nodes, Janeway lesions, distal gangrene, necrotic bullous lesions, and ecthyma gangrenosum.1 Although these cutaneous findings are well-described in the literature, they occur in the minority of patients with endocarditis and they are less common in children than in adults. In a study conducted by Martin et al.,2 the presenting sign of infective endocarditis in children was fever in 99%, petechiae in 21%, Osier nodes in 5%, and splinter hemorrhages in 5%.

Petechiae can be located on both mucosal and nonmucosal surfaces. Subungal (splinter) hemorrhages are linear, red streaks beneath the nail. They are most often secondary to trauma, but location in the middle third of the nail is more suggestive of bacterial endocarditis.3

Osler's nodes were first described by William Osier in 1893 as "hive-like," erythematous, painful, nonhemorrhagic lesions with a white center, chiefly in the skin of the hands and feet.4 They can range in size from a millimeter to a centimeter or more in diameter and are classically described on the fingertips. These lesions are transient and can last hours to days.

Edward Janeway in 1899 described Janeway lesions as numerous, nontender, small hemorrhages with a slightly nodular character.5 Janeway lesions are several millimeters in diameter, often occurring on the palms and soles. These lesions can last for days to weeks. The Janeway lesion is nontender, in contrast to Osier's nodes, which are tender and painful, as Osier's nodes occur in densely innervated locations.

Since their original descriptions, Osier's nodes and Janeway lesions have been controversial topics. Authors have described these lesions in both acute and subacute endocarditis, as hemorrhagic or erythematous, and as located on the palms, soles, and digital pads.6 Their pathogenesis also has been controversial, with more recent support for the hypothesis that septic microemboli are the cause of these lesions, rather than a hypersensitivity or immune-complex mediated vasculitis.7

Histologically, both lesions show neutrophilic dermal microabscesses and vessel thrombosis without vasculitis. Bacteria, often S. aureus, have been identified in these abscesses and in the vessel lumen.8'10 These septic microemboli consisting of fibrin and bacteria originate from the endocardial vegetation and embolize to the skia Initial histopathological reports conducted in the 1960s emphasized the absence of emboli or bacteria; they described endothelial swelling and perivasculitis suggesting that these lesions were immunologie reactions to the etiologic agent of the disease resulting in a small vessel arteritis of the skin. This difference can be explained on the basis of the different ages of the respective lesions. Initially, organisms can be identified; however, as a lesion ages, it is possible that the host defenses destroy these organisms and result in the inflammatory response in the tissues.11

Cutaneous vasculitis, bacterial and viral infections, and bleeding abnormalities should be considered in the differential diagnosis of petechial and purpuric lesions. Endocarditis should always be considered in a child with fever and underlying congenital heart disease.

1. Kimyai-Asadi A, Usman A, Milani F. Cutaneous manifestations of maiantic endocarditis. InUDermatol. 2000;39(4): 290-292.

2. Hoyer A, Silberbach M. Infective endocarditis.…

A 10-year-old boy with a medical history significant for a ventricular septal defect presented with a 1-week history of fever, hip pain, and skin lesions. The lesions appeared 2 days after he started amoxicillin for presumed bacterial pharyngitis. The amoxicillin was stopped, and the patient was started on oral acyclovirby his family physician for presumed varicella.

The lesions began on his lower extremities but others appeared on his trunk, upper extremities, palms, and soles. The lesions were slightly painful and without pruritus. There was no history of recent dental or invasive procedures, travel, tick bites, ill contacts, or trauma. A review of systems was positive for anorexia, headache, and general malaise. The remainder of the review of systems was negative.

On physical examination, the patient was alert and cooperative. Scattered on his trunk and extremities were numerous petechiae and 2- to 5-mm firm purpuric necrotic papules (see image above). He had 1- to 2-mm nonblanching, nontender, purpuric papules on his palmar and plantar surfaces; his nails did not show evidence of subungal hemorrhages (see images on page 437). He had a III/VI holosystolic murmur heard best at the left sternal border. No organomegaly was appreciated. The left hip range of motion was limited due to pain. Neurologic examination was normal.

Laboratory examination revealed leukocytosis and thrombocytopenia. Coagulation profile revealed normal PT/PTT with an elevated d-dimer level. Urinalysis was negative for blood. A left hip aspirate and blood cultures grew methicillinsusceptible Staphylococcus aureus.

DIAGNOSIS

Osier's nodes and Janeway lesions, with skin manifestations of bacterial endocarditis

DISCUSSION

In addition to the above observations, an echocardiogram revealed two large intracardiac vegetations, one mobile pedunculated mass in association with a conal ventrical septal defect, the other nonmobile attached to the pulmonary valve leaflet. Spiral computed tomography showed a pulmonary embolus in the right main pulmonary artery, and a consolidation with nodules in the left lobe of the right lung. Computed tomography of the head and renal ultrasound were normal. The patient required surgical removal of the intracardiac vegetations.

Bacterial endocarditis is associated with skin lesions including petechiae, subungal (splinter) hemorrhages, Osier's nodes, Janeway lesions, distal gangrene, necrotic bullous lesions, and ecthyma gangrenosum.1 Although these cutaneous findings are well-described in the literature, they occur in the minority of patients with endocarditis and they are less common in children than in adults. In a study conducted by Martin et al.,2 the presenting sign of infective endocarditis in children was fever in 99%, petechiae in 21%, Osier nodes in 5%, and splinter hemorrhages in 5%.

Petechiae can be located on both mucosal and nonmucosal surfaces. Subungal (splinter) hemorrhages are linear, red streaks beneath the nail. They are most often secondary to trauma, but location in the middle third of the nail is more suggestive of bacterial endocarditis.3

Osler's nodes were first described by William Osier in 1893 as "hive-like," erythematous, painful, nonhemorrhagic lesions with a white center, chiefly in the skin of the hands and feet.4 They can range in size from a millimeter to a centimeter or more in diameter and are classically described on the fingertips. These lesions are transient and can last hours to days.

Edward Janeway in 1899 described Janeway lesions as numerous, nontender, small hemorrhages with a slightly nodular character.5 Janeway lesions are several millimeters in diameter, often occurring on the palms and soles. These lesions can last for days to weeks. The Janeway lesion is nontender, in contrast to Osier's nodes, which are tender and painful, as Osier's nodes occur in densely innervated locations.

Since their original descriptions, Osier's nodes and Janeway lesions have been controversial topics. Authors have described these lesions in both acute and subacute endocarditis, as hemorrhagic or erythematous, and as located on the palms, soles, and digital pads.6 Their pathogenesis also has been controversial, with more recent support for the hypothesis that septic microemboli are the cause of these lesions, rather than a hypersensitivity or immune-complex mediated vasculitis.7

Histologically, both lesions show neutrophilic dermal microabscesses and vessel thrombosis without vasculitis. Bacteria, often S. aureus, have been identified in these abscesses and in the vessel lumen.8'10 These septic microemboli consisting of fibrin and bacteria originate from the endocardial vegetation and embolize to the skia Initial histopathological reports conducted in the 1960s emphasized the absence of emboli or bacteria; they described endothelial swelling and perivasculitis suggesting that these lesions were immunologie reactions to the etiologic agent of the disease resulting in a small vessel arteritis of the skin. This difference can be explained on the basis of the different ages of the respective lesions. Initially, organisms can be identified; however, as a lesion ages, it is possible that the host defenses destroy these organisms and result in the inflammatory response in the tissues.11

Cutaneous vasculitis, bacterial and viral infections, and bleeding abnormalities should be considered in the differential diagnosis of petechial and purpuric lesions. Endocarditis should always be considered in a child with fever and underlying congenital heart disease.

REFERENCES

1. Kimyai-Asadi A, Usman A, Milani F. Cutaneous manifestations of maiantic endocarditis. InUDermatol. 2000;39(4): 290-292.

2. Hoyer A, Silberbach M. Infective endocarditis. PedialrRev. 2005;26(11):394400.

3. SahnEE,BluesteinE.Purpuricpalmarmacule in a child with fever of unknown origin. Arch Dermatol. 1992;128(5):682-683, 685-686.

4. Osier W. Chronic infectious endocarditis. QJ Med. 1908-1909;2:219-230.

5. Janeway EG. Certain clinical observations upon heart disease. MedNews. 1899;75: 257-262.

6. Vinson R, Chung A, Elston D, Keller R. Septic microemboli in a Janeway lesion of bacterial endocarditis. J Am Acad Dermatol 1996;35(6):984-986.

7. Dalton J, Robinson J. Off-center fold: palpable purpura on the extremities. Diagnosis: leukocytoclastic vasculitis mimicking Janeway lesions of bacterial endocarditis. Arch Dermatol 2001;137(7):957-962.

8. Cardullo AC, Silvers DN, Grossman ME. Janeway lesions and Osier's nodes: a review of histopathologic findings. J Am Acad Dermatol 1990;22(6Pt 1): 1088-1090.

9. Parikh SK, Lieberman A, Colbert DA, Silvers DN, Grossman ME. The identification of methicillin resistant Staphylococcus aureus in Osier's nodes and Janeway lesions of acute bacterial endocarditis. J Am Acad Dermatol 1996;35(5 Pt l):767-768.

10. Kerr A, Tan JS. Biopsies of the Janeway lesion of infective endocarditis. JCutanPathol 1979;6(2): 124-129.

11. AlpertJS.KrousHE.DalenJE.O'RourkeRA, Bloor CM. Pathogenesis of Osier's nodes. Arm Intern Med. 1976;85(4):471473.

10.3928/0090-4481-20060601-09

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