Janeway lesions and splinter hemorrhages are a cutaneous sign of infective endocarditis (IE). Janeway lesions are nontender, erythematous or violaceous maculae on the palms and/or soles that are also found in several noninfective diseases, such as systemic lupus erythematosus and myxoma.1 Splinter hemorrhages on multiple nails without any obvious trauma are indicative of systemic causes: not only IE, but antiphospholipid syndrome, vasculitis, and treatment with systemic tyrosine kinase inhibitors or hemodialysis.2 However, to our knowledge, Janeway lesions or splinter hemorrhages due to noninfective endomyocarditis, such as eosinophilic endomyocarditis, have not been reported.
Herein, we present a case of both Janeway lesions and splinter hemorrhages that arose in eosinophilic endomyocarditis and faded away as the underlying disease improved.
A man in his 30s was referred to our department for asymptomatic skin lesions of the fingernails and toes that arose approximately 1 month after diagnosis with multiple cerebral infarctions and cardiac failure. His consciousness and vital signs were normal. Nontender, small, and erythematous maculae on the toes, suggestive of Janeway lesions, were observed (Figure 1A). Linear reddish-brown streaks were noted on the distal portions of the nail plates of all fingers, which were consistent with splinter hemorrhages (Figure 1B). There was no history of direct trauma to the fingertips or toes.
A, Nontender, small purpuric spots on all toes. B, Linear reddish-brown streaks on the distal portions of the nail plates of all fingers. C and D, After 3 months of treatment, all lesions on the toes and fingers have completely disappeared.
A skin biopsy specimen obtained from the macular lesion on the right fifth toe showed thrombi or thromboemboli within the blood vessels in the dermis (Figure 2) without any abscess formation, which is seen in Janeway lesions in IE.3 Blood tests on 2 occasions in the course of 2 weeks revealed eosinophilia (eosinophil counts, 15 340/μL and 7520/μL) despite the absence of other causes of secondary eosinophilia. These findings fit the definition of hypereosinophilic syndrome proposed by Simon et al4 in 2010: eosinophilia found on more than 1 occasion and exclusion of secondary eosinophilia for the diagnosis.
Histopathological findings include thrombi or thromboemboli within the blood vessels in the dermis (hematoxylin-eosin, original magnification ×100).
Electrocardiograms, which initially showed a small negative T-wave in leads II, III, and aVF that became flat in subsequent days, suggested cardiomyopathy. Findings of diagnostic imaging studies with contrast-enhanced computed tomography, cardiac magnetic resonance imaging, and transthoracic echocardiography suggested thrombi in the right ventricle, subendocardial late and poor gadolinium enhancement, and noncompaction of the left ventricle with the hypertrophied wall, all of which are consistent with eosinophilic myocarditis rather than IE.5 A diagnosis of IE was excluded because the patient had no sign of infection (ie, no fever, negative blood cultures, and no vegetation on cardiac valves).
Based on these detailed clinical evaluations, we diagnosed the patient as having Janeway lesions and splinter hemorrhages associated with eosinophilic endomyocarditis secondary to hypereosinophilic syndrome, also known as Loeffler endomyocarditis. With systemic steroids and thrombolytic therapy, the patient’s general condition, eosinophilia, and abnormal findings on imaging studies were mostly improved 3 months later. As he recovered from the underlying disease, the Janeway lesions and the splinter hemorrhages eventually completely disappeared (Figure 1C and D).
To our knowledge, this is the first reported case of Janeway lesions or splinter hemorrhages in eosinophilic endomyocarditis. Both cutaneous manifestations are results of distal cutaneous vascular insufficiency following multiple embolisms, which can occur from any source.1 We speculate that the source of the embolisms in the present case was intraventricular thrombus.
The present case has reminded us that noninfective endocarditis should be included in the differential diagnosis of Janeway lesions and splinter hemorrhages. In addition, these cutaneous signs may indicate how well the treatment for the underlying disease works.
Corresponding Author: Teruki Dainichi, MD, PhD, Department of Dermatology, Kyoto University Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo, Kyoto 606-8507, Japan (firstname.lastname@example.org).
Published Online: April 29, 2015. doi:10.1001/jamadermatol.2015.0388.
Conflict of Interest Disclosures: None reported.
Funding/Support: This study was supported in part by a Fellowship Shiseido Award from the Japanese Society for Investigative Dermatology and a Grant-in-Aid for Scientific Research (KAKENHI) from the Japan Society for the Promotion of Science.
Role of the Funder/Sponsor: The funding institutions had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.
Usui S, Dainichi T, Kitoh A, Miyachi Y, Kabashima K. Janeway Lesions and Splinter Hemorrhages in a Patient With Eosinophilic Endomyocarditis. JAMA Dermatol. 2015;151(8):907-908. doi:10.1001/jamadermatol.2015.0388