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Australasian Journal of Dermatology (2015) 56, 218–220

doi: 10.1111/ajd.12301

BRIEF REPORT

Cutaneous embolism of an atrial myxoma Ana Isabel Rodríguez Bandera,1,2 Nicholas Charles Stewart,2 Pablo Uribe,2,3 Rashi Minocha2 and James Young Joon Choi2 1

Department of Dermatology, Hospital Universitario La Paz, Madrid, Spain; 2Department of Dermatology, Westmead Hospital, Sydney, New South Wales, Australia; and 3Department of Dermatology, School of Medecine, Pontificia Universidad católica de Chile, Santiago, Chile

ABSTRACT Cardiac myxoma often presents with heterogeneous symptoms and signs and represents a challenging diagnosis. The cutaneous manifestations, if present, are often transient and non-specific and the clinician must possess a high degree of suspicion to secure the diagnosis. We present the case of a 36-year-old woman with a 6-month history of intermittent, painful, violaceous, non-blanching macules on the thumb and fingertips of the left hand and right ankle. A cutaneous embolic phenomenon was suspected and an urgent echocardiogram demonstrated an atrial mass, with subsequent histopathology confirming the clinical suspicion of atrial myxoma. Early diagnosis and excision of the tumour avoided serious complications. Key words: atrial, cardiac, cutaneous, embolism, myxoma.

INTRODUCTION The diagnosis of atrial myxoma represents a significant challenge to the clinician. Although histologically benign, it can result in devastating systemic sequelae as a result of its problematic location. The dermatologist may be instrumental in the diagnosis if skin lesions represent an early

Correspondence: Mrs Ana Isabel Rodríguez Bandera, Department of Dermatology, Hospital Universitario La Paz, Paseo de la Castellana 261, 28046 Madrid, Spain. Email: [email protected] Ana Isabel Rodríguez Bandera, MD. Nicholas Charles Stewart, MBBS. Pablo Uribe, PhD. Rashi Minoccha, MBBS. James Young Joon Choi, FACD. Conflict of interest: none Submitted 10 August 2014; accepted 27 November 2014. © 2015 The Australasian College of Dermatologists

manifestation of the disease. We present a case of a young woman with isolated cutaneous changes as a result of an atrial myxoma.

CASE REPORT A 36-year-old Asian woman presented to the emergency department at our institution with a 6-month history of intermittent pain, swelling and discolouration of the left hand and right ankle. Initially, she had presented to her general practitioner and was provided with reassurance and simple analgesia. After several further episodes she was referred to a private rheumatologist. Autoimmune serology (antinuclear antibodies, antibodies to extractable nuclear antigen, anti-double stranded DNA antibody levels and complement ©4 levels) was unremarkable and she was again reassured and discharged without follow up. Over the following months, the episodes increased in frequency and intensity and were resulting in significant anxiety and frustration. On the day of presentation, the patient reported that an episode of pain and swelling in the left hand had woken her from her sleep. After review in the emergency department, she was referred to the dermatology department for a further opinion. Her past medical history was unremarkable: she was a non-smoker and she was not on any regular medication. In particular, she denied fever, asthenia, arthralgias, chest pain, dyspnoea, palpitations and neurological symptoms. She denied risk factors for cardiovascular disease. On examination, she presented with a violaceous, nonblanching, macular, reticulate discolouration on the thenar eminence and lateral border of the left thumb and similar, but subtler, changes affecting the fingertips on the ipsilateral hand (see Fig. 1). No changes were identified on the right ankle. No other cutaneous abnormality was identified on full skin examination. In particular, lentigines, blue naevi and café au lait macules were not seen. Abnormalities in radial or carotid pulses were not detected. Our differential diagnoses included Takayasu’s arteritis and smaller vessel vasculitis, but the lack of pulse abnormalities and the fact that the skin lesions were unilateral, painful, transient and recurrent orientated our suspicions to a microvascular occlusion syndrome. Clinical findings could be consistent with an occlusion by cryoglobulins but

Cutaneous embolism of an atrial myxoma

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Figure 2 Echocardiography showed an elliptical and mobile mass 40 × 32 × 20 mm in the left atrium (circle).

Figure 1 Mottled, tender, non-blanching and reticulate macules on the volar aspect of the left thumb.

episodes were not related to cold exposure. Vascular occlusion by an opportunistic organism such as Pseudomona aeruginosa or Aspergillus was not considered because the patient was not immunocompromised. Because of the lack of fever, infectious endocarditis was considered unlikely. As the patient was young, did not undergo any arterial or coronary catheterisation and was not under any anticoagulation treatment, a cholesterol embolism was deemed unlikely. Oxalate embolus was also unlikely because the patient did not report any renal insufficiency or bowel disease. However, the age of the patient, the appearance of the skin lesions and their clinical course were consistent with cutaneous embolism from an atrial myxoma. An urgent echocardiogram was arranged and demonstrated a 40 × 32 × 20 mm mass in the left atrium (see Fig. 2). The patient was referred immediately to the cardiothoracic surgical team and underwent a sternotomy and resection. Subsequent histopathology confirmed the diagnosis of an atrial myxoma (see Fig. 3). A 3-mm punch biopsy of the affected thumb was also obtained. Histopathology demonstrated occlusion of the deep dermal vessels by myxoid material and spindled cells with a mild perivascular and interstitial inflammatory infiltrate consisting of small lymphocytes and occasional eosinophils. These features were consistent with embolisation from cardiac myxoma (see Fig. 4). The patient had an uneventful post-operative recovery and was discharged on day 3. She attended follow up with

Figure 3 Pediculated mass attached to cardiac muscle composed by myxoid stroma and scattered spindle cells (HE stain ×20).

the cardiothoracic surgeons 2 months after her operation and a repeat echocardiography showed no recurrence of the atrial myxoma. She presented to the dermatology outpatient department 3 months after her discharge and denied any further symptoms. She is scheduled for a repeat echocardiogram in 1 year.

DISCUSSION Cardiac myxoma, although rare, is the most common primary tumour of the heart.1,2 It is most frequently reported in women aged 30 to 60 years,1 and approximately 75% occur in the left atrium.2 Clinically, cardiac myxoma is considered a great imitator3 because of the wide variety of signs and symptoms it can produce. Symptoms can be divided into three groups:2 those secondary to: (i) intracardiac obstruction (occurring in 67%),4 such as dyspnoea, chest pain, syncope, palpitations; © 2015 The Australasian College of Dermatologists

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AI Rodríguez Bandera et al. Diagnosis is confirmed with echocardiography and the demonstration of myxomatous material occluding the vessel lumen in the affected organ. The demonstration of a myxomatous embolus in a skin biopsy is not always possible,8,9 and it often requires the examination of multiple sections1 or multiple biopsies.11 A cutaneous embolism from an atrial myxoma has been reported on eight occasions previously,4,12 however, most of these cases also presented with neurological symptoms,1–3,5–9,11,12 which facilitated the diagnosis. In addition, cases presenting with isolated skin lesions were diagnosed only after a histopathological correlation of biopsy specimens.4 In conclusion, this case illustrates the importance of recognising potentially serious cutaneous presentations of thromboembolic disease. In particular, transient, recurrent, painful, mottled macules or papules affecting acral areas should raise clinical suspicion of a cardiac myxoma and a prompt investigation with an echocardiogram. To the best of our knowledge, this is the first report of a patient with atrial myxoma with cutaneous embolism diagnosed before a histopathological correlation.

REFERENCES 1. Figure 4 Myxomatoid embolus (arrows). (HE stain ×100).

within

the

dermal

vessels

(ii) systemic symptoms resulting from cytokines released by the tumor (occurring in 90%),4 including fever, myalgias or arthralgias; and (iii) extracardiac embolisms (occurring in 40%),1,2,4,5 mainly involving the central nervous system.1 However, any organ can be affected. The reported cases of skin lesions secondary to cutaneous embolism from cardiac myxomas have presented in ways similar to that in our patient: painful, transient and recurrent erythematous, pale or reticulate macules and papules in an acral distribution.1–9 Splinter haemorrhages,6 cyanosis1 and ulcerative lesions have been reported7 as well as features related to cytokine release, including Raynaud’s phenomenon7 and a violaceous malar rash.3 a differential diagnosis includes vasculitis and other causes of microvascular occlusion, as mentioned previously. A full skin examination should be performed, looking for the presence of lentigines, blue naevi, freckling, café-au-lait spots and cutaneous angiomyxomas to exclude Carney complex, an autosomal, dominantly inherited syndrome characterised by lentigines, cardiac and cutaneous myxomas, schwannomas and endocrine overactivity.10 In our patient these cutaneous findings were not found.

© 2015 The Australasian College of Dermatologists

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