Erythromelalgia: a cutaneous manifestation of polycythemia vera
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A 77-year-old woman presented in March 2025 for diffuse widespread pruritus and palmar erythema with burning pain that onset approximately 4 months prior. She was initially treated with Dupixent, 300 mg subcutaneously every 2 weeks, and antihistamines with a presumed diagnosis of atopic dermatitis. The patient noted her pruritus worsened after a shower and did not fully resolve with prescribed therapies. Despite the widespread pruritus, the patient did not have any other primary lesions suggestive of a papulosquamous or eczematous disorder; the palmar erythema was her only cutaneous manifestation. A review of systems was otherwise negative for other concerning symptoms. Given her refractory symptomatology, additional labs were ordered and demonstrated elevations across myeloid blood cell lines: leukocytes, 13.6 K/uL (reference range, 4.5–10.0); erythrocytes, 6.16 10*6/uL (reference range, 3.5–5.5); hemoglobin, 17.8 g/dL (reference range, 11.0–16.0); hematocrit, 56.6 % (reference range, 32.0–48.0); platelets, 465 K/uL (reference range, 150–400); neutrophils, 9.81 K/uL (reference range, 1.80–8.5); eosinophils, 0.61 K/uL (reference range, 0.00–0.50); and basophils, 0.24 K/uL (reference range, 0.00–0.20). A physical examination revealed diffuse palmar erythema (Figure 1) without any cutaneous evidence of atopic dermatitis clinically consistent with erythromelalgia. The patient was diagnosed with polycythemia vera and referred to hematology for continued management. Routine phlebotomies resolved her pruritus and erythromelalgia of her hands after two sessions (Figure 2).
Diffuse and confluent erythema of the bilateral ventral hands.
Marked improvement of bilateral palmar erythema after two therapeutic phlebotomies.
Polycythemia vera is a chronic myeloproliferative neoplasm indicated by the clonal overproduction of erythrocytes, leukocytes, and platelets [1], 2]. This cellular overproduction increases the risk of vascular complications, including erythromelalgia. Erythromelalgia is a distinct yet underrecognized cutaneous manifestation presenting with erythema, episodic burning pain, and warmth of the extremities. These symptoms are typically triggered by heat, exertion, or limb dependency, and are alleviated through cooling [3], 4]. However, prolonged cooling can result in immersion foot and exacerbate the condition [5]. Erythromelalgia affects an estimated 2–15 per 100,000 individuals, with a greater female predominance usually during the onset of middle age [3], 6]. In polycythemia vera, erythromelalgia results from various microvascular thrombotic events driven by platelet activation, thus leading to localized ischemia and neuropathic pain [1], 7]. Given the patient’s treatment refractory pruritus and palmar erythema, in addition to the author’s high clinical suspicion, a complete blood count with differential was initially ordered. This is reasonable given the characteristic association of erythromelalgia and myeloproliferative disorders [3]. Differential diagnoses of erythromelalgia include Raynaud’s phenomenon, small fiber neuropathy, and complex regional pain syndrome [3]. Additionally, palmar erythema can be a manifestation of other systemic etiologies such as pregnancy, liver cirrhosis, systemic lupus erythematosus, rheumatoid arthritis, hyperthyroidism, Kawasaki disease, and drug-induced toxic erythema of chemotherapy. Therefore, it behooves clinicians to perform a thorough history and physical examination, consider other ancillary diagnostics like serum studies, skin biopsies, or imaging, and clinically correlate them to provide an accurate and sound diagnosis. First-line therapy is low-dose of aspirin, which mitigates the pain by inhibiting platelet aggregation [1], 8]. It is not uncommon for symptoms to precede a hematologic diagnosis by months or even years for some patients. This underscores the importance of early recognition and a thorough blood count evaluation to prevent serious vascular complications [7].
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Research ethics: Not applicable.
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Informed consent: The patient provided written consent for publication of her photographs.
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Author contributions: The authors have accepted responsibility for the entire content of this manuscript and approved its submission.
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Use of Large Language Models, AI and Machine Learning Tools: None declared.
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Conflict of interest: None declared.
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Research funding: None declared.
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Data availability: Not applicable.
References
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© 2025 the author(s), published by De Gruyter, Berlin/Boston
This work is licensed under the Creative Commons Attribution 4.0 International License.
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Articles in the same Issue
- Frontmatter
- Behavioral Health
- Original Article
- The effectiveness of training student physicians in culturally sensitive patient care using an interprofessional culturally competent curriculum
- General
- Original Article
- Equity reporting in systematic reviews and meta-analysis for geographic atrophy: a PROGRESS-Plus assessment
- Innovations
- Commentary
- Artificial intelligence and osteopathic medicine: preserving human-centered care in an era of technological advancement
- Medical Education
- Original Article
- The impact of a summer research internship program on research engagement of osteopathic medical students
- Neuromusculoskeletal Medicine (OMT)
- Clinical Practice
- The mechanism of muscle energy for a superiorly subluxed rib one
- Clinical Image
- Erythromelalgia: a cutaneous manifestation of polycythemia vera
- Letter to the Editor
- Expanding the role of OMT in chronic rhinosinusitis: a welcome step forward
