What is the diagnosis and treatment for a patient with a chronic history of bilateral hand erythema (redness) and excessive sweating (hyperhidrosis), who experiences severe redness and profuse sweating in their hands?

Diagnosis: Erythromelalgia with Secondary Palmar Hyperhidrosis

The clinical presentation of chronic bilateral hand erythema with severe redness and profuse sweating is most consistent with erythromelalgia (EM), a rare disorder characterized by episodic burning pain, erythema, and warmth of the extremities, often accompanied by hyperhidrosis. 1

Clinical Diagnosis

The diagnosis of EM is primarily clinical, based on history and physical examination during a symptomatic episode, as there are no specific diagnostic tests for this condition. 2 Key diagnostic features include:

• Intense burning pain of the extremities (mainly feet, but hands are also frequently affected) 2
• Erythema and heat of the skin during episodes are essential for diagnosis 2
• Bilateral symmetric involvement is typical 1
• Episodes are often triggered by physical activity, exposure to warm temperatures, and characteristically relieved by cooling 1

Critical Diagnostic Workup

You must perform the following evaluations to identify secondary causes and associated conditions:

• Complete blood count with platelet count to screen for myeloproliferative disease (essential thrombocythemia, polycythemia vera), which underlies EM in approximately 5% of patients 1
• Blood glucose and HbA1c to rule out diabetes 2
• Autoimmune screening (ANA, ENA) to evaluate for lupus erythematosus 2
• Medication review to identify potential triggering drugs (calcium antagonists, ergot derivatives) 2
• Genetic testing for SCN9A mutations if early onset (childhood/adolescence) or family history is present 2

Neurophysiological Assessment

Approximately 90% of EM patients have evidence of small fiber neuropathy on thermoregulatory sweat testing, and nearly 50% show large fiber neuropathy on electromyography and nerve conduction studies. 1, 2 These tests should be considered to fully characterize the condition, though they are not required for diagnosis.

Treatment Algorithm

First-Line: Lifestyle Modifications (Essential Foundation)

Patients must learn to control symptoms without causing tissue damage, as cooling behaviors can lead to immersion foot, ulcerations, and disability. 1

• Avoid ice or water immersion entirely, as these cause tissue damage, swelling, and ulceration 1
• Limit cooling to maximum 10 minutes, 4 times daily if absolutely necessary 3
• Avoid powerful fans continuously, as they lead to windburn-equivalent damage and rebound flushing 1
• Minimize triggers: heat exposure, exercise, prolonged dependency of extremities 1, 3
• Wear moisture-wicking materials (silver-fiber cotton or bamboo socks) to manage associated hyperhidrosis 4
• Apply urea-based emollients (10% cream) to maintain skin barrier and prevent fissuring 4
• Continue normal activities and exercise despite symptoms, as avoiding precipitating factors does not worsen long-term outcomes 1

Second-Line: Pharmacologic Treatment for EM

For myeloproliferative-associated EM specifically, aspirin 325 mg daily for 1 month should be tried, as this subtype is more likely to respond. 1, 3

For primary EM or aspirin-refractory cases, no single medication has proven universally effective, but the following have evidence:

• Topical treatments should be attempted before systemic therapy 1
• Oral medications may be necessary for severe cases, though specific agents are not standardized 1

Third-Line: Treatment of Associated Palmar Hyperhidrosis

Since hyperhidrosis is a prominent feature in this patient, targeted treatment can significantly improve quality of life. 4, 5

• Topical aluminum chloride solution is initial treatment for palmar hyperhidrosis 5, 6
• Iontophoresis is safe and effective, requiring 3-4 sessions per week initially, then 1-2 maintenance sessions weekly 4, 5
• Oral anticholinergic medications (glycopyrrolate 1-2 mg once or twice daily) can treat both conditions simultaneously, though monitor for dry mouth, blurred vision, and urinary retention 4, 6
• Botulinum toxin injections (onabotulinumtoxinA) for palmar hyperhidrosis, but may cause temporary hand muscle weakness 4, 5

Fourth-Line: Advanced Interventions

For refractory cases:

• Nerve blocks before botulinum toxin injection to minimize pain during palm administration 4
• Phototherapy or systemic therapy for severe cases 1
• Dermatology consultation for recalcitrant symptoms 1

Critical Pitfalls to Avoid

• Do not dismiss this as simple hyperhidrosis or contact dermatitis without considering EM, as the combination of erythema and sweating is characteristic 1
• Do not allow patients to continue ice water immersion, despite symptom relief, as tissue damage is inevitable 1
• Do not confuse primary focal hyperhidrosis with secondary causes such as hyperthyroidism, medications, or menopause 4
• Do not overlook myeloproliferative disease screening, as this changes management and prognosis 1
• Do not use topical steroids as primary treatment, as this is not EM or standard hyperhidrosis management 1