What are the next steps for an 18-year-old female (18yo F) with a 1-month history of soreness and achiness, without skin irritation, that worsens with heat, friction, and walking?

Evaluation and Management of Genital Irritation in an 18-Year-Old Female

This presentation is most consistent with erythromelalgia (EM) affecting the genital region, and you should initiate first-line therapy with topical treatments and aspirin 325 mg daily while evaluating for underlying causes. 1

Clinical Recognition

The key diagnostic features that point toward erythromelalgia include:

• Episodic soreness and achiness without visible skin changes suggests vasomotor dysfunction rather than infectious or inflammatory dermatologic conditions 1
• Flares triggered by heat, friction, and walking are pathognomonic for EM, as these activities increase local temperature and blood flow 1
• One-month duration indicates this has progressed beyond acute irritation and requires systematic evaluation 1

The absence of skin irritation is particularly important—it distinguishes EM from infectious vulvovaginitis, contact dermatitis, or epidermolysis bullosa, which would show visible lesions, blistering, or erythema 1.

Initial Evaluation

Before initiating treatment, you must evaluate for underlying causes:

• Complete blood count with differential to screen for myeloproliferative disorders (essential thrombocytosis, polycythemia vera), as EM secondary to these conditions responds dramatically to aspirin within days 1
• Comprehensive metabolic panel to assess for renal or hepatic disease 2
• Thyroid function tests to rule out thyroid dysfunction 2
• Review all medications for drug-induced causes, as 20-30% of cases have underlying systemic triggers 2
• Assess for small fiber neuropathy through clinical examination, as approximately 90% of EM patients have associated neuropathy 1

First-Line Treatment Algorithm

Lifestyle Modifications (Essential for All Patients)

• Counsel the patient to maintain normal activities despite symptoms, as avoiding triggers leads to progressive disability 1
• Limit cooling measures to safe practices: ice or water immersion should be restricted to 10 minutes maximum, 4 times daily, to prevent immersion injury and ulceration 1
• Recommend elevation of affected areas during symptom flares 1
• Advise loose-fitting, breathable cotton underwear to minimize friction and heat retention 1

Topical Therapy (Trial Each for 4 Weeks)

Start with compounded midodrine 0.2% in Vanicream applied 3 times daily to the affected genital area, as this α1-agonist causes vasoconstriction that reduces redness and pain in 12/12 patients in case series 1

Alternative topical options if midodrine is unavailable:

• Compounded amitriptyline-ketamine cream applied 2-3 times daily 1
• Topical capsaicin 0.025% patches applied for 30 minutes, repeated every 3 months (not more frequently) 1
• Over-the-counter diclofenac 1% gel applied twice daily 1

Warn about rebound erythema with prolonged use of vasoactive topicals like oxymetazoline or brimonidine 1

Systemic Therapy

Aspirin 325 mg orally daily for at least 1 month should be initiated immediately if no contraindications exist (bleeding disorders, peptic ulcer disease, aspirin allergy) 1. This is especially effective if underlying myeloproliferative disease is present, with dramatic improvement occurring within days 1.

Second-Line Therapy (If No Response After 4 Weeks)

If symptoms persist despite topical therapy and aspirin:

• Add systemic medications such as gabapentin, pregabalin, or duloxetine, each trialed for 3-4 months 1
• Consider clonidine transdermal patches (0.1-0.3 mg daily, changed weekly) as they decrease central sympathetic outflow and can be applied anywhere on the body 1

Third-Line Therapy

Refer to a pain rehabilitation center if symptoms remain refractory to first- and second-line treatments 1

Critical Pitfalls to Avoid

• Do not dismiss this as simple irritation or anxiety without proper evaluation, as EM patients have increased morbidity, mortality, and suicide risk compared to the general population 1
• Do not recommend unlimited ice or cold water immersion, as this causes tissue damage, ulceration, and "immersion foot" with progressive disability 1
• Do not delay aspirin trial if no contraindications exist, as this is low-cost, low-risk, and potentially highly effective, particularly if myeloproliferative disease is present 1
• Do not use sedating antihistamines long-term if pruritus develops, especially given the patient's young age, as these have limited efficacy for chronic symptoms 2
• Do not assume heat or cold therapy alone will suffice—while both provide mild symptomatic relief for musculoskeletal pain, the underlying vasomotor dysfunction in EM requires targeted pharmacologic intervention 3

Follow-Up and Monitoring

• Reassess at 4 weeks after initiating first-line therapy to determine response 1
• If no improvement after 4-6 weeks of appropriate therapy, refer to dermatology or pain medicine for specialized evaluation 2
• Monitor for skin breakdown from excessive cooling behaviors and optimize wound healing with frequent emollient application if breakdown occurs 2
• Screen for depression and anxiety, as chronic pain conditions significantly impact quality of life and may require cognitive-behavioral therapy referral 1