What is the best management for Erythromelanosis faciei et colli?

Management of Erythromelanosis Faciei et Colli

The best management for Erythromelanosis Faciei et Colli (EFFC) includes topical treatments targeting erythema and pigmentation, with topical corticosteroids and sun protection as first-line therapies.

Understanding EFFC

EFFC is a rare disorder characterized by a clinical triad of:

• Well-demarcated erythema
• Hyperpigmentation
• Follicular papules primarily affecting the face and neck 1, 2

The condition typically presents on the maxillary, preauricular regions, and cheeks, but can also involve eyebrows, forehead, upper lips, and pinna 3. It affects both males and females, with onset typically during adolescence or early adulthood 4, 3.

First-Line Management

Topical Treatments for Erythema

• Topical vasoconstrictors:
  • Compounded midodrine 0.2% (preferred treatment)
  • Alternative options: phenylephrine, oxymetazoline 0.05%, timolol maleate 0.5%, or brimonidine tartrate 0.33% 5
  • Apply once daily to affected areas

Topical Treatments for Pigmentation and Follicular Papules

• Topical corticosteroids:
  • For body areas: Moderate-potency corticosteroids like betamethasone valerate 0.1% 6
  • For facial application: Mild-potency corticosteroids like hydrocortisone 1% 6
  • Limit use to 2-3 weeks to avoid side effects 6

Sun Protection (Essential)

• Daily application of broad-spectrum sunscreen (SPF 30+) containing zinc oxide or titanium dioxide 5, 6
• Consistent sun avoidance and use of protective clothing 5
• Wearing hats when outdoors 5

Skin Care Regimen

• Use gentle pH-neutral soaps and shampoos with tepid water 5
• Apply hypoallergenic moisturizing creams daily 5
• Avoid greasy creams that might facilitate folliculitis 5
• Pat skin dry rather than rubbing after washing 5

Second-Line Management

For Persistent Cases

• Topical retinoids may be considered under dermatologist supervision 5
• Oral tetracyclines (doxycycline or minocycline) for anti-inflammatory effects if follicular component is prominent 5

For Symptomatic Relief of Pruritus (If Present)

• Oral antihistamines:
  • Non-sedating: fexofenadine 180mg or loratadine 10mg daily
  • Sedating: cetirizine 10mg if sleep is affected 6

For Severe or Refractory Cases

• Consider systemic treatments for pruritus:
  • Gabapentin (100-300mg TID)
  • Pregabalin (starting at 75mg BID) 6

Monitoring and Follow-up

• Reassess after 4 weeks of topical treatment 6
• If no improvement, consider:
  1. Increasing potency of topical corticosteroids
  2. Adding systemic treatments
  3. Dermatology referral for consideration of advanced therapies 6

Important Considerations

• Avoid treatments that may worsen condition:

  • Excessive washing with hot water
  • Over-the-counter anti-acne medications
  • Chemical irritants 5, 6
  • Manipulation of skin (increases infection risk) 5

• Watch for signs of secondary infection:

  • Yellow crusting or pustules may indicate bacterial infection
  • Consider topical or oral antibiotics if infection suspected 6

• Dermatology consultation is recommended for:

  • Severe cases
  • Cases unresponsive to first-line treatment after 4 weeks
  • Cases with suspected secondary infection 6

EFFC is often underdiagnosed despite being more common than previously thought 3. Early recognition and appropriate management can significantly improve quality of life for affected individuals.