Erythrokeratodermia Variabilis et Progressiva  Treatment & Management

Updated: May 30, 2017
  • Author: Gabriele Richard, MD, FACMG; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Approach Considerations

The goals of the therapy are to diminish the hyperkeratosis and minimize the discomfort.

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Medical Care

Systemic retinoids are the treatment of choice in extensive erythrokeratodermia variabilis et progressiva (EKVP). [46, 47] Systemic retinoid therapy with acitretin (Soriatane) or isotretinoin (Accutane) can induce dramatic improvement. [48] Systemic retinoids are the treatment of choice for extensive or generalized erythrokeratodermia variabilis. The effect of acitretin or etretinate is superior to that of isotretinoin. The use of retinoids should be considered carefully because long-term therapy is required to achieve continuing results. The minimal effective dose for persons with EKVP usually is very low.

The use of topical agents in the management depends on the symptoms and focuses on hydration, lubrication, and keratolysis. Therapy may include the use of emollients and keratolytics, such as urea, alpha-hydroxy acids, propylene glycol, salicylic acid, and topical vitamin D analogs and retinoid preparations. Newer synthetic retinoids, such as short-contact topical tazarotene, combined with moisturizers seem promising. [49] Masking the erythematous lesions of uncovered skin with makeup and other forms of camouflage may provide a cosmetic benefit. In case of pruritus and burning, mild sedative H1 antihistamines are useful.

The avoidance of trauma to the skin may be also beneficial.

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Complications

Complications are rare with erythrokeratodermia variabilis et progressiva (EKVP). Complications mainly involve the adverse effects of systemic retinoid therapy. Heat intolerance may occur in individuals with generalized hyperkeratosis.

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Activity

Triggers for skin lesions, such as exposure to cold, drastic temperature changes, and mechanical irritation of the skin, should be avoided.

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Long-Term Monitoring

Erythrokeratodermia variabilis et progressiva (EKVP) patients receiving systemic retinoid therapy should be followed up on a regular basis to monitor treatment effectiveness and adverse effects. Establishing a long-term relationship with patients is important in re-evaluating effectiveness of topical therapy and in directing the families to appropriate resources for social and family support. Careful monitoring for adverse effects of retinoid therapy is necessary.

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