Medical Care

Systemic retinoid therapy with acitretin (Soriatane) or isotretinoin (Accutane) can induce dramatic improvement.^[56]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.^[57]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 H₁antihistamines are useful. Anecdotally, narrowband ultraviolet B has been beneficial in the treatment of EKVP.

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

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.

Medication

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Media Gallery

Figurate erythema. Courtesy of M. King and J. Crawford.
Targetlike erythema. Courtesy of M. King and J. Crawford.
Generalized hyperkeratosis with scaling, accentuated skin lines, and figurate erythema. Courtesy of M. King and J. Crawford.
Thick hyperkeratotic plates with hystrixlike spines. Courtesy of M. King and J. Crawford.
Sharply demarcated, figurate, hyperkeratotic plaques in a symmetric distribution. Courtesy of M. King and J. Crawford.
Figurate hyperkeratotic plaque with erythematous patches. Courtesy of M. King and J. Crawford.
Plantar keratoderma with peeling. Courtesy of M. King and J. Crawford.
Diffuse glovelike palmar keratoderma. Courtesy of M. King and J. Crawford.

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Sections Erythrokeratodermia Variabilis et Progressiva
Overview
Presentation
DDx
Workup
Treatment
Medication
Media Gallery
References

Erythrokeratodermia Variabilis et Progressiva Treatment & Management

Medical Care

Long-Term Monitoring

References

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