Medication Summary
Because of the rarity of this syndrome, all treatment options are based on sporadic experience and are off-label uses. Topical treatment is usually inadequate, although keratolytics (ie, salicylates, urea) and retinoids can alleviate keratoderma. Systemic retinoids can reverse both the keratoderma and pseudoainhum; however, relapse is the rule upon discontinuation of treatment. [19] If topical retinoids are used in women with childbearing potential, inform patients about teratogenicity and apply current guidelines. Low-dose tretinoin was reported to prevent digital amputation. [20]
Retinoids
Class Summary
Retinoids decrease the cohesiveness of abnormal hyperproliferative keratinocytes and may reduce the potential for malignant degeneration. They modulate keratinocyte differentiation and have been shown to reduce the risk of skin cancer formation in patients who have undergone renal transplantation.
Isotretinoin (Accutane)
Isotretinoin is an oral agent that treats serious dermatologic conditions. It is a synthetic 13-cis isomer of naturally occurring tretinoin (trans-retinoic acid). Both agents are structurally related to vitamin A. It decreases sebaceous gland size and sebum production and may inhibit sebaceous gland differentiation and abnormal keratinization.
An FDA–mandated registry is now in place for all individuals prescribing, dispensing, or taking isotretinoin. For more information, see iPLEDGE. The registry aims to further decrease the risks of pregnancy and other unwanted and potentially dangerous adverse effects during a course of isotretinoin therapy.
Tretinoin topical (Retin-A)
Tretinoin topical inhibits microcomedo formation and eliminates the lesions present. It makes keratinocytes in sebaceous follicles less adherent and easier to remove. It is available as 0.025%, 0.05%, and 0.1% creams. It is also available as 0.01% and 0.025% gels.
Keratolytics
Class Summary
Keratolytics cause cornified epithelium to swell, soften, macerate, and then desquamate.
Salicylic acid topical (Sal-Plant, Salactic film)
By dissolving the intercellular cement substance, salicylic acid produces desquamation of the horny layer of skin, while not affecting the structure of viable epidermis (concentrations of 12-17.6%). Consider the benefit-to-risk ratio in off-label use in Vohwinkel syndrome.
Urea (Ureaphil)
Urea promotes hydration and removal of excess keratin in conditions of hyperkeratosis. Use topical preparations of 10-30%.
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Starfish-shaped plaques.
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Palmar hyperkeratosis.
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Pseudoainhum.
