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Case reports: multiple nonmelanoma skin cancers n a patient with epidermolytic hyperkeratosis on long-standing retinoid therapy.(Case study)

Publication: Journal of Drugs in Dermatology
Publication Date: 01-MAY-08
Format: Online
Delivery: Immediate Online Access

Article Excerpt
Abstract

Epidermolytic hyperkeratosis is a rare genetic disorder of kcratinization. In childhood, patients are erythrodermic and have a compromised stratum corneum, replaced with generalized hyperkeratosis as the patients age. Treatment consists of topical emollients as well as, topical A...

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...and oral retinoids. Ultraviolet (UV) light, often in combination with psoralen ultraviolet (PUVA) is widely used as a therapeutic modality for a multitude of hyperproliferative disorders. Although not strictly indicated for epidermolytic hyperkeratosis, it has been utilized as experimental treatment, particularly in the days prior to retinoids. Psoralen ultraviolet A has also been implicated in the development of nonmelanoma skin cancers, especially, squamous cell carcinoma (SCC). Retinoids are well-known to protect against nonmelanoma skin. A patient with epidermolytic hyperkeratosis with multiple nonmelanoma skin cancers, previously treated with PUVA and long-standing oral retinoids is reported.

Introduction

Nonmelanoma skin cancers are the most common forms of human malignancy, with an estimated yearly incidence of 1 000 000 in the [US.sup.1] Ninety-five percent of these cancers are comprised of basal cell carcinoma (BCC) and squamous cell carcinoma (SCC). The etiology most frequently reported is DNA damage from ultraviolet (UV) light [exposure.sup.2-4] Interestingly, UV light is also in the dermatologist's therapeutic armamentarium. It is useful in treating a variety of dermatologic diseases especially those characterized by kcratinocyte hyperproliferation such as psoriasis in the form of ultraviolet B (UVB) light therapy, narrowband UV light therapy, and ultraviolet light A (UVA) therapy along with psoralen ultraviolet A (PUVA).' Psoralen ultraviolet A has a known association with the development of nonmelanoma skin cancer, particularly squamous cell carcinoma.(6)

Epidermolytic hyperkeratosis (EHK) is a genodermatosis that falls into the category of a proliferative disorder and is generally treated by emollients and systemic retinoids, the latter of which have been shown to prevent the development of skin [cancers.sup.7-9] Prior to the advent of systemic retinoids for this disorder, experimental therapies such as PUVA were often employed. Herein, a case of multiple nonmelanoma skin cancers in a patient with epidermolytic hyperkeratosis previously treated with PUVA and long-standing retinoids is reported.

Case

A 55-year-old Caucasian woman...

NOTE: All illustrations and photos have been removed from this article.



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