Netherton syndrome is a rare disorder inherited in an autosomal recessive pattern consisting of ichthyosiform dermatosis, hair shaft abnormalities (trichorrhexis invaginata), and an atopic diathesis. Patients with Netherton syndrome have been found to have a mutation on chromosome 5q32 in a gene named SPINK5 (serine protease inhibitor, Kazal type-5), which encodes an inhibitor of serine proteases called LEKTI. We report a female patient with previously undiagnosed Netherton syndrome who presented to participate in a clinical research trial investigating the benefit of topical tacrolimus 0.03% ointment [Protopic (Fujisawa Pharmaceutical Co. Ltd., Japan)] for the treatment of atopic dermatitis. This patient was confirmed to have a gene mutation in SPINK5. Current literature suggests a relative contraindication for use of topical tacrolimus in patients with Netherton syndrome owing to concern for increased systemic absorption of the drug. Our patient was not able to tolerate topical tacrolimus owing to local irritation, and did not derive any benefit from therapy. Though rare, when evaluating patients with a possible diagnosis of atopic dermatitis, an index of suspicion for Netherton Syndrome must be maintained. History and overall clinical findings, especially in regards to examination of the hair, will aid in diagnosis.
Bowen disease usually presents as an irregular, asymptomatic, scaly or crusted erythematous plaque that can occur anywhere on the skin. An unusual clinicopathologic variant is described which presents as a well-circumscribed, papillated, exophytic and endophytic, sometimes keratotic lesion. This papillated variant of Bowen disease exhibits keratinocytes with prominent perinuclear halos suggestive of koilocytic change associated with human papillomavirus (HPV) infections. Classic Bowen disease has been associated in previous studies with a variety of HPV types, especially types 16, 18, and 31. Twenty-six patients with papillated Bowen disease were evaluated. The patients included 15 males and 11 females, ranging in age from 33 to 87 years old. Fifty-four percent (14) of the lesions involved the head and neck, 8% (2) involved the trunk, and the remaining 38% (10) involved extremities (including 3 lesions from the hands). Lesions were examined using in situ hybridization with widely screening genomic probes for HPV types 6, 11, 16, 18, 30, 31, 33, 35, 45, 51, and 52. None of the specimens contained HPV DNA from the more common oncogenic HPV types. Given the striking histologic appearance of these lesions, however, this does not exclude HPV infection detectable by more sensitive screening methods such as polymerase chain reaction. Papillated Bowen disease is distinct from other variants, including the verrucous-hyperkeratotic type.
Nonmelanoma skin cancer is extremely common and is increasing in incidence. It would be very useful to have forms of therapy that would prevent precancerous changes from going on to form cancer, or to reverse the precancerous changes. Epidemiologic evidence in humans, in vitro studies on human cells, and clinical experiments in animals have identified polyphenol compounds found in tea to be possibly useful in reducing the incidence of various cancers, including skin cancer. To examine the potential for a polyphenol from green tea, epigallocatechin gallate, to act as a chemopreventive agent for nonmelanoma skin cancer, a randomized, double-blind, placebo-controlled phase II clinical trial of topical epigallocatechin gallate in the prevention of nonmelanoma skin cancer was performed.
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