Faculty Bibliography

Gyrate erythema, which also is known as erythemaannulare centrifugum (EAC), is a reactive dermatitisthat is thought to occur in response to an underlyingtrigger. The superficial form is characterized bythe typical, centrifugally-expanding, annular,erythematous patches or plaques with a distincttrailing scale. The deep form also is a centrifugallyexpanding,erythematous plaque but with induratedborders and absence of scale. These cutaneousfindings are thought to be reactive, most often inresponse to infections or drugs and, less likely, tounderlying malignant conditions.

Keratolysis exfoliativa (KE) is a palmoplantar eruption of air-filled bullae on an erythematous base, which results in lamellar peeling with hallmark superficial collarettes of scale. It is distinct from other diseases of volar skin, such as dyshidrosis, contact dermatitis, tinea, epidermolysis bullosa, and acral skin peeling. We present a 55-year-old woman with extensive disease on the hands and feet, who failed to respond to standard topical therapy but showed a marked dose-response improvement with the use of oral acitretin. Recent histopathologic and molecular studies have linked KE to premature corneo-desmosomal disruption. Acitretin has previously been used to treat diseases of abnormal corneocyte desquamation, for example Netherton's disease. To the best of our knowledge, this report is the first that documents the efficacy of the use of systemic acitretin in KE.

Chronic actinic dermatitis (CAD) is a photosensitivity disorder that is characterized by a persistent eczematous eruption in sun-exposed sites. The hallmark of CAD is a reduced minimal erythema dose (MED) to ultraviolet B (UVB), ultraviolet A (UVA), and/or to visible light, which makes phototesting the essential diagnostic investigation. The uncommon subgroup of patients with atopic dermatitis (AD) that are affected by CAD has primarily been described in young patients in the United Kingdom. We present an atopic adult women with CAD who was diagnosed years after symptoms began. We believe it is important that dermatologists perform phototests on AD patients with features of a photoaggravated dermatitis in order to avoid delay in diagnosis of a true photosensitivity condition and provide appropriate management.

Oral allergy syndrome (OAS) or pollen-food allergy syndrome (PFS) is a hypersensitivity reaction to plant-based foods, manifesting most commonly with pruritus of the lips, tongue, and mouth. Unlike simple food allergy, OAS requires prior sensitization to a cross-reacting inhalant allergen rather than direct sensitization to a specific food protein. In this review, we summarize the clinical features and pathophysiology of OAS and provide an overview of known pollen-food associations.

Netherton syndrome is a rare, autosomal recessive disorder that is characterized by congenital ichthyosis, trichorrhexis invaginata, and atopic diathesis. Ichthyosis presents at birth with erythroderma and subsequently evolves into ichthyosis linearis circumflexa; hair shaft abnormalities tend to present later. The disorder is caused by loss-of-function mutations in the SPINK5 (serine protease inhibitor Kazal-type 5) gene that encodes LEKTI (lympho-epithelial Kazal-type related inhibitor), which is a protease inhibitor that counteracts epidermal proteases involved in desquamation. Use of topical medications is limited by potential for systemic absorption and toxicity in the setting of a defective skin barrier. Therapeutic options include topical glucocorticoids and retinoids, oral retinoids, and narrowband ultraviolet B phototherapy. Topical tacrolimus has been shown to be efficacious and may be used safely with careful laboratory monitoring.