clinical dermatology research journal
The term erythrokeratodermia is applied to a group of inherited disorders of keratinisation characterized by well demarcated erythematous lesions and hyperkeratotic plaques. Erythrokeratoderma en cocardes is an atypical variant characterised by the presence of transient hyperkeratotic and erythematous plaques. The pathogenetic mechanisms of most of the erythrokeratodermia are related to mutations in the connexin gene family, but until now, there is no information concerning the genetic alterations that are responsible for the appearance of the erythrokeratoderma en cocardes. We report the case of a 31-year-old woman with a 5-year history of fixed erythematous plaques with scaly rims on the arms, trunk, axillae and limb flexures that disappeared spontaneously and recurred every summer.
Oral Manifestations of Pemphigus Vulgaris: Clinical Presentation, Differential Diagnosis and Management
Pemphigus vulgaris is a chronic autoimmune mucocutaneous disease characterized by the formation of intraepithelial blisters. It results from an autoimmune process in which antibodies are produced against desmoglein 1 and desmoglein 3, normal components of the cell membrane of keratinocytes. The first manifestations of pemphigus vulgaris appear in the oral mucosa in the majority of patients, followed at a later date by cutaneous lesions. The diagnosis is based on clinical findings and laboratory analyses, and it is usually treated by the combined administration of corticosteroids and immunosuppressants. Detection of the oral lesions can result in an earlier diagnosis. We review the oral manifestations of pemphigus vulgaris as well as the differential diagnosis, treatment, and prognosis of oral lesions in this uncommon disease.