Abstract BACKGROUND: Hailey-Hailey disease (HHD) is a rare hereditary disease in which the genetic defect is characterized by mutation in the ATP2C1 gene coding for a transmembrane calcium pump. It is generally considered a non-immunologic acantholytic dermatosis in which direct…
年別アーカイブ: 2012
Acantholytic rosacea of the forehead and scalp in a patient with Hailey-Hailey disease
BACKGROUND: Rosacea is a common dermatosis that can involve the bald area of the scalp. We report the case of a man presenting clinical symptoms of rosacea of the forehead and the scalp, but with a histological picture of familial…
A case of stable oral pemphigus vulgaris on low dose immunosuppressive therapy
The clinical and epidemiological features of pemphigus vulgaris (PV) are well documented but there remain few reports of oesophageal involvement of PV. Although previously considered to be rare, recent reports have suggested that up to 87% of patients with PV…
Pemphigus foliaceus masquerading as IgA pemphigus and responding to dapsone
A 14-year-old male presented with seven years history of recurrent episodes of fluid filled, itchy and eroded lesions over the body not responding to oral corticosteroids and azathioprine. Dermatological examination revealed crusted plaques and erosions in a seborrheic distribution. Histopathology…
Findings around hair follicles and sweat glands in immunobullous disease
Background Inherent to some immunobullous disorders is potential for intraepidermal or dermal–epidermal junction fragility, a phenomenon that may compromise biopsy specimen integrity and direct immunofluorescence (DIF) interpretation. In these situations, cutaneous adnexal structures (e.g. hair follicles, sweat apparatus) usually remain…
Polymorphisms of HLA microsatellite marker in Tunisian pemphigus foliaceus.
Pemphigus foliaceus (PF) is an autoimmune blistering skin disease that partly results from genetic factors, especially from human leucocyte antigen (HLA) class II genes. Several data have reported the involvement of microsatellite (STR) markers across different regions of the HLA…
Clinical and immunological profile of umbilical involvement in pemphigus vulgaris and pemphigus foliaceus
Background. Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune vesicobullous disorders with IgG autoantibodies directed against desmoglein (Dsg)1 and 3, which lead to intraepidermal acantholysis. Aim. To characterize the clinical and immunological profile of patients with PF or PV with umbilical…
タグ: desmoglein, Erythema, pemphigus foliaceus,
尋常性天疱瘡
カテゴリー: Around the Globe, 臨床試験, News and Information
カテゴリー: Around the Globe, 臨床試験, News and Information
Analysis of High-dose Intravenous Immunoglobulin Therapy in Patients
High-dose intravenous immunoglobulin (IVIG) therapy is used in patients with severe autoimmune blistering diseases that are refractory to standard immunosuppressive therapy. To determine the efficacy and frequency of adverse events of IVIG therapy, we retrospectively analysed data for 16 patients…
カテゴリー: Around the Globe, News and Information
Familial benign chronic pemphigus and doxycycline
Background Hailey-Hailey disease (HHD) or familial benign chronic pemphigus is a rare autosomal dominant inherited skin disorder, characterized by flaccid vesicles and erosions on the intertriginous areas. Current treatments are not particularly effective. We report 6 cases dramatically improving with doxycycline.…
Specific immunoglobulin isotypes correlate with disease activity, morphology, duration and HLA association in Pemphigus vulgaris.
The molecular basis of disease heterogeneity in autoimmune conditions such as Pemphigus vulgaris is poorly understood. Although desmoglein 3 (Dsg3) has been well established as a primary target of immunoglobulin (Ig) autoantibodies in PV, there remain several questions regarding the…
タグ: autoantibodies, desmoglein 3, HLA, IgG1, IgG4, immunoglobulin, isotype,
尋常性天疱瘡
カテゴリー: Around the Globe, News and Information
カテゴリー: Around the Globe, News and Information
