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…
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…
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…
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…
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…
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…
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…
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…
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.…
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…
An autoimmune disease develops when the body’s immune system fails to recognize normal body tissues and attacks and destroys them as if they were foreign, rather than attacking an outside organism. The cause is not fully understood, but in some…