Dsg: desmoglein H&E: hematoxylin-eosin IL: interleukin PH: pemphigus herpetiformis INTRODUCTION Pemphigus herpetiformis (PH) is a rare variant of pemphigus and accounts for approximately 7% of cases. PH combines the clinical features of dermatitis herpetiformis with the histologic features of pemphigus. Clinically, the plaques are herpetiform or even annular. PH follows a more benign course wit...

Hailey-Hailey disease (HHD, MIM 16960) is an autosomal dominant disease characterized by suprabasal cell separation (acantholysis) of the epidermis. The clinical features vary and include crusted erosions with vesicular pustules, and erythematous scaly plaques at sites of friction and flexures. The skin lesions are often exacerbated by heat, sweating, mechanical trauma, infection and exposure i...

Pemphigus vulgaris (PV) is a chronic autoimmune bullous disease characterized by the formation of suprabasal cleavage and acantholysis. As this disease almost always affects the oral mucosa, conventional cytological smears of oral lesions can be used for the initial diagnosis of PV. We report two cases of PV that were initially diagnosed based on cytological smears of an oral sample. As atypica...

Brownstein described a previously unrecognized benign acanthoma, the "Acantholytic acanthoma" in 1988. We report a rare case of acantholytic acanthoma on the right labia minor of vulva in a 34-year-old woman. Due to the rarity of this lesion in the female genitalia, it may be confused with other lesions more commonly affecting this region. Correct diagnosis of acantholytic acanthoma may result ...

pemphigus vulgaris (pv) is one of the most common and studied autoimmune disorders of skin. the knock down of desmogleins in the skin by igg antibodies will eventually leads to mucocutaneous problems in both genders. targeting the cardinal molecules involved in the pathways of immune response is the main goal of given therapy. with regard to the side effects of long term use of immunosuppressiv...

EBRT: electron beam radiation therapy GRT: Grenz ray therapy HHD: Hailey-Hailey disease INTRODUCTION Hailey-Hailey disease (HHD), or familial benign chronic pemphigus, is an autosomal dominant genodermatosis. In this condition, dysregulation of intracellular calcium homeostasis leads to impaired desmosome function and suprabasilar acantholysis of the epidermis. Clinically, this condition presen...

BACKGROUND Pemphigus vulgaris is a rare autoimmune blistering disease seen only, rarely, in children. CASE-REPORT Two young girls, aged four and 15 years respectively, presented with oral and/or cutaneous blisters (case 2). The diagnosis of pemphigus vulgaris was confirmed by histology (suprabasal acantholysis) and immunopathological analysis (direct and indirect immunofluorescence, antidesmo...

Pemphigus erythematosus (PE) is an autoimmune blistering disease that is characterized by erythema and blisters, but rarely pustules, on the upper torso, face, and scalp. A 37-year-old woman presented with a 20 day history of intense pruritus as well as multiple papules and pustules on the trunk and face. Initially, the disease was misdiagnosed as impetigo due to the presence of pustules, nonty...

Pemphigus herpetiformis (PHF) is a rare variant of pemphigus that combines the clinical features of dermatitis herpetiformis and immunopathological features of pemphigus. In previous case reports, distribution of lesions is usually generalized, involving trunk and limbs. We report two cases of localized PHF presenting with grouped pustules on bilateral dorsal feet and left preauricular area, re...