Pemphigus

A group of rare, autoimmune blistering diseases
Includes pemphigus vulgaris and pemphigus foliaceus, and, much more rarely, paraneoplastic pemphigus and neonatal pemphigus

Disease	Frequency	Clinical Manifestations	Diagnostic Clues
Pemphigus vulgaris	60-90%	Almost universal mucosal involvement, mostly oropharyngeal, nasal, and genital	Mucosal lesions, Nikosly sign, positive direct immunofluoresnence
Pemphigus foliaceus	10-30%	Crusted erosions, erythema, scales, mainly in seborrheic areas, and lacks mucosal involvement	Crusted erosions, no mucosal lesions, Nikolsky sign, positive direct immunofluoresncence
Paraneoplastic pemphigus	5%	Severe stomatitis involving tongue and vermilion, erythema multiforme-like and lichenoid skin lesions, associated with leukemia and lymphoma	Severe stomatitis, presence of malignancy, dyskeratosis and interface dermatitis
IgA pemphigus	3%	Flaccid pustules, annular erosions, crusts, and erythematous plaques	Pustules, staining of intercellular IgA in the epithelium by direct immunofluorescence

Further Reading