Pemphigoid

Pemphigoid diseases typically manifest with blistering of the skin and mucous membranes and are associated with autoantibodies that target structural proteins of the hemidesmosome.  Specific disorders are described within the pemphigoid spectrum, and it is important to distinguish the variants because medical implications and treatments differ for the various types.   Bullous pemphigoid, the most common of the pemphigoid disorders, primarily affects the elderly and may be associated with neurologic disease and other chronic illnesses.   A nonbullous variant occurs in up to 20% of patients with pemphigoid  and is associated with delayed diagnosis because it resembles pruritic dermatoses.  Mucus membrane pemphigoid affects ocular and/or oral mucosal surfaces primarily; skin can be affected as well.  Pemphigoid gestationis occurs during pregnancy, generally in the second trimester, and often resolves within 6 months postpartum ; an association with celiac disease serum antibodies has been described.  Two other subtypes of pemphigoid are defined by basement membrane zone (BMZ) antigens targeted by the autoantibodies anti-p200 (laminin gamma-1) and anti-laminin-332, and up to 30% of patients with antilaminin-332 pemphigoid have an associated malignancy. See the Pemphigoid Indications and Clinical Features table below for additional characteristics of the disorders.

The diseases described using the term “pemphigoid” and the various disorders with BMZ antibodies have overlapping clinical manifestations. Certain clinical features can help distinguish the diseases, and they can be further differentiated by specific patterns of antibody binding and specific antigens targeted; therefore, evaluation of tissue-bound and serum autoantibodies is generally required for diagnosis.   Testing involves direct immunofluorescence (DIF) microscopy, serologic assays such as indirect immunofluorescence (also known as indirect fluorescent antibody [IFA]) testing and enzyme-linked immunosorbent assays (ELISAs), and may include immunoblotting or immunoprecipitation.  Broad serologic screening is recommended unless a specific immunobullous skin disease type is suspected.

Tabs Content
Content Review: 
October 2019

Last Update: October 2019