PH is a rare condition, with approximately 100 reported cases, typically manifesting in middle-aged adults as an intensely pruritic cutaneous eruption with herpetiform clusters of vesicles. It is even rarer in children, with fewer than 10 reported pediatric cases. No ethnic or sex predilections have been observed.
The inciting factors of the disease have not been identified. IgG autoantibodies bind to desmoglein 1 (less commonly, desmoglein 3 or desmocollin 1 or 3) on keratinocytes, stimulating upregulation of interleukin-8, which serves as a chemoattractant for eosinophils and neutrophils. In turn, these inflammatory cells secrete proteases that mediate eosinophilic or neutrophilic spongiosis and blister formation.
Unlike the classical pemphigus disorders, PH usually exhibits a benign clinical course. However, cases of PH progressing to PF or PV have been reported.
L10.89 – Other pemphigus
84855007 – Acantholytic vesicular dermatitis
- Dermatitis herpetiformis – History of gluten-insensitive enteropathy. Can differentiate with histopathological survey and immunofluorescence studies, as well as serologic testing.
- Pemphigus foliaceus (PF) – Clinical appearance of PH resembles dermatitis herpetiformis more so than PF.
- Pemphigus vulgaris (PV) – Clinical appearance of PH resembles dermatitis herpetiformis more so than PV. Lesions of PV tend to be painful, in contrast to the nontender, pruritic lesions of PH. PV commonly involves the mucous membranes, which are often spared in PH.
- Bullous pemphigoid – Can differentiate with histopathological survey and immunofluorescence studies.
- Linear IgA bullous dermatosis – Can differentiate with histopathological survey and immunofluorescence studies.
- IgA pemphigus – Can differentiate with histopathological survey and immunofluorescence studies.
- Bullous drug eruption
- Allergic contact dermatitis
Last Updated: 10/01/2019