Drug-induced pemphigus

Author: Brian Wu, MD candidate, Keck School of Medicine, Los Angeles, USA. DermNet New Zealand Editor in Chief: Hon A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy editor: Maria McGivern. June 2017.

What is pemphigus?

Pemphigus refers to a group of autoimmune diseases that cause blistering. Pemphigus is characterised by the development of antibodies directed against a protein (either desmoglein 1, desmoglein 3 or both) involved in cell-to-cell adherence in the epidermis.

Types of pemphigus include:

What is drug-induced pemphigus?

Drug-induced pemphigus is diagnosed when a form of pemphigus has been precipitated by a medication. Pemphigus may occur days, weeks, or as long as 6 months after the drug has been commenced.

Who gets drug-induced pemphigus?

Over 200 cases of drug-induced pemphigus have been reported in the medical literature. Risk factors include:

What causes drug-induced pemphigus?

Drugs that cause pemphigus include:

Drug-induced pemphigus is usually caused by the induction of an immune reaction to desmoglein proteins and it is identical to the idiopathic forms of pemphigus. In some cases, when no antibodies to desmoglein have been detected, a biochemical cause for the drug-induced pemphigus has been suspected.

What are the clinical features of drug-induced pemphigus?

The clinical features depend on whether the drug-induced pemphigus has been induced by thiol or non-thiol drugs.

Thiol drugs cause pemphigus foliaceus.

Non-thiol drugs most commonly cause pemphigus vulgaris.

How is drug-induced pemphigus diagnosed?

The diagnosis of drug-induced pemphigus can be challenging, especially in patients on multiple drugs as the period between the ingestion of the causative drug and the onset of symptoms can be prolonged. Careful, thorough history-taking is vital.

The diagnosis of pemphigus is confirmed by its characteristic intraepidermal blistering when seen on histology of a skin biopsy, and the presence of typical antibodies on direct immunofluorescence. Circulating pemphigus antibodies against desmoglein 1 and/or desmoglein 3 may be detected on serology.

How is drug-induced pemphigus treated?

The management of drug-induced pemphigus includes:

What is the outcome for drug-induced pemphigus?

The outcome for drug-induced pemphigus depends on whether the patient has developed antibodies and whether the cause of the condition is a thiol or non-thiol drug.

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