British Association of Dermatologists' Guidelines for the Management of Pemphigus Vulgaris 2017

K.E. Harman; D. Brown; L.S. Exton; R.W. Groves; P.J. Hampton; M.F. Mohd Mustapa; J.F. Setterfield; P.D. Yesudian


The British Journal of Dermatology. 2017;177(5):1170-1201. 

In This Article

Induced Pemphigus Vulgaris

Drugs can trigger pemphigus but this is uncommon. The diagnosis is challenging because drug-induced cases resemble idiopathic pemphigus, there are no clinical or laboratory tests that can distinguish them reliably and the latency between starting the drug and disease onset can be several months. Therefore, a thorough drug history is essential, cross-checking against drugs reputed to trigger pemphigus (Table 3).[275,276] A poor response to standard systemic treatments should also alert to the possibility of drug-induced pemphigus (DIP).

There are three groups of chemical structures that have been suggested to cause drug-induced pemphigus: thiol drugs, which have a sulfhydryl radical, phenol drugs and nonthiol, nonphenol drugs (Table 3). PF is the most common pattern of DIP, observed in up to 70% of thiol-induced cases. Nonthiol drugs tend to trigger a PV phenotype. Pruritus is more common in DIP than in idiopathic pemphigus.[275,277] Diagnostic investigations are as for idiopathic pemphigus, with no immunopathological features in routine investigations that differentiate.[278]

Initial management of DIP includes stopping the offending drug, possibly combined with conventional treatment in severe cases to hasten remission. Thereafter, it may follow two courses: the disease may continue in 50% in spite of drug withdrawal (DIP) while others recover completely (drug-triggered pemphigus).[279] Recovery following drug withdrawal is more likely in thiol-triggered cases. In patients who do not remit upon drug withdrawal, the course and prognosis are similar to those in idiopathic disease and should be managed as such.