Drug-induced pemphigus

What is pemphigus?

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

Types of pemphigus include:

• Pemphigus vulgaris
• Leaf Pemphigus
• Benign family pemphigus (Hailey – Hailey disease)
• Pemphigus erythematosus
• Paraneoplastic pemphigus
• Drug-induced pemphigus.

What is drug-induced pemphigus?

Drug-induced pemphigus is diagnosed when a form of pemphigus has been detected precipitate due to a medication Pemphigus may appear days, weeks, or up to 6 months after the medication has been started.

Who gets drug-induced pemphigus?

More than 200 cases of drug-induced pemphigus have been reported in the medical literature. Reports suggest that patients with drug-induced pemphigus may have features similar to non-drug-induced pemphigus.

• Race: Pemphigus may be more common in Jews of Ashkenazi descent
• Sex – more cases were seen in women than in men in some studies
• Age: Most literature reports cases seen in individuals between the ages of 30 and 90 years.

What causes drug-induced pemphigus?

Medicines that cause pemphigus include:

• Thiol drugs, including penicillamine, captopril

• Antibiotics: penicillins, cephalosporins, vancomycin.

• Antihypertensive medications: other angiotensin converters enzyme inhibitors such as cilazapril, lisinopril, enalapril
• Piroxicam.

Drug-induced pemphigus is usually caused by the induction of an immune reaction to desmoglein proteins and is identical to idiopathic forms of pemphigus In some cases, when antibodies against desmoglein have not been detected, a biochemical cause for drug-induced pemphigus is suspected.

What are the clinical characteristics of drug-induced pemphigus?

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

Thiol medications cause pemphigus foliaceus.

• Pemphigus foliaceus presents with erythematous, scaly or crusted plates.
• Vesicles, bullas and erosions may or may not be present.
• It most often affects the trunk.
• Oral mucous membrane He is not involved.

Non-thiol medications most commonly cause pemphigus vulgaris.

• Pemphigus vulgaris presents with pain flaccid vesicles and blisters.
• It affects the scalp, face and trunk.
• The blisters can affect the oral mucosa.

How is drug-induced pemphigus diagnosed?

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

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

How is drug-induced pemphigus treated?

Management of drug-induced pemphigus includes:

• Withdrawal of the offending drug
• Systemic corticosteroids

• Immunosuppressive drugs.

What is the outcome for drug-induced pemphigus?

The outcome of 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.

• Patients whose pemphigus has been induced by non-thiol drugs and have detected antibodies have better forecast.
• About 50% of thiol-induced cases will resolve when the offending drug is discontinued.