What Is Benign Familial Pemphigus?

Most people inherit benign familial pemphigus, but sporadic cases without familial genetic involvement may happen. Changes in the ATP2C1 gene, in chromosomes 3q 21-24, cause benign familial pemphigus.

Hailey-Hailey disease or benign familial pemphigus causes cells to fall apart from their usual packed position.

The typical symptom of benign familial pemphigus is painful, erosive, crusted skin rash in skin folds.

• The lesions come and go but do not leave any scars
• As the lesions grow in size, the center clears, revealing a typical ring shape
• If the lesions are present for an extended period, they may thicken
• The lesions are mostly bilateral and rarely unilateral

In most people, the condition could be mild. They may have issues, such as pain and smell problems. Secondary infection may lead to an unpleasant smell.

Herpes simplex virus can infect skin blisters, spread the lesion, and cause pain and itching.

Benign familial pemphigus is diagnosed by identifying its characteristic features and analyzing the person’s family history. A few skin conditions, such as impetigo, thrush, and tinea cruris could be misdiagnosed as other skin conditions.

• A skin biopsy could be required
• The immunofluorescence test for antibodies is negative
• There is no particular test for family members

There is no treatment for benign familial pemphigus. The focus of the treatment is symptom relief.

The topical prescription includes:

• Topical corticosteroid ointments to treat inflamed lesions
• Topical antibiotics, such as clindamycin and mupirocin, or a combination of antibiotics for short periods to fight infection
• Other corticosteroids
• Benzoyl peroxide (cream or wash)
• Ketoconazole to treat fungal infections
• Fluorouracil

The oral prescription includes:

• Long-term courses of antibiotics, such as tetracyclines
• Oral antiviral medications, such as acyclovir, to treat recurrent herpes infections
• Medications, such as glycopyrrolate, to reduce excess sweating
• Phototherapy and photodynamic therapy

Dermabrasion, CO₂ laser ablation, and pulsed dye laser therapy have varying degrees of success to treat benign familial pemphigus. Intracutaneous botulinum toxin injection reduces sweating.

1. Stay indoors
2. Avoid the sun and other trigger factors
3. Wash and dry skin folds regularly
4. Wear soft and loose clothes
5. Maintain a healthy weight
6. Take bleach baths two times a week to avoid superficial infections
7. Apply zinc paste to the infected areas