What Is Benign Familial Pemphigus?

Medically Reviewed on 1/9/2023
Benign Familial Pemphigus
Benign familial pemphigus causes cells to fall apart from their usual packed position.

Benign familial pemphigus or the Hailey-Hailey disease is a rare skin disorder and usually affects younger adults.

  • The predominant sign is red, raw, and blistered areas in skin folds (neck, under the breasts, groin, and armpits)
  • The affected areas get inflamed, crusty, and scaly and cause itching and burning sensation
  • When exposed to dry, hot weather, those rashes may worsen
  • Excess sweat and friction may exacerbate the condition

What are the causes of 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.

What are the signs and symptoms of 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

What are the complications of benign familial pemphigus?

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.

How to diagnose benign familial pemphigus

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

How is benign familial pemphigus treated?

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

The topical prescription includes:

The oral prescription includes:

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.

General tips for people with benign familial pemphigus

  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


Eczema Browse our medical image collection of allergic skin disorders such as psoriasis and dermatitis and more caused by allergies See Images
Medically Reviewed on 1/9/2023
Image Source: iStock image

Benign familial pemphigus. https://dermnetnz.org/topics/benign-familial-pemphigus

Hailey-Hailey disease. https://medlineplus.gov/genetics/condition/hailey-hailey-disease/

Hailey-Hailey Disease. https://rarediseases.org/rare-diseases/hailey-hailey-disease/