DISCUSSION
In 1939, two dermatologist-brothers in Georgia saw a patient with this previously unreported condition. They uncovered the family history and worked out the histologic basis, which they then described in the literature. They named the condition benign familial pemphigus, but it is now more commonly known as Hailey-Hailey disease in their honor.
Pemphigus vulgaris (PV), a serious blistering disease, was more common and far more feared at the time of the Hailey brothers’ discovery. Nearly 100% of PV patients died from the condition in that pre-steroid, pre-antibiotic era (most from secondary bacterial infection).
Fortunately, BFP is more benign, though it shares some features with PV. Both are said to be Nikolsky-positive, meaning the initial blisters can be extended with digital pressure. But BFP, unlike PV, does not involve deposition of immunoglobulins (IgA in the case of PV), nor is it accompanied by circulating auto-antibodies. BFP patients typically have no systemic symptoms, whereas in those with PV, the oral mucosae are often affected.
Herpes simplex virus, which was the primary care provider’s initial suspected diagnosis, can cause somewhat similar outbreaks, even in this area. However, it was effectively ruled out by the lack of response to treatment and by the biopsy results.
Although BFP is an inherited condition, it demonstrates variable penetrance, as in our case. It is rare enough that diagnosis is almost invariably delayed while other diagnoses are considered and treated. The actual “lesion” of BFP is still debated, but appears to involve the quality and quantity of desmosomes (microscopic structures that act as connecting fibers between layers of tissue) breaking down, often because of heat and friction, eventuating in blistering. This theory is bolstered by considerable research and by the fact that most cases present in intertriginous areas, such as the neck, axillae, and groin. Appearing episodically, it typically begins in the third to fourth decade of life, tending to diminish with age.
Biopsy is often necessary to confirm the diagnosis of BFP, with the sample best taken from perilesional skin to avoid separation of friable sample fragments. Additional specimens can be taken for special handling (Michel’s media) to detect immunoglobulins that might be seen in other blistering diseases.
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