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Hailey-Hailey disease (Familial Benign Pemphigus)

Familial benign pemphigus or Hailey-Hailey's disease is a rare genodermatosis with dominant inheritance that is classically described as a blistering disorder but actually presents as an erythematous, erosive, oozing condition with cracks and fissures localized to the nape of the neck, axillae, submammary regions, unguinal folds, and scrotum. The underlying pathologic process is acantholysis whereby the fragility of the epidermis is probably due to a defect in the adhesion complex between desmosomal proteins and tonofilaments. Onset is usually between the third and fourth decade, and the disease is often mistaken for intertrigo, candidiasis, frictional or contact dermatitis. Individual lesions consists of microscopically small flaccid vesicles on an erythematous background that soon turn into eroded plaques with the described, highly characteristic, fissured appearance. Crusting, scaling, and hypertrophic vegetative growths may occur. Histology explains the clinical appearance as epidermal cells lose their coherence with acantholysis throughout the epithelium, giving the appearance of a dilapidated brick wall.

Colonization of the lesions, particularly by Staphylococcus aureus is a trigger for further acantholysis and maintenance of the pathologic process. Secondary colonization by Candida has a similar effect.

Causes of Hailey-Hailey disease

Those with benign familial chronic pemphigus have a decreased number of desmosomes in the skin.  Desmosomes are like little rivets that hold the skin cells together.  Because the skin cells are not as attached together as well as normal skin, conditions such as heat and frictional pressures may cause Hailey Hailey disease to flare.

Benign Familial Chronic Pemphigus is a genetic disease. The predisposition to get this disease is carried within your genes.  Children of affected people have a fifty-fifty chance of also getting the disease.  About one third may get the condition without a family history.

Signs and symptoms of Hailey-Hailey disease

People with Hailey-Hailey disease usually lead full, normal lives - their condition being a nuisance rather than a serious problem, even though the affected skin is itchy and uncomfortable, and the patches come and go. The skin then tends to macerate leaving quite painful cracks. Secondary bacterial infection, which is not uncommon, can give rise to an unpleasant smell. White bands on the fingernails and pits in the palms can also occur. Heat, sweating and friction often exacerbates the disease, and most patients have worse symptoms during the summer months.

Diagnosis

Usually Hailey-Hailey disease is diagnosed by its appearance and the family history, but it is often is mistaken for other skin problem. Impetigo , thrush , tinea (jock itch) and other blistering conditions look similar.

Diagnosis may require a skin biopsy. The histology is characteristic, with layers of detached skin cells ('acantholysis'). Unlike pemphigus vulgaris , the immunofluorescence test for antibodies is negative.

Treatment of Hailey-Hailey disease

Treatment rests on anti-infective agents, administered both topically and systemically; systemically tetracyclines seem to work better than most. Topical glucocorticoids depress the anti-inflammatory response and accelerate healing. In severe cases, dermabrasion or carbon dioxide laser vaporization leads to healing with scars, which are resistant to re­currences. The condition becomes less troublesome with age.

The affected skin may smell unpleasant, particularly in moist areas. The smell is part of the skin condition and does not mean that the skin is dirty. Bacteria growing in the rash probably cause it. Careful washing is important and antiseptic solutions for the bath, antiseptic creams or antibiotics may help.

Prevention of Hailey-Hailey disease

Heat, sweating or friction may make the spots appear and it is sensible to try and avoid these, for example it may be helpful to wash a new shirt to soften a stiff collar before wearing it. Wear cool comfortable clothing and keep your weight down to minimise friction.