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Pemphigus and pemphigoid are autoimmune blistering diseases caused by antibodies targeting skin adhesion proteins, requiring immunosuppressive management.
Pemphigus and pemphigoid are a group of autoimmune blistering diseases in which the immune system produces antibodies against structural proteins responsible for maintaining the integrity of the skin and mucous membranes. These conditions result in the formation of blisters and erosions that can be debilitating if left untreated. Although relatively uncommon, pemphigus and pemphigoid require prompt diagnosis and long-term immunosuppressive therapy to achieve disease control.
Pemphigus encompasses conditions in which blistering occurs within the epidermis (intraepidermal). The most common subtypes are pemphigus vulgaris, characterised by flaccid blisters and mucosal erosions, and pemphigus foliaceus, which affects the superficial epidermis and typically spares the mucous membranes. Pemphigoid refers to a group of subepidermal blistering diseases, with bullous pemphigoid being the most prevalent form, particularly in elderly populations.
In pemphigus, autoantibodies are directed against desmogleins, the adhesion molecules that hold keratinocytes together within the epidermis. Pemphigus vulgaris is associated with antibodies against desmoglein 3 (and often desmoglein 1), while pemphigus foliaceus involves antibodies against desmoglein 1 alone. The loss of cell-to-cell adhesion, termed acantholysis, leads to intraepidermal blister formation.
In bullous pemphigoid, autoantibodies target the hemidesmosomal proteins BP180 (collagen XVII) and BP230 at the dermal-epidermal junction. This results in subepidermal separation and the formation of tense blisters. Bullous pemphigoid is the most common autoimmune blistering disease and predominantly affects individuals over the age of 70.
Risk factors and associations include:
The clinical presentation differs between pemphigus and pemphigoid, reflecting the distinct levels of blister formation within the skin.
Pemphigus vulgaris typically presents with painful erosions of the oral mucosa, which are often the earliest manifestation. Flaccid, fragile blisters develop on the skin and rupture easily, leaving behind raw, weeping erosions that are slow to heal. The Nikolsky sign (extension of a blister or separation of the epidermis with lateral pressure) is characteristically positive. Common findings include:
Pemphigus foliaceus presents with superficial, crusted erosions and scaly plaques, often in a seborrhoeic distribution (face, scalp, chest, and upper back). Mucous membrane involvement is typically absent in pemphigus foliaceus.
Bullous pemphigoid is characterised by:
The diagnosis of pemphigus and pemphigoid requires a combination of clinical assessment, histopathology, and immunological testing. A skin biopsy is essential and typically includes two specimens: one from the edge of a fresh blister for routine histology, and one from perilesional skin for direct immunofluorescence (DIF). DIF reveals characteristic patterns of antibody deposition that distinguish pemphigus from pemphigoid and from other blistering disorders.
Additional serological testing includes indirect immunofluorescence and enzyme-linked immunosorbent assay (ELISA) for specific autoantibodies. In pemphigus, ELISA for anti-desmoglein 1 and anti-desmoglein 3 antibodies is both diagnostic and useful for monitoring disease activity. In bullous pemphigoid, ELISA for anti-BP180 and anti-BP230 antibodies aids in confirming the diagnosis. Specialist evaluation by a dermatologist experienced in autoimmune blistering diseases is critical for accurate diagnosis and management.
The treatment of pemphigus and pemphigoid is guided by disease severity, the extent of skin and mucosal involvement, and the patient's overall health. The primary goals are to suppress autoantibody production, control blistering, promote healing of erosions, and minimise treatment-related side effects.
Systemic corticosteroids (prednisone) remain a mainstay for achieving initial disease control, particularly in pemphigus vulgaris. High-dose oral corticosteroids are typically initiated at diagnosis and gradually tapered as steroid-sparing agents take effect. In bullous pemphigoid, potent topical corticosteroids (clobetasol propionate applied to the entire body surface) have been shown to be as effective as systemic corticosteroids for moderate disease, with fewer systemic side effects.
Steroid-sparing agents are introduced early in the treatment course to allow corticosteroid tapering and to maintain long-term remission. Commonly used immunosuppressants include azathioprine, mycophenolate mofetil, and, less frequently, cyclophosphamide for severe or refractory disease. These medications require regular laboratory monitoring through a structured prescription management programme to ensure safety.
Rituximab, a monoclonal antibody targeting CD20-positive B lymphocytes, has emerged as a first-line treatment for pemphigus vulgaris in combination with low-dose corticosteroids. Rituximab has been shown to induce complete and sustained remission in a significant proportion of patients and is now recommended early in the disease course rather than being reserved for refractory cases. The role of rituximab in bullous pemphigoid is also being investigated, with growing evidence supporting its use in treatment-resistant disease. Biologic and advanced small molecule therapy is available at the Centre for Medical and Surgical Dermatology for eligible patients.
Topical wound care is an important component of the management of both pemphigus and pemphigoid. Non-adherent dressings, gentle cleansing, and topical antiseptics are used to promote healing of erosions and to prevent secondary infection. For localised bullous pemphigoid, potent topical corticosteroids may be sufficient as monotherapy. Oral hygiene measures and topical corticosteroid mouthwashes are used to manage oral mucosal involvement in pemphigus vulgaris.
Urgent medical evaluation is recommended if unexplained blistering or persistent erosions develop on the skin or within the mouth. Difficulty eating or swallowing due to oral erosions, widespread blistering, or signs of secondary infection (increasing redness, warmth, pus, or fever) at blister sites all warrant prompt assessment. Early diagnosis and treatment significantly improve outcomes and reduce the risk of complications.
A referral from a family physician is required to access specialist dermatology care. Dr. Maksym Breslavets at the Centre for Medical and Surgical Dermatology provides expert diagnosis and management of autoimmune blistering diseases, including immunosuppressive and biologic therapy.
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