Pemphigoid is a group of rare autoimmune blistering skin diseases. As its name indicates, pemphigoid is similar in general appearance to pemphigus, but, unlike pemphigus, pemphigoid does not feature acantholysis, a loss of connections between skin cells.
Signs and Symptoms:
Patients typically have skin lesions, some also have mucous membrane lesions.
1. Non-Bullous Pemphigoid
For some patients the only sign of the disease is cutaneous manifestations of BP with no bullae. Pruritic eczematous, papular, or urticaria-like skin lesions can persist for weeks to months.
2. Bullous Phase
The bullous stage of BP shows vesicles and bulla, appearing on apparently normal or erythematous skin, predominantly at the flexural aspects of the extremities and the lower trunk. Mucosal lesions, which typically are erosions of the oral mucosa, are present in 10 to 30 percent of patients. Occasionally, the blister fluid becomes blood-tinged. The blisters are tense, about 1–4 cm in diameter, leaving eroded and crusted areas, together with urticarial and infiltrated papule.
The pathogenetic mechanism of blister formation is known, the trigger to the formation of the antibodies to the hemidesmosome antigens is still unknown. Most of the bullous pemphigoid cases are due to autoantibodies (mostly IgG) directed at antigens (BP180 and BP230) arranged at the dermal-epidermal junction. However, most commonly, drug can be one of the cause of bullous pemphigoid, such as thiazide diuretics, antibiotics (e.g., penicillins, vancomycin), nonsteroidal anti-inflammatory drugs (NSAIDs) and angiotensin-converting enzyme (ACE) inhibitors (e.g., captopril) and possibly angiotensin receptor blockers (ARBs, e.g., valsartan).
Pemphigoid is more common than pemphigus, and is slightly more common in women than in men. It is also more common in people over 60 years of age than it is in younger people.
The treatment for bullous pemphigoid include:
i. Topical Corticosteroids
ii. Systemic corticosteroids
2. Glucocorticoid-sparing drugs
i. Immunosuppressive drugs
ii. Anti-inflammatory drugs
3. Biologic therapy
i. Intravenous immunoglobulin
For more information on this topic please click on the links below