Definition
Bullous pemphigoid is an inflammatory disease of the skin that leads to chronic bullous eruption.
The etiology of this dermatosis is autoimmune. In particular, bullous pemphigoid is characterized by the presence of autoantibodies directed towards antigens (represented by two structural proteins, called BPAg1 and BPAg2) present in the basement membrane of the epidermis. This interaction causes the latter to detach from the underlying dermis and the subsequent formation of bullous lesions.
Bullous pemphigoid predominantly affects older people, but has also been described in children.
Most common symptoms and signs *
- Oral aphthosis
- bubbles
- Burning in the mouth
- Skin discoloration
- Erythema
- Skin erosion
- papules
- plaques
- wheals
- itch
- Leg itch
- Itching in the hands
- Skin Ulcers
- blisters
Further indications
Bullous pemphigoid is characterized by the gradual appearance of tense bubbles of various sizes. These lesions arise on normal-looking skin or on the edge of erythematous plaques. The content of the bubble is clear or serum-blood. The areas most frequently affected by bullous pemphigoid are the trunk and the extremities (especially the flexor surfaces of the limbs).
See More Photos Pemphigoid Bolloso
The skin lesions are accompanied by itching, which can present various degrees of intensity (in most cases it is mild to moderate).
The Nikolsky sign is negative, therefore exerting a little lateral pressure on the healthy perilesional skin does not cause the epidermal detachment (this maneuver allows to confirm the presence of the acantholysis that is found, for example, in pemphigus vulgaris).
During the course of the disease, the rupture of the bubble's roof causes superficial erosions from the sharp edges that evolve into brown-blackish crusts. These lesions undergo progressive re-epithelialization, therefore they generally heal without the formation of scars or atrophy.
In bullous pemphigoid, the appearance of tense bubbles may be preceded by a prodromal phase, characterized by the simultaneous presence of many different erythematous-edematous lesions (erythema, pomfi, papula, vesicles and inflammatory plaques), similar to eczematous or urticarial reactions.
In about 1/3 of the patients, there is the concomitant presence of lesions of the mucous membranes.
In most cases, bullous pemphigoid presents with desquamative erosions and / or gingivitis in the oral cavity; however the lesions can potentially manifest also at the level of the pharynx, ocular conjunctiva, nose and genitals.
Bullous pemphigoid has a chronic-relapsing course and a relatively benign prognosis; the exacerbations of the disease are generally less severe than the initial episode and interspersed with long clinical remissions.
The diagnosis is based on the skin biopsy and on the search for tissue and circulating autoantibodies directed against specific antigens of the dermoepidermal junction (direct and indirect immunofluorescence), although the clinical presentation is usually characteristic. Bullous pemphigoid must be distinguished from pemphigus vulgaris.
The standard treatment is based on the commitment of corticosteroids in systemic form in cases with diffuse involvement. For localized forms, with few bubbles and / or slow evolution, topical cortisone therapy may be indicated. Sometimes, it is also necessary to resort to a systemic immunosuppressive therapy to control the disease.
