- Cicatricial pemphigoid
Cicatricial pemphigoid Classification and external resources ICD-10 L12.1 ICD-9 694.6 OMIM 164185 DiseasesDB 30757 MeSH D010390
Cicatricial pemphigoid (also known as "Benign mucosal pemphigoid," "Benign mucous membrane pemphigoid," "Ocular pemphigus," and "Scarring pemphigoid") is a rare chronic autoimmune subepithelial blistering disease characterized by erosive skin lesions of the mucous membranes and skin that results in scarring of at least some sites of involvement.:581
Cicatricial pemphigoid has been referred to by a variety of designations based largely on its site of involvements, with examples of such terminology including "desquamative gingivitis," "ocular pemphigus," and "benign mucous membrane pemphigoid." However, currently "...such designations are thought to be confusing or somewhat misleadings (e.g., pemphigus in this context is a misnomer , and this disorder is hardly benign given the extend of morbidity it can cause)." :581
In mucous membrane pemphigoid, the autoimmune reaction occurs in the skin, specifically at the level of the basement membrane, which connects the lower skin layer (dermis) to the upper skin layer (epidermis) and keeps it attached to the body.
When the condition is active, the basement membrane is dissolved by the antibodies produced, and areas of skin lift away at the base, causing hard blisters which scar if they burst. In other words, this is a desquamating/blistering disease in which the epithelium "unzips" from the underlying connective tissue, allowing fluid to gather that subsequently manifest as bullae, or blisters.
The autoimmune reaction most commonly affects the mouth, causing lesions in the gingiva or gums, but it can also affect areas of mucous membrane elsewhere in the body, such as the sinuses, genitals and anus. When the cornea of the eye is affected, repeated scarring may result in blindness.
Nikolsky's sign (gentle lateral pressure on unaffected mucosa or skin raises a bulla. If no lesions are present on examination it may be useful way of demonstrating reduced epithelial adhesion. In (Pemphigus) the epithelium tends to disintegrate rather than form a bulla. Edward W. Odell.
- antibodies (IgG) precipitates complement (C3) in the lamina lucida of the basement membrane.
- Circulating auto-antibodies to BP-1 antigen (located in hemidesmosome). 50% have BP-2.
- Positive Nikolsky sign.
- IgG, C3 deposition at BM creating smooth line in immunofluorescent analysis.
Diseases of the skin and appendages by morphology GrowthsPigmentedDermal and
PurpuraMacularthrombocytopenic purpura · actinic purpuraPapularIndurated
Vesiculobullous disease (L10–L14, 694) Acantholysis
(epidermis)Pemphigus vulgaris: Pemphigus vegetans (Pemphigus vegetans of Hallopeau, Pemphigus vegetans of Neumann)Pemphigus foliaceus: Pemphigus erythematosus · Endemic pemphigusParaneoplastic pemphigusIgA pemphigus (Subcorneal pustular dermatosis, Intraepidermal neutrophilic IgA dermatosis)Other
Other bullous In diseases
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