Cicatricial pemphigoid
Encyclopedia
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.
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)."
, 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.
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)."
Presentation
In mucous membrane pemphigoid, the autoimmune reaction occurs in the skin, specifically at the level of the basement membraneBasement membrane
The basement membrane is a thin sheet of fibers that underlies the epithelium, which lines the cavities and surfaces of organs including skin, or the endothelium, which lines the interior surface of blood vessels.- Composition :...
, 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
Epithelium
Epithelium is one of the four basic types of animal tissue, along with connective tissue, muscle tissue and nervous tissue. Epithelial tissues line the cavities and surfaces of structures throughout the body, and also form many glands. Functions of epithelial cells include secretion, selective...
"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
Gingiva
The gingiva , or gums, consists of the mucosal tissue that lies over the mandible and maxilla inside the mouth.-General description:...
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.
Diagnosis
Diagnostic techniques:- antibodiesAntibodyAn antibody, also known as an immunoglobulin, is a large Y-shaped protein used by the immune system to identify and neutralize foreign objects such as bacteria and viruses. The antibody recognizes a unique part of the foreign target, termed an antigen...
(IgG) precipitates complement (C3) in the lamina lucidaLamina lucidaThe lamina lucida is a component of the basement membrane which is found between the epithelium and underlying connective tissue...
of the basement membrane. - Circulating auto-antibodies to BP-1 antigen (located in hemidesmosomeHemidesmosomeHemidesmosomes are very small stud- or rivet-like structures on the inner basal surface of keratinocytes in the epidermis of skin. They are similar in form to desmosomes when visualized by electron microscopy. While desmosomes link two cells together, hemidesmosomes attach one cell to the...
). 50% have BP-2. - Positive Nikolsky sign.
- IgG, C3 deposition at BM creating smooth line in immunofluorescent analysis.
See also
- Bullous pemphigoidBullous pemphigoidBullous pemphigoid, also referred to as BP, is an acute or chronic autoimmune skin disease, involving the formation of blisters, more appropriately known as bullae, at the space between the skin layers epidermis and dermis.-Signs and symptoms:...
- Gestational pemphigoidGestational pemphigoidGestational Pemphigoid or Pemphigoid Gestationis is a dermatosis of pregnancy, being an autoimmune blistering skin disease that occurs during pregnancy, typically in the second or third trimester, and/or immediately following pregnancy...
- List of cutaneous conditions