dermathosis with autoimmune component. pemphigus pemphigus is a rare group of blistering autoimmune...
Post on 14-Jan-2016
Embed Size (px)
Dermathosis with autoimmune component
Pemphigus is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes.
There are three types of pemphigus which vary in severity: pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus.
The most common form of the disorder is pemphigus vulgaris (PV - ICD-10 L10.0). It occurs when antibodies attack Desmoglein 3. Sores often originate in the mouth, making eating difficult and uncomfortable. Although pemphigus vulgaris may occur at any age, it is most common among people between the ages of 40 and 60. It is more frequent among Ashkenazi Jews. Rarely, it is associated with myasthenia gravis. Nail disease may be the only finding and has prognostic value in management.
Pemphigus foliaceusPemphigus foliaceus (PF) is the least severe of the three varieties. Desmoglein 1, the protein that is destroyed by the autoantibody, is only found in the top dry layer of the skin. PF is characterized by crusty sores that often begin on the scalp, and may move to the chest, back, and face. Mouth sores do not occur. It is not as painful as pemphigus vulgaris, and is often mis-diagnosed as dermatitis or eczema
Paraneoplastic pemphigusThe least common and most severe type of pemphigus is paraneoplastic pemphigus (PNP). This disorder is a complication of cancer, usually lymphoma and Castleman's disease. It may precede the diagnosis of the tumor. Painful sores appear on the mouth, lips, and the esophagus. In this variety of pemphigus, the disease process often involves the lungs, causing bronchiolitis obliterans (constrictive bronchiolitis). Complete removal and/or cure of the tumor may improve the skin disease, but lung damage is generally irreversible .
Pemphigus is a group of autoimmune blistering diseases that may be classified into the following types
Pemphigus vulgaris, of which there several forms: Pemphigus vegetans Pemphigus vegetans of Hallopeau Pemphigus vegetans of Neumann Pemphigus foliaceus, of which there several forms: Pemphigus erythematosus Endemic pemphigus Paraneoplastic pemphigus IgA pemphigus, of which there several forms: Subcorneal pustular dermatosis Intraepidermal neutrophilic IgA dermatosis
Pemphigus is recognized by a dermatologist from the appearance and distribution of the skin lesions. It is also commonly diagnosed by periodontists, oral and maxillofacial surgeons (specialists qualified in both medicine and dentistry)and ophthalmologists (eye doctors) as lesions can affect the eyes and mucous membrane of the oral cavity.
Definitive diagnosis requires examination of a skin or mucous membrane biopsy by a dermatopathologist or oral pathologist. The skin biopsy is taken from the edge of a blister, prepared for histopathology and examined with a microscope. The pathologist looks for an intraepidermal vesicle caused by the breaking apart of epidermal cells (acantholysis). Thus, the superficial (upper) portion of the epidermis sloughs off, leaving the bottom layer of cells on the "floor" of the blister. This bottom layer of cells is said to have a "tombstone appearance".
TreatmentIf not treated, pemphigus can be fatal due to overwhelming infection of the sores. The most common treatment is the administration of oral steroids, especially prednisone, and often in high doses. The side effects of cortico-steroids may require the use of so-called steroid-sparing or adjuvant drugs. The immuno-suppressant CellCept (Mycophenolic acid) is among those being used
Mucosal lichen planus
Mucosal lichen planus, or oral lichen planus (OLP), is an inflammatory auto-immune disease that affects oral mucosa, with or without the involvement of the skin and other mucous membranes.
EpidemiologyOLP affects women more than men (at a ratio of 3:2), and occurs most often in middle-aged adults. OLP in children is rare.
Lichen planus affecting the lower lip.
The cause of lichen planus is not known. It is not contagious and does not involve any known pathogen. OLP has been reported as a complication of chronic hepatitis C virus infection and can be a sign of chronic graft-versus-host disease of the mucous membrane (and skin). It has been suggested that OLP may respond to stress, where lesions may present on the mucosa (or skin) during times of stress in those with the disease
The reticular form is the most common presentation and manifests as white lacy streaks on the mucosa (known as Wickham's striae) or as smaller papules (small raised area). The lesions tend to be bilateral and are asymptomatic. The lacy streaks may also be seen on other parts of the mouth, including the gingiva (gums), the tongue, palate and lips. The bullous form presents as fluid-filled vesicles which project from the surface. The erosive form presents with erythematous (red) areas that are ulcerated and uncomfortable. The erosion of the thin epithelium may occur in multiple areas of the mouth, or in one area, such as the gums, where they resemble desquamative gingivitis. Wickham's striae may also be seen near these ulcerated areas. This form may undergo malignant transformation.
The microscopic appearance of lichen planus is pathognomonic for the conditionHyperparakeratosis with thickening of the granular cell layer Development of a "saw-tooth" appearance of the rete pegs Degeneration of the basal cell layer Infiltration of inflammatory cells into the subepithelial layer of connective tissue
Micrograph of lichen planus. H&E stain
The clinical presentation of OLP may also resemble other conditions, including:Lichenoid drug reaction. This entity is identical to OLP both clinically and histologically. However, lichenoid lesions may be single (in comparison to the usual bilateral appearance of OLP) with proximity to amalgam (metal alloys) dental restoration. Other oral vesiculo-ulcerative conditions such as Pemphigus vulgaris and Benign mucous membrane pemphigoid Discoid lupus erythematosus Chronic ulcerative stomatitis Frictional keratosis and Morsicatio buccarum (chronic cheek biting) Oral leukoplakia Chronic graft-versus-host-disease may manifest as lichenoid reaction. This type of lichenoid lesions have a higher risk of malignant transformation to oral squamous cell carcinoma in comparison to the classical oral lichen planus. Graft-versus-host-disease-associated oral cancer may have more aggressive behavior with poorer prognosis, when compared to oral cancer in non-hematopoietic stem cell transplantation patients.
Medicines used to treat lichen planus include:Oral and topical steroids. Oral retinoids immunosuppressant medications hydroxychloroquine tacrolimus dapsone Aloe vera Pusley Portulaca oleracea
Lichen planus is a chronic mucocutaneous disease that affects the skin, tongue, and oral mucosa. The disease presents itself in the form of papules, lesions, or rashes. Lichen planus does not involve lichens, the fungus/algae symbionts that often grow on tree trunks; the name refers to the dry and undulating, "lichen-like" appearance of affected skin. It is sometimes associated with certain medications and diseases, but is basically of unknown cause
Configuration Annular lichen planus Linear lichen planus Morphology of lesion Hypertrophic lichen planus Atrophic lichen planus Vesiculobullous lichen planus Ulcerative lichen planus Follicular lichen planus Actinic lichen planus Lichen planus pigmentosus
Site of involvement Lichen planus of the palms and soles (Palmoplantar lichen planus) Mucosal lichen planus Lichen planus of the nails Lichen planus of the scalp ( leading to cicatricial alopecia) Inverse lichen planus Special forms Drug-induced lichen planus Lupus erythematosus-lichen planus overlap syndrome Lichen planus pemphigoides Keratosis lichenoides chronica Lichenoid reaction of graft-versus-host disease Lichenoid keratosis Lichenoid dermatitis
Signs and symptoms
The typical rash of lichen planus is well-described by the "5 Ps": well-defined pruritic, planar, purple, polygonal papules. The commonly affected sites are near the wrist and the ankle. The rash tends to heal with prominent blue-black or brownish discoloration that persists for a long time. Besides the typical lesions, many morphological varieties of the rash may occur. The presence of cutaneous lesions is not constant and may wax and wane over time. Oral lesions tend to last far longer than cutaneous lichen planus lesions.
Oral lichen planus (OLP) may present in one of three forms. The reticular form is the most common presentation and manifests as white lacy streaks on the mucosa (known as Wickham's striae) or as smaller papules (small raised area). The lesions tend to be bilateral and are asymptomatic. The lacy streaks may also be seen on other parts of the mouth, including the gingiva (gums), the tongue, palate and lips. The bullous form presents as fluid-filled vesicles which project from the surface.
Thirth formThe erosive forms (Atrophic LP & Ulcerative LP) present with erythematous (red) areas that are ulcerated and uncomfortable. The erosion of the thin epithelium may occur in multiple areas of the mouth (more prominent on the posterior buccal mucosa), or in one area, such as the gums, where they re