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Pemphigus

Mary Ann S. Tacula

Pemphigus

is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes. In pemphigus, autoantibodies form against desmoglein. Desmoglein forms the "glue" that attaches adjacent epidermal cells via attachment points called desmosomes. When autoantibodies attack desmogleins, the cells become separated from each other and the epidermis becomes "unglued", a phenomenon called acantholysis. This causes blisters that slough off and turn into sores. In some cases, these blisters can cover a significant area of the skin.

Originally, the cause of this disease was unknown, and "pemphigus" was used to refer to any blistering disease of the skin and mucosa.

CLINICAL MANIFESTATIONS
Most patient present with oral lesions appearing as irregularly shaped erosions that are painful, bleed easily, and heal slowly. The skin bullae enlarge, rupture, and leave large, painful eroded areas that aare accompanied by crusting and oozing. A characteristic offensive odor enamates from the bullae and the exuding serum.

There is blistering or sloughing of uninvolved skin when minimal pressure is applied(nikolskys sign). The eroded skin heals slowly, and huge areas of the body eventually are involved.

Bacterial super infection is common.

Types
There are three types of pemphigus which vary in severity: pemphigus vulgaris pemphigus foliaceus paraneoplastic pemphigus

Pemphigus vulgaris (PV - ICD-10 L10.0).

the most common form of the disorder. 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 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 pemphigus (PNP).

the least common and most severe type of pemphigus . 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 .

Diagnosis

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. Intraorally it resembles the more common diseases lichen planus(a chronic mucocutaneous disease that affects the skin, tongue, and oral mucosa) and mucous membrane pemphigoid(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 ).

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".

Definitive diagnosis also requires the demonstration of anti-desmoglein autoantibodies by direct immunofluorescence on the skin biopsy. These antibodies appear as IgG deposits along the desmosomes between epidermal cells, a pattern reminiscent of chicken wire. Anti-desmoglein antibodies can also be detected in a blood sample using the ELISA technique.

Half of pemphigus patients have oral lesions alone during the first year but develop skin lesions later.

Treatment
If not treated, pemphigus can be fatal from an 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 steroidsparing or adjuvant drugs. The immunosuppressant CellCept (Mycophenolic acid) is among those being used.

Intravenous gamma globulin (IVIG) may be useful in severe cases, especially paraneoplastic pemphigus. Mild cases sometimes respond to the application of topical steroids. Recently, Rituximab, an anti-CD20 antibody, was found to improve otherwise untreatable severe cases of Pemphigus vulgaris. All of these drugs may cause severe side effects, so the patient should be closely monitored by doctors. Once the outbreaks are under control, dosage is often reduced, to lessen side effects.

If paraneoplastic pemphigus is diagnosed with pulmonary disease, a powerful cocktail of immune suppressant drugs is sometimes used in an attempt to halt the rapid progression of bronchiolitis obliterans, including methylprednisolone, ciclosporin, azathioprine and thalidomide. Plasmapheresis may also be useful. If skin lesions do become infected, antibiotic may be prescribed. Tetracycline antibiotics have a mildly beneficial effect on the disease, and are sometimes enough for Pemphigus Foliaceus. In addition, talcum powder is helpful to prevent oozing sores from adhering to bedsheets and clothes.