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Pemphigus

Pemphigus is an autoimmune disorder that causes blistering and raw sores on skin and mucous membranes. As with other autoimmune disorders, it is caused when the body's defenses mistake its own tissues as foreign, and attack the cells. more...

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Medicines

Types

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 (ICD-10 L10.0). It occurs when antibodies attack Desmoglein 3, a protein that keeps cells bound together. Thus, cells simply fall apart, causing skin to slough off. Although pemphigus vulgaris may occur at any age, it is quite rare in children, and most common in the middle aged and elderly. Sores often originate in the mouth, making eating difficult and uncomfortable.
  • Pemphigus foliaceus (ICD-10 L10.2) is the least severe of the three varieties. Desmoglein 1, the protein that is destroyed by the body's immune system is only found in the top dry layer of the skin, so mouth sores do not occur. Pemphigus foliaceus is characterized by crusty sores that often begin on the scalp, and may move to the chest, back, and face. It is not as painful as pemphigus vulgaris, and is often mis-diagnosed as dermatitis or eczema.
  • The least common and most severe type of pemphigus is the neoplastic variety. This disorder is usually found in conjunction with an already-existing malignancy. Very painful sores appear on the mouth, lips, and the esophagus. A diagnosis of neoplastic pemphigus may prompt a search for an existing tumor. Sometimes, the tumor is not malignant. In these cases, tumor removal may lead to a remission of the pemphigus.

Treatment

If not treated, pemphigus is usually fatal, due to overwhelming systemic infection. The most common treatment is the administration of oral steroids, especially prednisone. Mild cases sometimes respond to the application of topical steroids. 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 skin lesions do become infected, antibiotics may be used for treatment. In addition, talcum powder is helpful to prevent oozing sores from adhering to bedsheets and clothes.

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Pemphigus Vulgaris: A Medical Dictionary, Bibliography, And Annotated Research Guide To Internet References $39.41 Anti-CD20 monoclonal antibody (rituximab) in the treatment of pemphigus.(WASHINGTON WHISPERS) : An article from: Journal of Drugs in Dermatology $5.95
Pemphigus: Update for the general practitioner : An article from: New York State Dental Journal $10.00 Treatment of pemphigus. : An article from: Dermatology Nursing $5.95
Extrusion of sebaceous gland into a blister of pemphigus vulgaris: An unusual processing artifact.(Letter To Editor)(Letter to the Editor) : An article ... of Dermatology, Venereology and Leprology $5.95 Dexamethasone cyclophosphamide pulse therapy for pemphigus.(Letters to Editor)(Letter to the Editor) : An article from: Indian Journal of Dermatology, Venereology and Leprology $5.95
Practice pearl: gargling with cholinergic ophthalmic drops for treating the oral lesions of pemphigus vulgaris.(LETTERS TO THE EDITOR) : An article from: Journal of Drugs in Dermatology $5.95 Pemphigus: A Medical Dictionary, Bibliography, And Annotated Research Guide To Internet References $39.41
Treatment of pemphigus with intravenous pulse cydophosphamide.(Study) : An article from: Indian Journal of Dermatology, Venereology and Leprology $5.95 Savage fire, $25.98

Using rituximab in a patient with paraneoplastic pemphigus - anti-cd20 antibody - Case Reports
Abstract Paraneoplastic pemphigus (PNP) is a rare autoimmune mucocutaneous blistering disease that is commonly associated with underlying B-cell neoplasms.
Phase II study of high-dose cycloposphamide in patients with refractory pemphigus - Brief Article
This study is currently recruiting patients. Sponsored by Johns Hopkins Oncology Center This study is designed to determine the response rate and ...
A case of co-incident phenylketonuria, pemphigus foliaceus, and tinea amiantacea treated with tetracycline and nicotinamide - Case Reports
I report a patient with phenylketonuria who presented with pemphigus foliaceus or tinea amiantacea. The rash resolved with treatment with tetracycline ...
Anti-CD20 monoclonal antibody in the treatment of pemphigus
Anti-CD20 Monoclonal Antibody (Rituximab) in the Treatment of Pemphigus Arin MJ, et al. Br J Dermatol. 2005;153:620-625. Summary The authors present ...
Practice pearl: gargling with cholinergic ophthalmic drops for treating the oral lesions of pemphigus vulgaris
Painful oral lesions are seen in almost all patients with pemphigus vulgaris and are the presenting complaint in 50-70% of patients (1). Mucosal lesions ...
Pemphigus vulgaris acantholysis ameliorated by cholinergic agonists
The authors in this study injected littermates of neonatal athymic nude mice with pemphigus vulgaris (PV) to determine whether a cholinergic agonist ...
Paraneoplastic Pemphigus Associated With Bronchiolitis Obliterans - )
Paraneoplastic pemphigus (PNP) is an autoantibody-mediated mucocutaneous blistering disease associated with underlying neoplasms. Autoantibodies of PNP bind to the plakin family of cytoplasmic prot
Diphenhydramine gargling may slow the healing of oral lesions of pemphigus vulgaris - Letters to the Editor - Letter to the Editor
Oral lesions are seen in almost all patients with pemphigus vulgaris and are the presenting complaint in 50-70% of patients (1). As these lesions are ...

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