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. This particular autoimmune reaction is sometimes associated with the use of Penicillamine. Pemphigus is derived from the Greek pemphix, meaning bubble or blister. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // L00-L99 - Diseases of the skin and subcutaneous tissue (L00-L08) Infections of the skin and subcutaneous tissue (L00) Staphylococcal scalded skin syndrome (L01) Impetigo (L02) Cutaneous abscess, furuncle and carbuncle (L03) Cellulitis (L04) Acute lymphadenitis (L05) Pilonidal cyst (L08) Other local infections of skin and subcutaneous tissue (L081) Erythrasma... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ... Beyond overall skin structure, refer below to: See-also. ... The mucous membranes (or mucosae; singular: mucosa) are linings of mostly endodermal origin, covered in epithelium, and are involved in absorption and secretion. ... Drawing of the structure of cork as it appeared under the microscope to Robert Hooke from Micrographia which is the origin of the word cell being used to describe the smallest unit of a living organism Cells in culture, stained for keratin (red) and DNA (green) The cell is the...

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 vulgaris often affects people between the ages of 40-60, and mainly of Jewish [1], Middle Eastern, or Mediterranean descent. Some patients are associated with myasthenia gravis, but it's unusual.

• Foliaceus 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, also known as paraneoplastic pemphigus. This disorder is usually found in conjunction with an already-existing malignancy. Very painful sores appear on the mouth, lips, and the esophagus. In this variety of pemphigus, the disease process often involves Bronchiolitis obliterans, a fatal destruction of alveoli in lung tissue. 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. However, any decline in pulmonary function is generally irreversible. With Intravenous Immunoglobulin (IVIG) treatment and aggressive chemotherapy for the underlying lympoma, there are few known survivors of Paraneoplastic Pemphigus with pulmonary involvement (contact information in External Links below).

The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... Each antibody binds to a specific antigen; an interaction similar to a lock and key. ... The desmogleins are a family of cadherins consisting of proteins DSG1, DSG2, DSG3, and DSG4. ... male human mouth The mouth, also known as the buccal cavity or the oral cavity, is the orifice through which an organism takes in food and water. ... For other uses, see Jew (disambiguation). ... Myasthenia gravis (sometimes abbreviated MG; from the Greek myastheneia, lit. ... The desmogleins are a family of cadherins consisting of proteins DSG1, DSG2, DSG3, and DSG4. ... The scalp is the anatomical area bordered by the face anteriorly and the neck to the sides and posteriorly. ... Dermatitis is a blanket term literally meaning inflammation of the skin. It is usually used to refer to eczema, which is also known as Dermatitis eczema. ... For the beetle, see Exema. ... A paraneoplastic phenomenon is a disease or symptom that is the consequence of the presence of cancer in the body, but is not due to the local presence of cancer cells. ... Cancer is a class of diseases or disorders characterized by uncontrolled division of cells and the ability of these to spread, either by direct growth into adjacent tissue through invasion, or by implantation into distant sites by metastasis (where cancer cells are transported through the bloodstream or lymphatic system). ... The esophagus (also spelled oesophagus/Å“sophagus, Greek ), or gullet is an organ in vertebrates which consists of a muscular tube through which food passes from the pharynx to the stomach. ... Bronchiolitis obliterans is a disease of the lungs in which the bronchioles are plugged with granulation tissue. ... The alveoli (singular:alveolus), tiny hollow sacs which are continuous with the airways, are the sites of gas exchange with the blood. ...

Diagnosis

Diagnosis relies on microscopic examination of skin lesion samples. A biopsy of a suspected lesion is taken: a sample of the blistered skin is removed and examined under the microscope to determine if the cells are separated in the manner characteristic of pemphigus. Unlike in the related pemphigoid, pemphigus manifests as intra-epithelial clefting, meaning the spinous cells of the epithelium break apart, a phenomenon known as acantholysis. This is because the desmosomes are attacked. In pemphigoid, the epitheium remains intact, but is entirely "unzipped" from the underlying connective tissue bed, or lamina propria, because the hemidesmosomes are attacked. Also apparent in pemphigus is a "tombstone appearance" of the basal cell layer and Tzanck cells. Robert Hookes microscope (1665) - an engineered device used to study living systems. ... Cell adhesion in desmosomes A desmosome is a cell structure specialized for cell-to-cell adhesion. ... The lamina propria is a thin vascular layer of connective tissue beneath the epithelium of an organ. ... The basal cell layer is also called the Stratum Germinativum or Stratum Basale and is the innermost layer of the epidermis. ...

Direct immunoflourescence on the biopsy skin sample can be used to detect desmoglein antibodies in the skin. The presence of these antibodies indicates pemphigus. Indirect immunofluorescence and ELISA can measure desmoglein antibodies in blood serum. A Fluorescence Microscope is a light microscope used to study properties of organic or inorganic substances using the phenomena of fluorescence and phosphorescence instead of, or in addition to, reflection and absorption. ... Elisa (born Elisa Toffoli on 19 December 1977) is an Italian singer and solo artist, writing and performing within several genres, notably rock, blues, soul and ambient. ...

Treatment

If not treated, pemphigus is fatal, due to overwhelming systemic infection. The most common treatment is the administration of oral steroids, especially prednisone. Recently, there has been great promise of surviving some forms of pemphigus (especially PNP) by using a pooled blood product known as gamma globulin or IVIG. Mild cases sometimes respond to the application of topical steroids. In 2007, a clinical trial including a one-weak treatment with Rituximab, a monoclonal chimaeric Anti-CD20-Antibody, approved by the FDA for the treatment of B-Cell-Non-Hodgkin-Lymphoma and severe cases of rheumatoid arthritis, showed a remission in 18 of 21 otherwise untreatable, severe cases of Pemphigus vulgaris.^[1] An infection is the detrimental colonization of a host organism by a foreign species. ... This article or section does not adequately cite its references or sources. ... Rituximab, sold under the trade names Rituxan® and MabThera®, is a monoclonal antibody used in the treatment of B cell non-Hodgkins lymphoma, B cell leukemia, and some autoimmune disorders. ... Non-Hodgkin lymphoma is a cancer arising from lymphocytes, a type of white blood cells. ... Rheumatoid arthritis (RA) is traditionally considered a chronic, inflammatory autoimmune disorder that causes the immune system to attack the joints. ...

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. Some drugs used include solumedrol, ciclosporin, azathioprine, and in rare instances, extremely controlled use of thalidomide in eligible patients. Immune phoresis procedures are also a possible treatment. Bronchiolitis obliterans is a disease of the lungs in which the bronchioles are plugged with granulation tissue. ... Methylprednisolone (molecular weight 374. ... Ciclosporin (INN), cyclosporine or cyclosporin (former BAN), is an immunosuppressant drug. ... Azathioprine is a chemotherapy drug, now rarely used for chemotherapy but more for immunosuppression in organ transplantation, autoimmune disease such as rheumatoid arthritis or inflammatory bowel disease such as Crohns disease. ... // Thalidomide is a sedative, hypnotic, and anti-inflammatory medication. ...

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.^{[citation needed]} Staphylococcus aureus - Antibiotics test plate. ... Talc block Talc is a mineral composed of hydrated magnesium silicate with the chemical formula H2Mg3(SiO3)4 or Mg3Si4O10(OH)2. ...

Pemphigus in domestic animals

Pemphigus foliaceus skin eruption on the ventral abdomen of a dog

Pemphigus foliaceus has been recognized in pet dogs, cats and horses and is the most common autoimmune skin disease diagnosed in veterinary medicine. Pemphigus foliaceus in animals produces clusters of small vesicles that quickly evolve into pustules. Pustules may rupture, forming erosions or become crusted. Left untreated, pemphigus foliaceus in animals is life-threatening leading to loss of condition and secondary infection. Image File history File linksMetadata Download high-resolution version (1024x768, 340 KB) File links The following pages on the English Wikipedia link to this file (pages on other projects are not listed): Pemphigus Metadata This file contains additional information, probably added from the digital camera or scanner used to create... Image File history File linksMetadata Download high-resolution version (1024x768, 340 KB) File links The following pages on the English Wikipedia link to this file (pages on other projects are not listed): Pemphigus Metadata This file contains additional information, probably added from the digital camera or scanner used to create...

Pemphigus vulgaris is a very rare disorder described in pet dogs and cats. Paraneoplastic pemphigus has been identified in pet dogs.

References

1. ^ Joly P, Mouquet H, Roujeau JC, D'Incan M, Gilbert D, Jacquot S, Gougeon ML, Bedane C, Muller R, Dreno B, Doutre MS, Delaporte E, Pauwels C, Franck N, Caux F, Picard C, Tancrede-Bohin E, Bernard P, Tron F, Hertl M, Musette P. A single cycle of rituximab for the treatment of severe pemphigus. N Engl J Med. 2007 Aug 9;357(6):545-52. PMID 17687130

External links

• International Pemphigus Foundation
• Pemphigus Vulgaris
• Ahmed AR, Spigelman Z, Cavacini LA, Posner MR (2006). "Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin". N. Engl. J. Med. 355 (17): 1772-9. DOI:10.1056/NEJMoa062930. PMID 17065638. 
• www.blisters.org.au
• DermAtlas 65817101
• http://tray.dermatology.uiowa.edu/PemVul01.htm