Pemphigus is a group of chronic bullous diseases.
The characteristic symptom of pemphigus is blisters on the skin and mucous membrane. These blisters lead to ulcers creating raw open areas on the skin which increases chances of infection. Depending upon the type of pemphigus, symptoms  varies:
Blisters and ulcers occur in a number of conditions thus diagnosis is difficult to reach in case of pemphigus. A skin specialist will be required to take a complete medical history and for examination of skin and mucosa . Clinically, on examination one will see typical blisters and ulcers on the skin and oral mucosa, apart from that rubbing a patch of normal skin near the affected area with a cotton swab will result in peeling of top layers of skin. This is known as Nikolsky sign and is usually positive in case of pemphigus.
Skin biopsy can be done and will reveal the typical acantholytic cells embedded within the blisters just in the superficial layers of the epidermis. The internal structure of all blisters shows intracellular edema and separation of epidermal cells to produce spaces and clefts. Histopathology helps to differentiate the various types of pemphigus. Certain cases may show eosinophilic spongiosis prior to blistering.
Blood tests can be done to detect the antibodies in circulation, desmoglein antibodies. Patients show elevated levels of these antibodies when pemphigus is initially diagnosed. This blood test is the indirect immunofluorescence test. Levels of antibodies fluctuate as symptoms improve and patient becomes better. Skin biopsy staining by direct immunofluorescence reveals antibodies and is a confirmatory test for pemphigus. Various types of pemphigus are distinguished based on the level of epidermis involved and the type of antibodies circulating in the body.
The faster the treatment is started, the lesser are the chances of infections and complications. The approach towards pemphigus is mainly medications and in extreme cases, hospitalization . The aim of initiating medications soon is to prevent further blister formation and enable healthy healing of previously existing lesions. In medications, corticosteroids are mainly used to control and suppress the immune system. The main steroid used is prednisone which should be used with caution and care. Prolonged usage of this steroid can have serious side effects such as increase in sugar, weight gain, bone damage and higher risk of infections. Immunosuppressants such as azathioprine , methotrexate can also be given to suppress immune system from damaging own body. For concurrent infections antibiotics, antivirals or antifungals can be given.
Widespread pemphigus vulgaris requires hospital admission as the sores and blisters are so severe that a high risk of superadded infection is possible. Severe sepsis can spread to the entire body and lead to death. Along with medications, fluid balance is maintained through IV fluids along with intravenous feeding since eating is painful due to sores and ulcers. Skin and wound care is vital for healthy healing and preventing secondary infection. Plasmapheresis is also done to reduce the levels of antibodies in the blood circulation.
With the above treatment outlined, patients have shown good results and a decrease in most of the symptoms. Though chances of relapse is quite common due to which certain patient need to continue medications for a longer period .
With the advent of systemic steroid therapy, the outlook of this life threatening condition has improved. If treatment is not given, pemphigus is fatal and complications common. Due to corticosteroids, the mortality rate  has decreased considerably. The most common complications which lead to death are infection, in case of pemphigus vulgaris they spread all over the body especially in the elderly patients. In these cases, high doses of steroids need to be given to suppress the immune system.
Hospitalization may be required in cases of severe blistering, special care has to be taken of the skin to prevent widespread secondary infection. Antibiotics may be prescribed for these infections along with dressing of open skin areas. Prognosis mainly depends upon the extent of spread of the disease and is worse in pemphigus vulgaris. Healing in most cases is without scarring unless secondary infection supervenes which can also lead to sepsis.
Pemphigus is a rare autoimmune disorder. In pemphigus, the immune system produces antibodies against desmoglein, which is a protein which glues the cells of the epidermis together as a result the building blocks of the epidermis called keratinocytes that get damaged and fall apart or become flaccid. This leads to collection of fluid between the dermal layers, resulting in erosions and blisters of the skin.
The exact reason as to why the immune system behaves like this is still not known. Thus, the exact triggering factor of the immune system that leads to production of autoantibodies remains unclear. Recent investigations and research suggest a combination of genetic  and environmental factors in the etiology of this autoimmune disease. Certain medications can cause pemphigus to occur such as cephalosporins, penicillin and others, but it usually resolves once medication stopped.
Various types of pemphigus:
This is a rare disorder with an overall prevalence  of approximately 4.2 cases reported per million every year in the US. Overall, in the world one to ten new cases are reported per one million people. The data regarding the epidemiology of this condition is very limited and scarce. The incidence and prevalence rates are different across various parts of the globe. It affects people of all castes and races, but people of Mediterranean, Jewish, Middle East and Indian descent seem to have a higher risk which could be due to some genetic reason.
Pemphigus affects both sexes equally and usually occurs around 50-60 years of age; though nowadays, certain pediatric cases have been noted. The symptom presentation of pediatric cases is similar, but not common. No socioeconomic factors are involved. This disease is said to have a genetic predisposition and this trait seems to run in certain types of families. There are certain genes in the MHC complex on chromosome 6 which seem to be seen in people with this condition. This is a not an infectious disease and cannot be transmitted from person to person.
Acantholysis is the primary pathological feature of pemphigus. The separation of the keratinocytes in the epidermis leads to cleft formation which becomes bigger to form blisters and later merge to form ulcers. Histopathology  reports of pemphigus vulgaris always show separation of epidermal keratinocytes leading to an intraepithelial vesicle formation.
The various types of pemphigus are always accompanied by circulating autoantibodies which are basically intercellular antibodies against the antigen proteins on the keratinocytes cell surfaces. The immunological aspect of this condition is very well understood with the exact role of antibodies. Antibodies against desmoglein protein are widely found in the patients of pemphigus especially desmoglein 3 in pemphigus foliaceus since this protein is abundantly found in superficial a layers of epidermis and desmoglein 1 in vulgaris as this is found in lower layers of epidermis. These autoantibodies can be detected in the circulation by indirect immunofluorescence and recently specific ELISAs are used for detection.
Primary prevention of pemphigus is not possible due to its immunological origin. Being an autoimmune disorder, there is no way to prevent this disease. Secondary prevention to avoid exacerbation of pemphigus should be followed by all patients suffering from this disorder. Certain precautionary steps should be followed such as:
Pemphigus is a group of immunological disorders characterized by formation of recurrent vesicles or blisters which break out on the skin and mucocutaneous areas such as lining of mouth and genital area. Pemphigus is not a common condition and occurs due to lysis of the binding spines of a protein known as desmoglein that holds together the cells of the epidermis. Autoantibodies are formed against this protein which results in the lysis of these cells. Such keratinocytes, called acantholytic cells, are found within the bullae of the patients. This leads to formation of intraepidermal bullae and consequently ulcers and erosions due to their rupture. Young and middle aged adults are affected and extensive oozing erosions may lead to fluid, electrolyte and protein loss. The different types in this group of autoimmune disorders are pemphigus vulgaris , pemphigus foliaceus, paraneoplastic pemphigus and other variants. About 70% individuals are affected by pemphigus vulgaris and each of these variants is characterized histologically by a different layer of epidermis being affected.
Pemphigus vulgaris affects oral mucosa which leads to severe pain and burning while eating or talking. Inability to eat food further aggravates electrolyte imbalance and leads to hypoproteinemia. Untreated and extensive disease leads to debility and death. Pemphigus foliaceus differs from vulgaris by being superficial in nature and sparing the mucosa, though if pemphigus foliaceus is left untreated, it can lead to erythema and even be fatal. Earlier this condition had a high mortality rate, but with the advent of steroids and immunosuppressants, results of this blistering disorder are much better. This rare autoimmune disorder  responds to high doses of systemic steroid therapy with an extended tapering over many months or even years. Pulse therapy with steroids is known to reduce the side effects of these medications.
Pemphigus is a rare group of skin disorders wherein severe blisters and ulcers appear in the skin and mucous membranes of the mouth, nose, throat, eyes and even genital areas. Pemphigus usually has many types with pemphigus vulgaris being the most common variant. The exact cause is not known, but since it is an autoimmune disorder, interplay of genetic and environmental factors has been suggested. In this disorder, the body's own immune system starts producing antibodies against healthy tissue and body cells. The immune system similar to how it fights germs and bacteria, wrongly starts damaging healthy cells and tissues. Certain races and ethnics are more prone to this condition and affects both sexes equally. The median age of onset of this disease is usually 50-60 years.
Clinical symptoms of pemphigus are blisters and ulcers on the healthy skin, which tend to rupture oozing out fluid. There may be severe burning and stinging pain. Due to rupture, secondary infection is also quite common if proper care is not taken. If you observe painful blisters and ulcers on your skin, especially mouth, seek medical help immediately. Timely treatment can prevent complications and reduce duration of disorder. Consult a skin specialist who will do a complete physical as well as advice certain tests including a skin biopsy. After a diagnosis, treatment consists of steroids and steroidal creams. Strict oral care should be followed. Immunosuppressants may be given as another option instead of steroids. Certain minor changes in lifestyle such as avoidance of too spicy food, sunscreen application, oral hygiene as well as refrain from stress and emotional worry. Follow up is essential for a successful treatment.