Immunofluorescent: immunoglobulin G (IgG)

Immunofluorescent studies: immunoglobulin G (IgG) deposited in the intracellular space around stratum cells.

Clinical Presentation

The bullae of pemphigus vulgaris arise from normal-appearing skin, there is essentially no surrounding inflammation. The blisters are also extraordinarily fragile. Consequently, intact bullae are found only during the prototypal day or two of their existence. Thereafter, the blister roof is broken, leaving a bright-red or crusted shallow erosion that requires weeks or months to heal. The initial lesions are usually found on the upper trunk and back, but since newborn lesions amend faster than older ones heal, there is sloping extension elsewhere with special predilection for the face, groin, and axillae. The prominence of these crusted erosions ofttimes suggests eczematous disease and obscures the fact that the patient has, in fact, a bullous condition.

Oral secretion membrane lesions are practically always present, and they ofttimes precede the appearance of the cutaneous lesions by weeks to months. These oral lesions begin as blisters, but they, too, quickly fortuity down to modify shallow erosions. These erosions are such larger in diameter than the ones found in patients with oral herpes simplex incident and oral aphthae. Characteristically, the posterior mouth is involved. The accompanying discomfort interferes with eating, and the level malnutrition contributes to the extreme debilitation that develops in untreated patients.

A suspected clinical diagnosis staleness be confirmed by biopsy. Light microscopy reveals a characteristic suprabasilar intraepidermal cyst with loss of stratum cell cohesion (acantholysis). Direct immunonuorescent studies carried out on perilesional skin demonstrate a pathognomonic pattern of IgG accumulation in a network-like pattern surrounding the stratum cells. Complement components are sometimes present. More than 90% of patients module also hit limited circulating autoantibodies. These antibodies crapper be demonstrated on backhanded immunofluorescent study. The titer of these antibodies roughly corresponds to the severity of the disease. Thus, reduction in the antibody titer crapper be used as one communication of response to therapy.

Atypical Presentations

Pemphigus joliaceus is a modify of pemphigus in which the intraepidermal clefting occurs high in the epidermis rather than just above the basal layer. Patients with pemphigus foliaceus amend erosions that are more ostensible than those found in pemphigus vulgaris. Oral status is less ofttimes present, and patients do not become as debilitated. Some patients with pemphigus foliaceus hit a considerable degree of facial erythema and may also hit a variety of lupus-like autoantibodies. The combination of these findings is known as pemphigus erythematosus (Senear-Usher syndrome). A modify of pemphigus found in Brasil (fogo selvagem) has epidemiologic features that suggest an communicable etiology. Paraneoplastic pemphigus is a recently described modify of pemphigus that occurs concomitantly with lymphoma or other types of malignancy. The histology shows acantholysis similar to that of pemphigus vulgaris, but the clinical features, because of evenhandedly marked erythema around the blisters, simulate erythema multiforme bullosum.

Course and Prognosis

Pemphigus begins most commonly in mid to New grown life. It is a chronic, seriously debilitating disease that, if left untreated, inevitably leads to death. With vigorous, primeval treatment the mortality evaluate is approximately 10%. Other autoimmune diseases are found with unexpected frequency in patients with pemphigus, and a rather small, but belike significant, number of patients hit thymomas. Pathogenesis

Pemphigus is an autoimmune disease in which limited IgG antibodies and, sometimes, hands components are deposited at the precise place of stratum cell damage; these same antibodies are regularly found in the circulation. Moreover, the antibodies, when isolated and injected into a suitable substrate, cause an stratum lesion identical with that found in the original disease. The acantholysis caused by these antibodies appears to amend as a termination of the release of proteolytic enzymes. This impact does not appear to require, though it may be optimized by, the activation of complement. The 85- and 130-kD antigens responsible for this autoimmune reaction are one or another of individual proteins that attain up adherence junctions, such as the desmosomes, responsible for the adherence of adjacent stratum cells. Genetic factors as echolike by the presence of destined HLA antigens and a high incidence in destined Jewish populations are belike also important. Perhaps most engrossing of all is the observation that whatever medications, most notably penicillamine and captopril, crapper rush in destined individuals a disease indistinguishable from idiopathic pemphigus.

Therapy

The landmark studies of Lever 35 years ago proved that pemphigus need not inevitably advance to death. He showed that very high doses of orally administered steroids (prednisone 120-240 mg/day) would almost always alter the disease low control. Unfortunately, the required long-term, high-dose administration of steroids causes its possess morbidity and mortality. In fact, the mortality associated with pemphigus today is mostly due to drug toxicity rather than to the disease itself. There are individual ways in which this potential toxicity crapper be minimized. In whatever patients, once initial response is obtained, it is possible to convert their steroids to an move day schedule and for others the steroid dose crapper be greatly reduced through the addition of steroid-sparing immunosuppressive agents such as methotrexate, azathioprine, or cyclophosphamide. In patients with relatively mild disease it is sometimes possible to obtain remission through long-term administration of gold salts. Other therapies sometimes used include pulsed IV methylprednisolone, plasmaphoresis, and oral cyclosporine.