Questionwhat is this medical term ... it has something to do with the swelling of the hands.
AnswerJackson,
You must mean: Bullous Pemphigus.
You will find the following interesting (from the Merck manual):
Pemphigus
An uncommon, potentially fatal autoimmune skin disorder characterized by intraepidermal bullae and extensive erosions on apparently healthy skin and mucous membranes.
Pemphigus usually occurs in middle-aged or elderly persons and is rare in children. Foci of high incidence occur in South America, especially Brazil. In active pemphigus, the serum and skin contain readily demonstrable IgG antibodies that bind at the site of epidermal damage. These antibodies can induce the same pathologic process in vivo and in vitro.
Symptoms and Signs
The primary lesions are flaccid bullae of various sizes (see Plate 120-1), but often the skin or mucosae just shear off, leaving painful erosions (see Plate 120-2). Lesions typically occur first in the mouth, where they rupture and remain as chronic, often painful, erosions for variable periods (see Plate 120-3) before the skin is affected. The bullae typically arise from healthy-appearing skin, rupture, and leave a raw area and crusting. Any area of stratified squamous epithelium may be affected, but the extent of skin and mucosal involvement varies (eg, lesions may occur in the oropharynx and upper esophagus). Itching is usually absent.
In some superficial varieties (eg, pemphigus foliaceus), bullae may not be prominent and usually are absent from the mouth. The lesions may be localized to the face, and the large crusty scales may suggest a combination of seborrheic dermatitis and subacute cutaneous lupus erythematosus. Pemphigus foliaceus may resemble exfoliative dermatitis, psoriasis, a drug eruption, or other forms of dermatitis.
Diagnosis
Pemphigus should be suspected in any bullous disorder or chronic mucosal ulceration. It must be differentiated from other chronic oral ulcers and from other bullous dermatoses (eg, bullous pemphigoid, benign mucosal [cicatricial] pemphigoid--see Ch. 96, drug eruptions, toxic epidermal necrolysis, erythema multiforme, dermatitis herpetiformis, bullous contact dermatitis).
In pemphigus vulgaris, the epidermis is easily detached from the underlying skin (Nikolsky's sign), and biopsy usually shows typical suprabasal epidermal cell separation. In pemphigus foliaceus, the separation does not occur in the suprabasal region but rather in the upper layers of the stratum spinosum or stratum granulosum.
A Tzanck test (see Special Diagnostic Methods in Ch. 109) is frequently diagnostic when Wright's or Giemsa stain is used on a smear of cells obtained by scraping the base of a lesion. The acantholytic cells typical of pemphigus are unattached and basal cell-like, with large centrally placed nuclei and condensed cytoplasm.
Direct immunofluorescence tests of perilesional skin or mucous membranes are most reliable and invariably show IgG on the epidermal or epithelial cell surfaces. Indirect immunofluorescence tests usually show pemphigus antibodies in the patient's serum, even when the lesions are localized in the mouth. The antibody titer may correlate with disease severity.
Treatment
Pemphigus is a serious disease with an inconsistent and unpredictable response to therapy, a prolonged course, and virtually inevitable complicating drug side effects. Referral to a dermatologist with expertise in treating this disease is recommended.
The aim of treatment, both immediate and subsequent, is to stop the eruption of new lesions. Specific therapy depends on the extent and severity of disease. The mainstay is systemic corticosteroids. Some patients with few lesions may respond to low-dose oral prednisone (eg, 20 to 30 mg/day), but most require much higher doses. Patients with disease that is not extensive may be treated as outpatients.
Hospitalization and high-dose corticosteroids are indicated for patients with widespread disease, which may be fatal if inadequately treated. The initial dose of oral prednisone, 30 to 40 mg bid (or equivalent), should be repeatedly doubled if new lesions continue to appear after 5 to 7 days. Very high doses may be necessary.
Corticosteroid dose should be tapered if no new lesions appear for 7 to 10 days, with the total daily dose given every morning at first, then every other morning. The maintenance dose should be as low as possible. Many patients require maintenance therapy, which can usually be discontinued after months or years if no new lesions appear during a trial of several weeks without treatment. Methotrexate, cyclophosphamide, azathioprine, gold, or cyclosporine used alone or with corticosteroids reduces the need for corticosteroids and thus minimizes the undesirable effects of long-term corticosteroid use, but the aforementioned drugs also carry serious risks. Plasmapheresis combined with an immunosuppressive drug to reduce antibody titers has also been effective.
Active skin infections are treated with systemic antibiotics. Reverse isolation procedures may be required. Generous use of talc on the patient and sheets may prevent oozing skin from adhering; hydrocolloid dressings may be useful. Silver sulfadiazine cream used on erosions can prevent secondary infection.
Ref: http://www.merck.com/mrkshared/mmanual/section10/chapter120/120b.jsp
and.....
Bullous Pemphigoid
A chronic, pruritic bullous eruption usually occurring in the elderly.
Bullous pemphigoid is considered an autoimmune disease because antibodies directed against the basement membrane zone of the epidermis (the site of histologic damage) are usually found in the serum and skin.
Symptoms, Signs, and Diagnosis
Characteristic tense bullae develop on normal-appearing or reddened skin, sometimes accompanied by annular, dusky-red, edematous lesions with or without peripheral vesicles (see Plate 120-4). Occasionally, rapidly healing oral lesions are seen. Itching is common, usually without other symptoms.
Bullous pemphigoid must be differentiated from pemphigus, linear IgA disease, erythema multiforme, drug eruptions, benign mucosal pemphigoid, dermatitis herpetiformis, and acquired epidermolysis bullosa. As in many other bullous diseases, subepidermal blisters are usually found on biopsy. Finding serum IgG antibodies to the basement membrane zone on an indirect immunofluorescence test usually confirms the diagnosis. IgG, complement, or both are bound to the basement membrane zone of perilesional skin in the direct immunofluorescence test.
Treatment
The eruption usually improves with oral prednisone 40 to 60 mg every morning. The dose can be tapered to a maintenance level after several weeks. Occasional new lesions in the elderly should be disregarded because increasing the prednisone dosage (as in pemphigus) adds more risk than benefit. The disorder occasionally responds to a combination of tetracycline and nicotinamide. Other treatment options include topical and intralesional corticosteroids, dapsone, sulfapyridine, erythromycin, and tetracycline for their anti-inflammatory rather than their antibiotic properties. Most patients do not require azathioprine, cyclophosphamide, cyclosporine, or plasmapheresis. As in pemphigus, corticosteroid-sparing drugs are recommended. The most popular and effective of these drugs, azathioprine and cyclophosphamide, require 4 to 6 wk before clinical efficacy is observed.
Ref: http://www.merck.com/mrkshared/mmanual/section10/chapter120/120c.jsp
hope this answers your questions.
JP Saleeby, MD
www.saleeby.net
