Pemphigus Vulgaris Disease

DESCRIPTION

Uncommon, debilitating, potentially fatal skin disorder characterized by painful intraepidermal bullae that appear on normal appearing skin without surrounding inflammation, often starting in the mouth

System(s) affected: Skin/Exocrine, Gastrointestinal
Genetics: HLA-A10 and HLA-DR4 and HLA-DRW6 antigens; higher incidence among persons of Jewish or Mediterranean descent
Incidence/Prevalence in USA: Rare
Predominant age: 30-60 years of age
Predominant sex: Male = Female

SIGNS AND SYMPTOMS

Oral mucous membrane lesions (particularly in the posterior mouth) often precede the cutaneous lesions (sometimes by several weeks or months)
Lesion distribution – upper trunk or back initially; gradual extension to face, groin, and axillae
Bullae arise from normal-appearing skin
Multiple shallow erosions which heal slowly
Blisters are fragile
Intact bullae are found only on the first day or two of their existence
After blister roof breaks, a bright red or crusted shallow erosion follows which requires weeks or months to heal
Outer layer of skin can easily be rubbed off (Nikolsky's sign)

CAUSES

An autoimmune disorder with specific IgG antibodies and sometimes complement arising from bone marrow plasma cells which are deposited at sites of epidermal cell damage; a few cases from captopril, penicillamine, piroxicam, penicillin, phenobarbital, pyritimol, heroin

RISK FACTORS

Genetic factors (more common in persons of Jewish or Mediterranean descent)
Medications (particularly penicillamine)

LABORATORY

Autoantibody titers (by immunofluorescent studies) present in 80–90%
Titer corresponds to severity of the disease. Increasing titer is found prior to relapse.

Drugs that may alter lab results: N/A
Disorders that may alter lab results: N/A

PATHOLOGICAL FINDINGS

Causative antigens are located on the exterior surface of the cytoplasmic membrane of epithelial cells
Biopsy shows acantholytic intraepidermal bullae
IgG deposition in the epidermal intercellular space is found 100% of the time in perilesional skin

SPECIAL TESTS

N/A

IMAGING

N/A

DIAGNOSTIC PROCEDURES

Biopsy of lesions
Light microscopy – suprabasal cleft formation and acantholysis

APPROPRIATE HEALTH CARE

Depends on severity of the disease and medical status of patient

GENERAL MEASURES

May require reverse isolation procedures
Topical treatment to prevent oozing skin from adhering to bed sheets
Soak active lesions to debride and remove crusts
Analgesic mouth washes (see Medications)
Plasmapheresis or cyclosporine if the patient fails to respond to an adequate trial of recommended regimens

SURGICAL MEASURES

N/A

ACTIVITY

As severity of disease and medical status of patient dictates

DIET

Liquid or soft for patient with mouth lesions. Regular diet when tolerated.

PATIENT EDUCATION

Use of oral analgesics
Teach side effects and adverse reactions of steroids

PREVENTION/AVOIDANCE

N/A

POSSIBLE COMPLICATIONS

Steroid complications that can lead to morbidity and mortality
Inadequate nutrition and debilitation due to pain of oral lesions
Sepsis/death for untreated or poorly controlled cases

EXPECTED COURSE AND PROGNOSIS

Chronic. Inevitably fatal if not treated.
10% fatality with vigorous treatment
Ruptured bullae require weeks to heal

ASSOCIATED CONDITIONS

Thymomas
Other internal malignancies
Other autoimmune diseases

AGE-RELATED FACTORS

Pediatric: Unusual in this age group
Geriatric:

This is the age group in which pemphigus is most likely to occur
Close follow-up is needed for elderly patients on high doses of steroids

Others: N/A

PREGNANCY

N/A

OTHER NOTES

Atypical presentation – pemphigus foliaceus has infrequent oral lesions and is not as debilitating
In mild disease, gold salts alone can sometimes produce remission. This has the advantage of avoiding immunosuppression; however, it is not uniformly beneficial and there are no controlled trials.

ABBREVIATIONS

N/A

Clinical Investigations

ROLE OF HOMOEOPATHY

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