Question:
Has any one hear heard of the disease called pemphegus?
This disease has symptoms that appear on the skin, mostly the scalp, and
in the mouth, along the gum line and inner check.
The disease produces things that look like scabs on the skin; and in the
mouth, it produces white, tissue-like specks that bleed if you scrap them
off.
Has any one heard of this? Are there many more people out there suffering
from this disease?
Your responses would be greatly appreciated.
Answer:
The term pemphigus refers to a group of autoimmune disorders
characterized by development of vesiculobullous lesions on the skin and
mucous membranes. It is most common in middle-aged and older adults
and affects both sexes equally. An increase prevalence has been noted
in the Jewish population. Patients with pemphigus develop
autoantibodies directed toward an antegen on the surface of squamous
epithelial cells, destroying the intercellular attachments and
resulting in intraepithelial blister formation. Although several
clinical variants are recognized, pemphigus vulgaris is the most common
form.
On the skin, the primary lesions of pemphigus vulgaris are vesicles and
bullae that can arise on normal skin or on an erythematous base. These
blisters are fragile and rupture easily, producing painful, raw,
denuded areas. In later stages of the disease, extensive areas of
epidermis may be sloughed. Scaling and crusting of hte ulcerated areas
are common.
Oral involvement in pemphigus vulgaris is extemely common: over 90% of
patients develop oral lesions at some time during the course of their
disease. In fact, the mouth is the most common presenting site for
lesion os pemphigus vulgaris, withs some studies showing over half of
all cases originating gher. The oral lesions often have a slow,
insidious onset with symptoms present for many months before a
diagnosis is made or skin lesions develop. Lesions may be found
anywhere, but are most common on the soft palate and buccal mucosa.
Gingival involvement may resemble "desquamative gingivitis" Although
the oral lesions are vesiculobullous in nature, intact blisters are
rarely seen; patients usually exhibit collapsed bullae, ulcers, or
widespread areas of erosion. Pain and resultant weight loss are common
complaints.
Diagnosis is established by biopsy and immunofluorescent studies.
titers on indirect immunofluorescence often correlate to the severity
of the disease. Before the advent of corticosteroid therapy, pemphigus
vulgaris was fatal in 60-90% of hte cases because of general
debilitation, malnutrintion, and protein loss. Even today, with
steroid therapy, pemphigus vulgaris is a serious disease with mortality
rate of 5 to 15%. Many of these deaths arise from complications of
steroid therapy. Immunosuppressive drugs and plasmapheresis may also
be used.
taken from "Color Atlas of Clinical Oral Pathology" by Neville, Damm,
White, and Waldron
