Page 167 - The Netter Collection of Medical Illustrations - Integumentary System_ Volume 4 ( PDFDrive )

Plate 5-4 Autoimmune Blistering Diseases

MUCOUS MEMBRANE PEMPHIGOID

Mucous membrane pemphigoid goes by other names,
including cicatricial pemphigoid, Brunsting-Perry
pemphigoid, ocular pemphigoid, and benign mucous
membrane pemphigoid. The last name should not be
used because this is a chronic progressive, disabling
disease with severe morbidity and mortality. The term
cicatricial inherently states that the disease is associated
with scarring, but this is not always the case. Hence,
one patient without scarring may be referred to as
having ocular pemphigoid and another with scarring
may be said to have cicatricial ocular pemphigoid.
Almost all patients will have some form of scarring,
albeit very mild in some cases, if monitored for a long
enough period. In reality, these are names given to a
heterogeneous group of autoimmune blistering diseases
that express a unique phenotype and have been shown
to have small variances in the basement membrane zone
autoantibodies they produce.
Clinical Findings: Mucous membrane pemphigoid
can be seen in any racial group and affects females more
often than males, in a 2 : 1 ratio. It is a disease of older
persons and is most commonly seen in the seventh and
eighth decades of life. Mucous membrane pemphigoid
is a severe, chronic autoimmune blistering disease with
grave consequences. It is a major cause of morbidity and
mortality, and therapy can be difficult. Up to one quarter
of these patients have eye involvement, which can lead
to decreased vision and blindness. Mucous membrane
disease is typically the initial sign: Patients present with
painful erosions in the nasal passages, oropharynx, geni-
talia, and pulmonary tree. Patients complain of pain and
difficulty eating secondary to severe discomfort. Ero-
sions are the most common clinical findings, but vesicles
and bullae may also be seen. Pulmonary and esophageal Ocular cicatricial pemphigoid. Scarring can become so severe as to cause vision loss.
involvement may lead to strictures that result in diffi- Symblepharon is commonly seen.
culty with breathing or eating. Weight loss typically
ensues, as does malaise and fatigue.
The skin can also be affected, leading to blister for-
mation that heals with scarring and milia. If blisters
develop on the scalp, they heal with a scarring alopecia.
This form of the disease has been given the name
Brunsting-Perry pemphigoid. This term is typically
reserved for only those cases involving the scalp and
skin that do not affect the mucous membranes.
Ocular pemphigoid is a chronic symmetric disease.
The initial symptoms are inflamed conjunctiva, discom- Epidermis
fort, pain, and increased tear production. Scarring soon
develops and forms fibrous adhesions between the palpe-
bral and bulbar conjunctivae. This scarring is termed
symblepharon. The scaring is progressive, and it may Dermis
cause the eyeball to become frozen in place. Entropion is
common, and as it progresses, the eyelashes turn inward
(trichiasis) and are forced against the cornea, which
causes severe pain, irritation, and corneal ulceration. Subepidermal cleavage plane in
Patients cannot entirely close their eyelids because of cicatricial and bullous pemphigoid
the severe scarring. The damaged cornea undergoes
keratinization, leading to opacity of the cornea and
blindness. immunofluorescent staining is present along the base- Treatment: Prednisone is the drug used to treat
Histology: Subepidermal blistering that heals with ment membrane zone. the disease initially. After the disease is under some
scar formation is the hallmark of this disease. The blis- Pathogenesis: Autoantibody formation against pro- control, the addition of a steroid-sparring immuno-
tering takes place just below the keratinocyte, within teins of the basement membrane zone has been linked suppressant should be attempted. Commonly used
in the lamina lucida. Immunohistochemical staining to cicatricial pemphigoid. Many different antibodies medications include azathioprine, methotrexate, myco-
with collagen type IV shows that the blister plane is against these proteins exist, including antibodies against phenolate mofetil, and cyclophosphamide. Dapsone
above the level of the lamina densa. The immunostain- the laminins, bullous pemphigoid antigens 180 and 230, and sulfapyridine, a similar medication that can be used
ing and routine hematoxylin and eosin staining show and many other proteins as yet unclassified. The het- in place of dapsone, have had some success treating this
a picture very similar to that of bullous pemphigoid. erogeneity in antibody production likely accounts for disease. Intravenous immunoglobulin (IVIG) has been
Linear immunoglobulin G and complement C3 the varying clinical phenotypes that are expressed. used with success in refractory cases.

THE NETTER COLLECTION OF MEDICAL ILLUSTRATIONS 153