Cronh’s Disease the process, tiny erosions of the overlying

normal mucosal lymphoid tissues

Crohn’s disease is an inflammatory and
eventually coalesce to form small aphthous
probably an immunologically mediated
ulcers or more diffuse ulceration of the
condition of unknown cause that primarily
mucosa. With progression, there is marked
affects the distal portion of the small bowel
hyperplasia of the lymphoid tissue
and the proximal colon. It is now well
extending through the wall, fibrosis and
established that the manifestations of
muscular hypertrophy leading to
Crohn’s disease may be seen anywhere in
constrictures, and inflammatory tracts.
the gastrointestinal tract, from the mouth to
Granulomas are present in about 50% of
the anus. In addition, other extraintestinal
patients
sites of disease involvement, such as the
skin, eyes, and joints, have also been Clinical Features
identified. The oral lesions are significant
Most patients with Crohn’s disease are
because they may precede the
teenagers when the disease first becomes
gastrointestinal lesions in as many as 30%
evident, although another diagnostic peak
of the cases that have both oral and
of disease activity occurs in patients more
gastrointestinal involvement. It is
than 60 years of age. Gastrointestinal signs
interesting that the prevalence of Crohn’s
and symptoms usually include abdominal
disease appears to be increasing, but the
cramping and pain, nausea, and diarrhea,
reasons for this increase have not been
occasionally accompanied by fever. Weight
determined.
loss and malnutrition may develop, which
Crohn’s disease affects all ages and both can lead to anemia, decreased growth, and
sexes and occurs most frequently in urban short stature.
women aged 20 to 39 years. The prevalence
of Crohn’s disease among firstdegree
relatives is 21 times higher than that among
non relatives.
Aetiology
The cause and evolution of Crohn’s disease
are unknown. The single strongest risk
factor for Crohn’s disease, overpowering A wide range of oral lesions has been
any influences of diet, smoking, stress, or clinically reported in Crohn’s disease;
hygiene, is having a relative with the however, many of the abnormalities
disease. The fact that first-degree relatives described are relatively nonspecific and
of Crohn’s disease patients exhibit may be associated with other conditions
increased intestinal permeability supports that cause orofacial granulomatosis (see
the theory of an inheritable permeability page 341). The more prominent findings
defect in Crohn’s disease. This abnormal include diffuse or nodular swelling of the
intestinal barrier could result in the oral and perioral tissues, a cobblestone
increased uptake of injurious materials appearance of the mucosa, and deep,
and/or enhanced immune reaction to granulomatous-appearing ulcers. The
intestinal antigens. Other theories have ulcers are often linear and develop in the
included vascular disease, lymphatic buccal vestibule (Fig. 17-40). Patchy
obstruction, and emotional stress. Whatever erythematous macules and plaques
involving the attached and unattached
gingivae have been termed mucogingivitis
and may represent one of the more common
lesions related to Crohn’s disease. Soft
tissue swellings that resemble denture-
related fibrous hyperplasia may be seen, as
well as smaller mucosal tags. Another
manifestation that has been reported is
aphthouslike oral ulcerations, although the
significance of this finding is uncertain
because aphthous ulcerations are found
rather frequently in the general population,
including the same age group that is
affected by Crohn’s disease. One large
study showed no difference in the
prevalence of aphthous ulcers in patients
with Crohn’s disease compared with a
control population. Fewer than 1% of
patients with Crohn’s disease may develop
diffuse stomatitis, with some cases
apparently caused by Staphylococcus
aureus, and others being nonspecific. In at
least one instance, recurrent severe buccal
space infections resulted in cutaneous
salivary fistula formation. Infrequently,
pyostomatitis vegetans (see next topic) has
been associated with Crohn’s disease.
Histopathologic Features
Histologically, the granulomas are typically
small, loose and with few multinucleate
giant cells. They are often deep in the
corium and may be difficult to find. A
biopsy may need to extend unusually
deeply.
Microscopic examination of lesional tissue
obtained from the intestine or from the oral
mucosa should show nonnecrotizing
granulomatous inflammation within the
submucosal connective tissue (Fig. 17-41).
The severity of the granulomatous
inflammation may vary tremendously from
patient to patient and from various sites in
the same patient. Therefore, a negative
biopsy result at any one site and time may
not necessarily rule out a diagnosis of
Crohn’s disease. As with the clinical
lesions, the histopathologic pattern is
relatively nonspecific, resembling orofacial
granulomatosis. Special stains should be
performed to rule out the possibility of deep
fungal infection, tertiary syphilis, or
mycobacterial infection.
Treatment and Prognosis
Most patients with Crohn’s disease are
initially treated medically with a sulfa type
of drug (sulfasalazine), and some patients
respond well to this medication.
Metronidazole may be used if no response
is seen with sulfasalazine therapy. With
moderate to severe involvement, systemic
prednisone may be used and is often
effective, particularly when combined with
the immunosuppressive drug, azathioprine.
Infliximab, a monoclonal antibody directed
against tumor necrosis factor-α (TNF-α),
has shown promise in refractory cases of
Crohn’s disease. Sometimes the disease
cannot be maintained in remission by
medical therapy, and complications
develop that require surgical intervention.
Complications may include bowel
obstruction or fistula or abscess formation.
If a significant segment of the terminal
ileum has been removed surgically or is
involved with the disease, then periodic
injections of vitamin B12 may be necessary
to prevent megaloblastic anemia secondary
to the lack of ability to absorb the vitamin.
Similar supplementation of magnesium,
iron, the fat-soluble vitamins, and folate
may also be required because of
malabsorption.
Oral lesions have been reported to clear
with treatment of the gastrointestinal
process in many cases. Occasionally
persistent oral ulcerations will develop, and
these may have to be treated with topical or
intralesional corticosteroids. Systemic
thalidomide and infliximab have been used
successfully to manage refractory oral
ulcers of Crohn’s disease.