Figure 2- Microscopic view of giant cell bodies and Asteroid bodies and
bundles of histiocytes.
With the consultation of a rheumatologist, Nisopred 50 mg was prescribed
and after a 2-month follow-up, the patient showed a relative response to
the drug, and was referred to a pulmonologist. and Due to the relative
response to the previous drug, and single coughs that had started prior
to taking it, Salbutamol spray was also prescribed. A chest x-ray was
performed and not showed anything in particular.
Within another follow-up, and after quitting Nisopred, the intra-oral
signs and lesions recurred, and therefore with the consultation of
another rheumatologist, this time Hydroxychloroquine therapy was
considered. Although currently the patient hasn’t checked in for a visit
and did not start taking Hydroxychloroquine(4), but with follow-up on
the phone, the patient reported betterment of the lip redness.
Discussion
Sarcoidosis is described as a disease in which the clinical signs and
symptoms are not severe enough to cause alarm. Although the prognosis of
sarcoidosis is good and in 60% of cases it regresses on its own, it is
still a disease associated with heart, kidney, CNS, and lung
involvement. And therefore must be taken seriously, due to the fact that
almost 4-10% mortality rate is based on these involvements(5).
Some researchers claim that lesions in the soft tissue of oral cavity
due to sarcoidosis are not very common(6), but our patient had a lesion
on her buccal mucosa.
Swelling of the cheek and lips suggested it to be Orofacial
Granulomatosis, although systemic diseases such as Tuberculosis,
Sarcoidosis, Crohn’s disease, and Melkersson-Rosenthal syndrome had to
be ruled out first(7), therefore further microscopic testing and
examination were required.
Also, in the intra-oral aspect of OFG, cobble-stone swellings, grooved
tongue, recurrent labial swelling, and gingival inflammation can be
seen(8). On the other hand, intra-oral signs of sarcoidosis includes the
occurrence of multiple nodules, xerostomia, and involvement of salivary
glands(9). The microscopic view of OFG shows aggregates of non-caseating
granulomatosis inflammations(10), while in Sarcoidosis we can see
aggregates of epithelial histiocytes and a surrounding rim of
lymphocytes and Asteroid bodies and Schaumann bodies.
Some studies suggest a biopsy of affected tissue for demonstration of
non-caseating granulomas that strongly support the diagnosis of
sarcoidosis(11). And in our case, as mentioned above, the biopsy result
revealed a granulomatous inflammation along with non-caseating
granulomas.
Measurement of serum angiotensin-converting enzyme (ACE) can be helpful
in diagnosis and monitoring the response to treatment, as the level of
this enzyme is raised in about 60-80% of patients with sarcoidosis(11,
12). Although due to its poor sensitivity, an increase in ACE levels
does not necessarily indicate the diagnosis of sarcoidosis(13).
Laboratory tests were performed on our patient and the results were much
like expected. ACE levels were higher than the normal range (78 IU/L)
which could support the diagnosis.
Another factor that could support the diagnosis of this condition is the
Erythrocyte Sedimentation Rate (ESR) which has a normal range of 0-22 in
women, and a recent case report study claims that some patients with
sarcoidosis can have an increase of ESR level(14). Laboratory tests
supported the slight increase of ESR levels to 25 mm/h in our patient as
well, although not significantly enough to verify the diagnosis.
Management of this particular disease can range from no interventions to
systemic corticosteroids to surgical excision(15). Because it is known
that corticosteroids are generally considered beneficial in the acute
phase of sarcoidosis, some studies have found that oral glucocorticoids
are the first option(16), much like our case in which the first-line
treatment was the prescription of Nisopred 50mg.
In cases of sarcoidosis, usually, an absence of treatment response is
rare and urges for verifying the absence of a diagnosis error(17). But
in this case, we can see resistance to treatment response from the
patient.
Nevertheless, studies have shown that oral sarcoidosis can be a
manifestation of a systematic disorder(18), therefore follow-up of the
patient in this stage is necessary. We hope to bring awareness to other
dentists and fellow researchers to notice the oral symptoms of
sarcoidosis which is usually assumed to be irrelevant to the disease.
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