| Abstract|| |
The Rationale: Sarcoidosis is a multisystemic granulomatous disease of unknown aetiology that usually presents with systemic lesions including the skin. Isolated cutaneous lesions are very rare. Patient Details: We present a middle-aged female patient, a known systemic lupus erythematosus patient, in remission developing an isolated, single sarcoidosis lesion in the perioral labial mucosa. The lesion was a rapidly growing nodular lesion with a papillary surface near the philtrum of the upper lip. Diagnosis: A biopsy revealed that to be a granulomatous lesion with large multinucleated giant cells. Using a panel of tests, by excluding tuberculosis and establishing the immunoprofile, the diagnosis was established. Although infrequent, perioral and oral lesions may constitute the first signs of systemic sarcoidosis. Outcomes and Take-away Lessons: For this reason, the oral health care provider needs to be watchful and when there is a suspicion of sarcoidosis, systemic sarcoidosis must be included in the differential diagnosis of oral and perioral granulomatous lesions.
Keywords: Isolated cutaneous lesions, perioral sarcoidosis, sarcoidosis
|How to cite this article:|
Balaji S M, Balaji P. A rare case report of perioral sarcoidosis. Indian J Dent Res 2021;32:261-3
| Background|| |
A single, long-standing verrucopapillary lesion in the perioral area has several differential diagnoses. The medical history, clinical examination and blood picture had to be considered before arriving at a proper diagnosis. As diagnosis is essential for proper management. Unusual lesions at unusual sites may pose a diagnostic and management challenge. Sarcoidosis is a rare, chronic, multisystemic disorder with an unknown cause and impaired tissue function. It is characterised by non-caseating granulomas, commonly involving the lungs, lymph nodes, skin and eyes. Evidence of the involvement of two organs is required for a definite diagnosis of sarcoidosis. The presence of non-caseating epithelioid cell granulomatous inflammatory infiltration in the cutaneous lesions is generally not regarded until all other histologically-mimicking diseases are ruled out. Some individuals with cutaneous non-caseating granulomas will have an indolent clinical course with no disease activity elsewhere, whereas others may eventually develop disease activity elsewhere. Due to its wide spectrum of presentation such as papules, plaques, lupus pernio, scar psoriasiform and ulcerative lesions, cutaneous sarcoidosis is referred to as the “great imitator” in dermatology. It is because of this that perioral cutaneous sarcoidosis are rare and pose a diagnostic challenge.
The autoimmune disease, systemic lupus erythematosus (SLE) as well as sarcoidosis are associated with antiphospholipid antibodies with both entities showing evidence of B cell hyperreactivity including hypergammaglobulinemia, increased response to alloantigens and the presence of blood cell isoantigens. There are a few reports of these entities occurring together.,
The aim of this manuscript is to present a case of perioral cutaneous sarcoidosis in a patient with SLE in remission, highlighting the diagnostic challenge.
| Case Report|| |
A 42-year-old female nursing matron sought treatment for a small papillary growth in the upper lip for the past 3 years. The growth started as a small insidious entity that enlarged to the present hemispherical size of 1.2 cm. No associated signs of irritation, pain or burning sensation were seen barring aesthetic concern. It has been diagnosed earlier as a viral infection by the dermatological colleagues and she was on topical Acyclovir for varying periods of time. History revealed that she was treated for SLE 4 years back and had been asymptomatic since then. She is under periodic review and follow-up for the same. Currently, she is not on any medications. On examination, no lymphadenopathy and no systemic disorders were observed. There was no similar lesion elsewhere in the body. The surface of the lesion was papillary, erythematous with distinct borders and no ulcerations. A working diagnosis of the papilloma was made and the lesion was excised in toto. Layered closure was placed. The healing was uneventful [Figure 1].
|Figure 1: (a) Preoperative view—small verrucopapillary growth in the upper lip. (b) Lesion excised (c) Excised specimen (d) Immediate postoperative|
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The histopathological study showed normal skin with appendages along with papillary projections and normal dermal epithelium as well as connective tissue interface. The connective tissue, under low power, showed characteristic chronic inflammatory cell infiltrate in the sheets in addition to numerous giant cells. These giant cells on high power resembled the classic Langhan's giant cell [Figure 2].
|Figure 2: (a and b) 4X: Hematoxylin and Eosin-stained section of the nodule showing the papillary projection of the skin. This section shows the normal skin and appendages in association with power characteristic chronic inflammatory cell infiltrate in sheets in addition to numerous giant cells. 4X power. (c) 10×: Haematoxylin and Eosin-stained section of the nodule showing the papillary projection of the skin. This section shows the normal skin and appendages in association with power characteristic chronic inflammatory cell infiltrate in sheets in addition to numerous giant cells. 10X power. (d) 40X: The giant cells have a typical Langhan's horseshoe-shaped arrangement, haematoxylin and Eosin stain, 40X.|
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A provisional diagnosis of tuberculosis was made and tests were performed. The chest X-ray was negative while the Montouex test was positive. However, the acid-fast stain was negative. The subsequent blood tests revealed that her erythrocyte sedimentation rate was 22 mm per h (Westergren) and C-reactive protein at 3.54 (normal range -8 mg/L). The angiotensin-converting enzyme levels by a kinetic spectrophotometric assay using FAPPG (N-[3-(2-furyl) acryloyl] L-phenylalanyl glycyl glycine) were 142 U/L (normal range: 9–67). The anti-nuclear antibodies (ANA) revealed ++ as speckled at 1:40 in indirect immunofluorescence while the anti-neutrophil cytoplasmic autoantibody (ANCA) at 1:20.
Considering the histopathology, raised ACE, ANA and ANCA levels, a diagnosis of cutaneous sarcoidosis was arrived at. The patient was referred to the rheumatologist for further management. Studies ruled out systemic involvement. She was prescribed prednisolone (1 mg/kg/day), with methotrexate (10 mg/week) for a month and the doses were tapered. For 4 years, there were no lesions elsewhere and the patient had no further episodes of SLE or sarcoid lesions.
| Discussion|| |
Isolated cutaneous sarcoidosis is a rare entity. It poses a diagnostic challenge due to the wide variation in clinical presentation. There is a female gender predilection for cutaneous sarcoidosis. The present case is also female, consistent with the literature. In a series of 40 cases of cutaneous sarcoidosis, only 2 were solitary and these cases had systemic involvement. However, in our case, the lesion was exclusively isolated cutaneous sarcoidosis with no other organ involvement. In all probability, this lesion could have been the earliest manifestation of the condition. An oral manifestation being the first, the presenting sign of the systemic disease has been previously reported. Cutaneous manifestations at times are the initial presenting sign of the condition. There is a polymorphous presentation, confusing the diagnosis. The literature has only a few case reports in similar a fashion.,,
In a series of cutaneous sarcoidosis (n = 40), 14 (35%) patients presented with isolated specific cutaneous lesions and later developed multiple lesions on long-term follow-up of 1.6–19 years with a median of 7 years. In the remaining 26 patients, half-developed systemic involvement in 4–9 years (mean = 6 years). This reinforces the concept that systemic involvement may develop later in cases of cutaneous sarcoidosis. Hence, periodical and prolonged follow-ups are needed for such cases.
In the present case, a prominent papillary lesion on the upper lip of about 6 month's duration caused an unpleasant look. It did not respond to conventional treatment. Hence, the patient consulted us. As the lesion was more than 4 weeks and not responding to any treatment, an excisional biopsy was carried out. The histopathological picture of a non-caseating granuloma prompted full workup. The pre-existing SLE in remission was another diagnostic pointer. The raised ACE, ANA and ANCA helped to arrive at the diagnosis. As there have been reports of SLE and cutaneous sarcoidosis occurring concomitantly, the diagnosis of isolated cutaneous sarcoidosis was arrived at. Owing to the previous history of SLE, the patient was referred to her rheumatologist. A full workup was done to rule out systemic sarcoidosis. As the systemic involvement at a later date is a possibility, as reported in the literature, the patient is on a regular follow-up.
Oral sarcoidosis has been reported to be a presenting manifestation of sarcoidosis in a third of the cases. In such cases, they involve the jawbones. Rarely, the buccal mucosa, gingiva, lips, tongue and palate are affected. For isolated lesions, intralesional and/or topical application of glucocorticoids is often prescribed. Rarely is surgery performed. In our case, as it was an excisional biopsy, the treatment was surgical mode and there was a good prognosis. In the case of systemic involvement, systemic steroids and immunosuppressive drugs are beneficial. Owing to the previous history of SLE and elevated markers, systemic steroids and immunosuppressive drugs were prescribed by the rheumatologist. Possibly, this single perioral lesion could have been a marker of the evolving disease process, in association with a pre-existing SLE. Recent gene co-expression network studies have identified common gene markers that play a critical role in SLE and sarcoidosis, notably the TLR1, ITGAL, TNFRSF1B, CD86, SPI1, BTK and IL10RA genes. This indicates a possible commonality in the pathogenesis of SLE and sarcoidosis as reported in the literature.,
The awareness of oral manifestation of sarcoidosis, even among the dental fraternity is low. This could be due to the rarity of the condition in regular dental practice. This low awareness has been previously documented. It is the histopathology report and past medical history that prompted us to consider this diagnosis. Tuberculosis was the initial diagnosis, as the patient is a medical care provider, a high-risk occupation for tuberculosis infection. However, the absence of other clinical features, as well as a negative acid-fast stain, prompted us to probe for other possible conditions. The blood picture helped us to pin the diagnosis of sarcoidosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
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Dr. S M Balaji
Director and Consultant, Oral and Maxillofacial Surgeon, Balaji Dental and Craniofacial Hospital, 30, KB Dasan Road, Teynampet, Chennai - 600 018, Tamil Nadu
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]