Case report
A five-year-old male presented with a chief complain of loose teeth bilaterally in the lower jaw since three months. On evaluation of the history, the parents summarized that the child first complained of pain while eating and occasional bleeding from gingiva six months back. Soon the parents also noticed swelling and structural distortion in the fingernails on left hand along with pus discharge from left ear. The parents also added that they had brought the child for check-up six months earlier but due to Covid-19 pandemic, no active intervention was possible.
On clinical examination, there was blackish discoloration and destruction of nail plate of middle and little finger of right hand and all the fingers of left hand (Figure 3). Also, watery discharge was seen from the left ear.
On intraoral examination, multiple ulcerations were present bilaterally on hard palate, extending posteriorly with the largest lesion measuring 1.5 X 1 cm2 in maximum dimension (Figure 1a). After six weeks, the ulcer showed central area of creamish white slough of variable thickness with erythematous periphery (Figure 1b). On further examination, Grade III mobility was present in relation to teeth 74 and 84 with gingival recession and grade II mobility were evident on 75 and 85. Also, the marginal and attached gingiva in relation to 75 and 85 was inflamed (Figure 2).
Panoramic radiograph was advised, however, the image was distorted as the patient was uncooperative. Interdental bone loss in relation to 64, 65, 74, 75, 84, and 85 teeth region with distortion of follicle of tooth 34 was observed.
Computer tomography (CT) scan of the head and neck region was performed under midazolam sedation which showed mild soft tissue thickening in the bilateral lateral aspect of hard palate. However, no obvious growth or erosion of palate was observed.
The blood investigation showed elevated level of alkaline phosphate (387 U/L) (normal range: 35-130 U/L) and serum glutamic-oxaloacetic transaminase (SGOT: 51 U/L) (normal range: 10-35 U/L). Also, there was slight increase in serum glutamic-pyruvic transaminase (SGPT: 47 U/L) (normal range: 09-43 U/L) and platelet (475000 cell/mm) (normal range: 150000-400000 cells/mm).
The clinical diagnosis of granulomatous disease was suggested and incisional biopsy was planned under general anesthesia. Two soft tissue specimens from hard palate and junction between hard and soft palate, measuring 17 X 10 X 4 mm and 9 X 8 X 6 mm respectively were obtained, fixed in 10% neutral buffered formalin, and sent for routine histopathological processing (Figure 4). The processed tissue were embedded and sectioned into 3 µm sections and stained with routine hematoxylin and eosin stain.
On histopathological examination a highly cellular connective tissue stroma with numerous scattered round to polygonal shaped cells with pale cytoplasm was evident (Figure 5a, 5b). On higher magnification (oil immersion), a characteristic coffee bean shaped and oval nuclei which were folded or indented with some cells having grooved nuclei were observed (Figure 5c, 5d). Background stroma was abundant with eosinophils (Figure 5d). Overlying epithelium was para to non-keratinized stratified squamous type with irregular rete ridges. The histopathological features along with clinical findings and altered serum biochemical parameters were suggestive for Langerhans cell histiocytosis as a probable diagnosis.
Further, on immune-histochemical analysis histiocytic cells showed positivity to CD1a, Langerin (CD-207), and S-100 markers confirming the diagnosis of LCH. 99MTC-MDP bone scan of the patient revealed normal bone with no evidence of skeletal metastasis (Figure 6). Ultrasonography of the upper abdomen and pelvic region showed normal scan with no spleen and liver involvement.
A final diagnosis of Langerhans cell histiocytosis – single system multifocal disease was made and the patient underwent chemotherapy (Vinblastine + Prednisolone) for 43 weeks. The lesions have healed and no new lesions have developed in one year follow-up visit (Figure 7).