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).