Multisystem Langerhans cell histiocytosis presenting as an oral lesion
An 8-month-old female patient was brought to the dentist by her mother with a complaint of an ulcerative growth in the hard palate. Her mother had noticed an erupting tooth in the anterior part of the upper gums when the patient was 3 months old. She also noted a small ulcerated swelling in the same region about 3 months ago. The lesional area was impacted with food debris resulting in foul odor. There was bleeding from the lesional site on manipulation. There was no other complaint except for occasional mild rashes on the scalp and trunk. The patient was afebrile, and active with no disturbance during sleep.\nOn examination, there were no significant extra-oral findings. Intra-oral examination revealed an ulcerative growth present in the alveolus of the right maxilla extending from the region of 51 to 55 t and also extending onto hard palate, measuring 1.5 cm × 1.5 cm in size. The ulceration had a raised edge. The crown of 52 was noted within the lesion and was mobile []. The other teeth present in the oral cavity were the erupting 71 and 81. The clinical impression was a slow growing ulcerative lesion of the right maxillary alveolus extending onto the hard palate.\nThe posterior-anterior and lateral skull views showed a cystic lesion present in the right maxillary region, and a radiological diagnosis of a maxillary alveolar cyst was given. There was no lesion extending to the skull vault.\nComputed tomography (CT) scan showed a nearly spherical mass in the right side of the palate measuring 1.37 cm (anteroposteriorly) × 1.44 cm (width) × 0.63 cm (height). Destruction of the adjacent alveolar process of the maxilla and anterior aspect of the hard palate on the right side was noted along with the loss of normal bony crypts of upper teeth. Tooth 52 was pushed out prematurely. Multiple enlarged lymph nodes were observed in the neck bilaterally. No focal lesion was seen in the visualized lung apices.\nBlood investigation showed no significant findings. During biopsy, all the crowns of the developing teeth were seen floating within the lesion. The dental surgeon was unable to separate the lesion from the surrounding normal tissue; hence tissue had to be obtained as piecemeal.\nThe gross specimen consisted of one large and several small soft tissues. All the soft-tissue bits were white to dark brown in color and firm in consistency. The largest tissue measured 1.3 cm × 0.3 cm × 0.6 cm while, the smaller soft-tissues collectively measured 1.0 cm × 0.3 cm × 0.6 cm in size.\nMicroscopically the soft-tissues showed ulcerated hyperplastic parakeratinized stratified squamous epithelium with a moderately collagenous connective tissue stroma that was diffusely infiltrated with pale staining cells resembling histiocytes. The lesional cells had an indistinct eosinophilic cytoplasm and exhibited indentation of nuclei. Varying numbers of eosinophils, lymphocytes and plasma cells were interspersed among the lesional cells []. Abundant hemorrhagic foci were evident. The lesional cells stained positive for S100 and CD1a []. Odontogenic epithelial cells, dentine and enamel matrix were also present. As the lesional cells were positive for CD1a a diagnosis of LCH was confirmed.\nOur patient was referred to the oncology department where she was staged as a multisystem high risk case. Our patient was totally asymptomatic with the multisystem involvement being totally unrecognized leading to delay in parents seeking treatment. The patient was placed under ARM B protocol for LCH consisting of an initial treatment with prednisolone, vinblastine, etoposide and a continuation treatment with oral 6-mercaptopurine followed by pulses of oral prednisolone, vinblastine and etoposide. Despite the treatment plan adopted, the baby succumbed to her disease within 6 months after the initial diagnosis.