Oral manifestations of lamellar ichthyosis in association wi
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A 5-year-old girl was referred by the department of dermatology with a chief concern of missing upper and lower front teeth. There were no prenatal complications and she was born covered with a waxy-parchment-like colloid membrane also termed as a ‘collodion baby’. This later started to shed by itself within 1 month of birth, followed by desquamation and formation of thick polygonal brown scales in the skin. Persistent itching in eyes and ears was also reported. The workup for the diagnosis was done in this tertiary care hospital and she was confirmed to have LI with rickets at the age of 8 months.

She had dry and sparse body hairs with scanty eyebrows. Examination of the skin revealed large dark-brown polygonal scales, most severely affecting the limbs, especially lower limbs. Hypopigmentation and atrophic scars were also observed on dorsal surfaces of hands and legs. There was a prominent involvement of face and scalp with xerosis of skin with the presence of ectropion and eclabium.

The intraoral examination revealed that the child was having primary dentition; 51, 52, 61, 62 and 71 were clinically missing. Developmental defects of enamel formation including discontinuity on the labial surface were evident with 81, 82 and 72. An intraoral periapical radiograph confirms the missing primary maxillary incisors. Primary mandibular incisors and canines were supra erupted and irregularly placed. Grade II
mobility with flaring was present with 72, 73, 81, 82 and 83. Oral hygiene was good with normal colour, consistency, contour and scalloping of gingiva without any debris and calculus. Treatment comprised of oral prophylaxis followed by application of fluoride varnish (Fluoritop 22600 ppm slow release) and pit and fissure sealant. The missing teeth were replaced with removable partial denture prosthesis. Treatment of rickets includes administration of vitamin D 60000IU/day for the first 10 days followed by 4000IU/day as a maintenance dose. The management of skin lesions includes moisturisers with propylene glycol keratinolytics combined with salicylic acid and topical retinoids.

Oral hygiene instructions were reinforced and the patient was
recalled every 3 months. Patient is maintaining good oral hygiene and there are no fresh complaints.

Source: https://casereports.bmj.com/content/13/7/e235008