Papillon-lefevre syndrome: Case series and review of literature
A 14-year-old girl, visited to outpatient Department of Periodontia, Government Dental College and Hospital, Ahmedabad, India with chief complain of premature loss of deciduous and permanent teeth and mobility in remaining teeth.\nShe was the first child born to apparently healthy non-consanguineous parents. History revealed that her deciduous teeth had erupted normally, but exfoliated gradually by the age of 4-5 years. Similarly, her permanent teeth were lost prematurely after erupting normally. Patient was having complained of mobility and bleeding gum before 2 years, visited a periodontist and undergone treatment in the form of periodontal flap surgery. After one and half year, patient was having same complain and came to Government Dental College and Hospital, Ahmedabad.\nThere was no family history of ichthyosis or hereditary or acquired palmoplanter keratodermas. The patient also gave a history of development of fissures, thickening and flaking in the skin of palms and soles that resulted in peeling off of skin leaving red thin area underneath since age of 3-4 years. She had exacerbation and remissions of the skin lesions since early childhood, visiting dermatologist regularly, but no improvement was seen. She repeatedly contracted systemic infections.\nExtra-oral examination\nOn extra-oral examination, patient presented with well-demarcated, yellowish, keratotic plaques over the skin of palms and soles extending on to the dorsal surfaces. Skin of both palms and soles was peeling off leaving underlying red shiny area suggestive of keratoderma []. Well circumscribed, psoriasiform, erythematous, scaly plaques were also present on the elbows and knees bilaterally along with dystrophy and transverse grooving of the nails. The fingers were pointed giving clawed appearance.\nIntra-oral examination\nIntra-oral examination revealed edentulous area between teeth 44 and 33 and 11, 21 and 22 were also missing. Edentulous maxillary and mandibular ridges were covered with normal mucosa. The teeth present were 12, 13,14,15,16,22, 24, 25, 26, 33, 34, 35, 36, 44, 45 and 46 covered with soft deposits and band of subgingival calculus. Gingiva was fiery red in color with inflammatory gingival enlargement Iva Ingle Classification grade II. Pathological migration was associated with 12, 14, 23, 24, 33 and 34. On assessment grade III mobility was present in relation with 14, grade II in relation with 24, 25, 33, 34, 36 and 44. Remaining teeth were present with grade I mobility. There was the presence of recession grade III in relation with 14 and 33 [Figures –].\nOrthopantogram showed extensive alveolar bone loss in all remaining teeth. The alveolar bone around the mobile teeth was devoid of definable lamina dura. An extensive alveolar bone loss was noted, giving the teeth 31, 41 and 43 a “floating in air appearance,” which were extracted afterwards [].