Papillon-Lefevre syndrome-like presentation in chronic arsenicosis: A rare mimicry
A 20-year-old unmarried, young girl from the remote village of South Bengal, who had slightly poor physical but normal mental development, presented with occasional high rise of temperature, frequent dental caries, and fall-out of teeth from time to time since the age of 3–4 years along with persistent thickening, flaking, and scaling of the skin of her palms and soles for past 14 years. She also complained of poor formation of saliva (xerostomia), poor formation of tear (xerophthalmia), recurrent conjunctivitis, alopecia, frequent pharyngitis, otitis, rhinitis, and pyogenic skin lesions since childhood. The past dental history revealed that her deciduous teeth had erupted normally, but exfoliated gradually by the age of 4–6 years. Similarly, her permanent teeth too were lost prematurely after having erupted normally. At the age of 6 years, her parents had noticed the presence of rough skin on the plantar surface of her feet, with subsequent involvement of the palmar surface of the hands by the age of 9–10 years. The remainder of her past medical history was unremarkable. There was a family history of acquired palmoplantar keratodermas of her parents. She also informed that many of her neighbors of her locality had similar palmar-plantar hyperkeratosis without any other complaints she presented, particularly dental problems.\nOn examination, there were symmetrical, well-demarcated, keratotic, and confluent plaques affecting the skin of the palms and soles [Figures and ]. The skin was dry and rough on palpation. She also had chronic eczematous dermatitis in elbows and knees, few areas of hypo/hyper-pigmentation of anterior and posterior part of the chest. Her hair was sparse, fine, and lusterless, brittle along with alopecia; she also had decreased body hair and sparse or absent eyebrows and eyelashes. On intraoral examination, it was found that patient′s central incisors, mandibular lateral incisors, maxillary first premolars, and all permanent first molars were missing []. All permanent teeth that were present exhibited marked mobility and were malformed, rudimentary, and conical or pegged teeth. Enamel defects were present with dental caries. Detailed physical and systemic examinations were unremarkable, except for mild to moderate pallor. Routine hematological examinations and routine biochemical examinations revealed normal results, except for anemia. Serological tests like anti-nuclear antibody, anti-cytoplasmic neutrophilic antibody, HIV-I and II, and VDRL were negative. Chest X-ray posterior-anterior view was within normal limit. Orthopantomonograph (OPG) of the patient showed severe alveolar bone loss in relation to the existing permanent teeth up to the level of apical third of roots, giving the teeth a “floating in air” appearance. At this point, we made a clinical provisional diagnosis of Papillon-Lefevre syndrome, with a possible differential diagnosis of chronic arsenicosis. All the clinical features were in favor of Papillon-Lefevre syndrome, except for those that were similar palmo-plantar hyperkeratotic lesions of her parents and of few neighbors. Histopathology of skin lesions was not done as her parents declined a biopsy. To rule out chronic arsenicosis, we decided to send her hair and nail for estimation of arsenic level by neutron activation analysis at our own costs as she was from a poor family. Neutron activation analysis of hair and nail proved the diagnosis of chronic arsenicosis. She and her parents and affected neighbors were advised to change the source of drinking water. She was initiated with a standard dose of penicillamine as a chelating agent and was referred to a dermatologist for treatment of palmo-planter hyperkeratosis.