Nevus Comedonicus Syndrome—Nevus Comedonicus Associated with Ipsilateral Polysyndactyly and Bilateral Oligodontia Feroze Kaliyadan, M.D., D.N.B., M.N.A.M.S.,* Sheela Nampoothiri, M.D., D.C.H.,  V. Sunitha, M.D., and Vikas Elias Kuruvilla, M.D.S.à *Department of Dermatology, Amrita Institute of Medical Sciences, Kochi, Kerala, India,  Department of Pediatric Genetics, Amrita Institute of Medical Sciences, Kochi, Kerala, India, àDepartment of Oral & Maxillofacial Surgery, PSM College of Dental Science & Research, Thrissur, Kerala, India Abstract: A 7-year-old boy diagnosed as a case of nevus comedonicus syndrome is presented because of the rarity of the condition and the asso- ciation of the previously unreported features oligodontia and unilateral polysyndactyly restricted to the same side as the nevus comedonicus. A 7-year-old boy presented to the department of Pediatric Genetics in our institute with a history of learning disabilities, unclear speech, skeletal defects, and skin lesions restricted to the right side of the body. The boy was the sixth child of nonconsanguineous par- ents. The mother had a history of two first trimester miscarriages; one child had expired on the first day of life (details regarding the cause were not available). The parents and the other living children have no history of any significant neurologic, cutaneous, skeletal, ocular, or dental defects. The boy had a history of unilateral (right) preaxial polysyndactyly of the hand and foot. Polydac- tyly had been corrected by removing the extra digits from hand and foot at the age of 1 year. There was no history of any other significant neurologic, cutaneous, skeletal, or ocular problems. The mother mentioned that only three temporary teeth had fallen off up to the time of presentation. Significant examination findings included: 1. Dysmorphic facies with telecanthus. 2. Radial deviation of right thumb (Fig. 1). 3. Medial deviation of right big toe. 4. Short index finger—right hand. 5. Multiple comedones—grouped together over the right pinna and right axilla (but not in a linear configu- ration) clinically suggestive of nevus comedonicus (NC) (Fig. 2). 6. Dental defects included—oligodontia—patient had 21 teeth with missing upper right incisor, left lateral incisor and third molars (both upper and lower jaws). 7. Neurologic examination was normal. The patient was investigated thoroughly to screen for other systemic abnormalities, especially imaging studies related to the skeletal, ophthalmic, and neurologic systems. All the pertinent investigations were within normal limits. A skin biopsy from the comedonal Address correspondence to Feroze Kaliyadan, M.D., D.N.B., M.N.A.M.S., Department of Dermatology, Amrita Institute of Medical Sciences, Kochi, Kerala 682028, India, or e-mail: ferozkal@ hotmail.com. DOI: 10.1111/j.1525-1470.2010.01170.x Ó 2010 Wiley Periodicals, Inc. 377 Pediatric Dermatology Vol. 27 No. 4 377–379, 2010