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OpenAthens Shibboleth
Special Feature
July 2012

Picture of the Month—Quiz Case

Author Affiliations


Author Affiliations: Departments of Pediatric Dentistry (Messrs Salinas and Martinez) and Basic Sciences (Drs Escamilla and Rivera), University of Monterrey, San Pedro, Mexico.

Arch Pediatr Adolesc Med. 2012;166(7):657. doi:10.1001/archpediatrics.2012.189a

A 16-year-old girl presented with a 3-week history of gingival pain and inflammation; mastication exacerbated the pain. She denied having experienced bleeding, rash, or fever. At the age of 1 year, the patient had a developmental hip dislocation that required surgical treatment.

Physical examination revealed a generalized tan pigmentation of the skin, micrognathia, microcephaly, the absence of the fifth finger on both hands, and growth retardation (height, 138 cm; weight, 26 kg; below the third percentile). An oral examination revealed prolonged retention of deciduous teeth and noneruption of permanent teeth, patchy brown discoloration of the tongue (Figure 1), and brown gums with smooth surface texture and generalized inflammation (Figure 2). Laboratory studies revealed a hemoglobin level of 8 g/dL (normal range, 11.0-16.0 g/dL [to convert to grams per liter, multiply by 10.0]), a mean corpuscular volume of 100 μm3 or more (to convert to femtoliters, multiply by 1.0), a neutrophil count of 1100/μL (normal range, 2000-8030/μL [to convert to ×109/L, multiply by 0.001]), and a platelet count of 35 000 × 103/μL (normal range, 150 000-300 000 × 103/μL [to convert to ×109/L, multiply by 1.0]). A panoramic dental radiographic scan shows the noneruption of permanent teeth (Figure 3). The bone marrow biopsy confirmed marrow hypoplasia.

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Figure 1. Photograph revealing small pigmented areas of the tongue of a 16-year-old girl.

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Figure 2. Photograph showing brown abnormalities of the gums of a 16-year-old girl.

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Figure 3. Panoramic dental radiographic scan showing the unerupted teeth of a 16-year-old girl.