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Picture of the Month Quiz

Image of figure 1
Image of figure 2

Figure 1.

Figure 2.

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 age 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, absence of the fifth finger on both hands, and growth retardation (height, 138 cm; weight, 26 kg; below the third percentile). 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 macrocytic anemia (hemoglobin level, 8 g/dL; to convert to grams per liter, multiply by 10.0) and decreased absolute neutrophil count and platelet count. Bone marrow biopsy confirmed marrow hypoplasia.

For a complete discussion of this month's case, read next month's Picture of the Month—Quiz Case. For a complete discussion of last month's case, read this month's Picture of the Month—Quiz Case.