Case history
Case history
A 4-year-old boy presents with a 2-month history of an enlarging scalp lesion. There is no history of trauma to the head or scalp. The patient is otherwise healthy. Physical examination shows a large parietal skull lesion on the right side of the scalp, which is hard and tender to touch. The rest of the physical examination is normal.
Other presentations
Presentation depends on the organ system(s) affected. Patients may present with bone pain and/or swelling, vertebra plana (i.e., spondylitis with reduction of the vertebral body to a thin disk), or soft-tissue mass (bone involvement); skin rashes (skin involvement); symptoms of diabetes insipidus, or growth or sexual maturation failure, headache, or neurologic signs (central nervous system involvement); hepatosplenomegaly, jaundice, ascites (liver/spleen involvement); cough, dyspnea, chest pain (lung involvement); orbital abnormalities (eye involvement); cytopenias (bone marrow involvement); ear pain or discharge, deafness, or perforated tympanic membrane (ear involvement); oral mucosal lesions (oral involvement); lymphadenopathy (lymph node involvement); thyroid enlargement (endocrine involvement); or bloody diarrhea (gastrointestinal involvement). Nonspecific symptoms include fever, anorexia, weight loss, and irritability.[2][4]
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