MULTIPLE CUTANEOUS MYXOMAS - REPORT OF A CASE WITHOUT OTHER ELEMENTS OF CARNEYS COMPLEX

An 11-year-old hispanic female was referred for evaluation of multiple asymptomatic papules which had slowly developed since age two. The pa tient had no significant medical history and no family members, includ ing an older sibling, were similarly affected: Physical examination re vealed multiple 2-6 mm flesh-colored to erythematous papules over the posterior scalp, trunk, and extremities, predominantly in a midline di stribution. No lentigines, blue nevi, or evidence of endocrine abnorma lities were noted. Echocardiogram was normal with no evidence of myxom a. Six cutaneous biopsies showed myxomas, as evidenced by well-circums cribed dermal tumors composed of stellate, dendritic cells embedded in a mucinous stroma. Special stains and electron microscopy confirmed t he H&E findings. Immunoperoxidase staining for Factor XIIIa was positi ve in the dendritic tumor cells, suggesting a relationship with dermal dendrocytes. This patient represents a case of multiple cutaneous myx omas without other components of Carney's complex of myxomas, spotty p igmentation, endocrine overactivity, and a variety of uncommon tumors. A long-term followup is indicated since some of the above cited eleme nts of the syndrome could appear over time.