Multiple cutaneous myxomas. Report of a case without other elements of Carney's complex.
An 11-year-old hispanic female was referred for evaluation of multiple asymptomatic papules which had slowly developed since age two. The patient had no significant medical history and no family members, including an older sibling, were similarly affected. Physical examination revealed multiple 2-6 mm flesh-colored to erythematous papules over the posterior scalp, trunk, and extremities, predominantly in a midline distribution. No lentigines, blue nevi, or evidence of endocrine ab-normalities were noted. Echocardiogram was normal with no evidence of myxoma. Six cutaneous biopsies showed myxomas, as evidenced by well-circumscribed dermal tumors composed of stellate, dendritic cells embedded in a mucinous stroma. Special stains and electron microscopy confirmed the H&E finding. Immunoperoxidase staining for Factor XIIIa was positive in the dendritic tumor cells, suggesting a relationship with dermal dendrocytes. This patient represents a case of multiple cutaneous myxomas without other components of Carney's complex of myxomas, spotty pigmentation, endocrine overactivity, and a variety of uncommon tumors. A long-term follow-up is indicated since some of the above cited elements of the syndrome could appear over time.
Murphy
C M
CM
University of Texas Medical Branch, Galveston 77555-0783, USA.
Grau-Massanés
M
M
Sánchez
R L
RL
D001415:Back / Q000473:pathology; D002648:Child; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D008854:Microscopy, Electron; D009232:Myxoma / Q000473:pathology* / Q000648:ultrastructure; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D012155:Reticulin / Q000032:analysis; D012535:Scalp / Q000473:pathology; D012878:Skin Neoplasms / Q000473:pathology* / Q000648:ultrastructure; D013194:Staining and Labeling; D011503:Transglutaminases / Q000276:immunology
D012155:Reticulin; D011503:Transglutaminases