Uncommon presentation of pityriasis rosea after yellow fever inoculation.
نویسندگان
چکیده
Discussion | Poland syndrome, named after the British surgeon Alfred Poland who first described it in 1841, is an uncommon, sporadic, and very rarely inherited birth defect characterized by unilateral chest wall hypoplasia (often right-sided) and ipsilateral hand deformity (most often syn-brachydactyly and less often oligodactyly).1 Poland syndrome affects boys 2 to 3 times as often as girls, with an estimated incidence of 1 in 10 000 to 100 000 live births. Its exact cause remains unclear, but a prevailing theory is hypoplasia of the subclavian artery during the critical sixth week of gestation, which leads to a range of musculoskeletal malformations.1 Poland syndrome has been associated with hematopoietic malignancies such as leukemia and non-Hodgkin lymphoma, as well as other syndromes, including Möbius syndrome (characterized by congenital bilateral facial paralysis with inability to abduct the eyes) and Klippel-Feil syndrome (characterized by congenital fusion of any 2 of the 7 cervical vertebrae).1 In addition to these known associations, a literature review revealed a few isolated cases of Poland syndrome with various congenital dermatologic findings, including recessiveX-linked ichthyosis, congenitalhemangioma, andcaféau-lait spots.2-4 To our knowledge, our case is the first reportedcaseofPolandsyndromeassociatedwithmultipleCMN. ACMNisabenign,clonalproliferationofmelanocytes inthe epidermal, dermal, or subcutaneous tissue that is present at or shortlyafterbirth,withanestimatedprevalenceof0.5%to31.7%, depending on the study. They are usually solitary, but 3% are multiple, occasionally arranged in a cluster and rarely in a lineardistribution.Indeed,only2casesofBlaschkolinearCMNhave been reported thus far.5,6Ourpatientwould represent the third such case, and it occurs in a patient with Poland syndrome. Thedefinite risk of developingmelanoma inBlaschkolinear CMNremainsunclear becauseof its rarity.Our patient had no clinical sign of malignant transformation by age 11 years, as in the 2 previously reported cases at age 15 and 28 years. However, long-term close clinical monitoring might be warranted, as in giant CMN. In summary, we report herein a rare case of Blaschkolinear CMN in a teenager with Poland syndrome. The association between the 2 conditions seems to be coincidental, as in previously reported casesofPoland syndromewithother congenital dermatologic findings.
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ورودعنوان ژورنال:
- JAMA dermatology
دوره 150 9 شماره
صفحات -
تاریخ انتشار 2014