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Poland Syndrome Coexisting With Blaschkolinear Congenital Melanocytic Nevi

Thomas Lam, BA1; Yongxue Yao, MD, PhD1; Joanne Trockman, MD1
[+] Author Affiliations
1Department of Dermatology, Indiana University School of Medicine, Indianapolis
JAMA Dermatol. 2014;150(9):1019-1020. doi:10.1001/jamadermatol.2013.9687.
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Poland syndrome is an uncommon congenital anomaly characterized by unilateral chest wall and upper extremity hypoplasia.1 It is theorized to be due to subclavian artery hypoplasia during embryogenesis.1 There have been only a few isolated reports of Poland syndrome with congenital dermatoses.24 Adding to this association list, we report a case of Poland syndrome with multiple congenital melanocytic nevi (CMN). Additionally, the nevi are in a Blaschkolinear arrangement, which is also rare; we know of only 2 such cases reported previously.5,6

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Figure 1.
Clinical Images of Patient With Poland Syndrome and Blaschkolinear Congenital Melanocytic Nevi

A, Hypoplasia of the left chest wall and oligodactyly of the left hand. The 2 digits of left hand seen in the photograph were reconstructed from his toes. B, Blaschkolinear distribution of bluish-black thin papules and plaques on his left leg.

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Figure 2.
Lesional Biopsy Specimen Obtained From the Left Thigh

The photograph shows a junctional nevus with congenital features (hematoxylin-eosin, original magnification ×200).

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