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Correspondence |

Cutaneous Polyarteritis Nodosa in a Patient With Fabry Disease

Hsiang-Jung Chen, MD; Chih-Chao Yang, MD; Cheng-Hsiang Hsiao, MD; Chia-Yu Chu, MD
Arch Dermatol. 2008;144(1):122-123. doi:10.1001/archdermatol.2007.20.
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Fabry disease is an X-linked lysosomal storage disease due to deficiency of the lysosomal hydrolase α-galactosidase A. We report herein a case of cutaneous polyarteritis nodosa (PAN) in a patient with Fabry disease manifesting as multiple tender nodules.

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PubMed Link to Article[[XSLOpenURL/10.2165/00128071-200304030-00005]]
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PubMed Link to Article[[XSLOpenURL/10.7326/0003-4819-138-4-200302180-00014]]
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PubMed Link to Article[[XSLOpenURL/10.7326/0003-4819-86-3-315]]
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PubMed Link to Article[[XSLOpenURL/10.1007/BF00714291]]
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Figure 1.

Clinical appearance of the patient’s lesions. A, Many angiokeratoma corporis diffusum lesions are apparent on the back and buttocks. B, Several subcutaneous tender nodules with overlying hyperpigmentation are present on the right thigh.

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Figure 2.

Specimens taken from the patient’s lesions. A, Histopathologic specimen shows inflammation of medium-sized arteries in the deep dermis and subcutis (orcein, original magnification ×100). B, Direct immunofluorescence reveals granular deposition of C3 (original magnification ×200). C, Electron microscopy shows lamellar structures in the cytoplasm of the endothelial cells in the affected vessels (original magnification ×7000).

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