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Clusters of Painful Blue Papules

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A woman in her 30s presented with multiple discrete and clustered blue papules on her face and extremities. The lesions had been present since childhood, but the size of the lesions and the intensity of pain associated with them had recently increased with even light touch causing paroxysms of pain. The pain associated with these papules worsened as the day progressed, and she could not tolerate elastic compression due to excessive pain on compression. Her medical and family histories were unremarkable. Physical examination revealed multiple discrete and agminated blue papules on the face, arms, hands, and thighs with a cobblestoned appearance (Figure 1). On palpation, the papules were exquisitely tender and partially compressible. The patient underwent duplex ultrasonography of representative papules on the thigh, and a skin biopsy specimen of a papule on the thigh was obtained for histopathologic evaluation.

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D. Glomuvenous malformation (GVM)

Histopathologic examination of the biopsy specimen revealed dilated, thin-walled vascular spaces lined by cuboidal glomus cells in the dermis and subcutis (Figure 2). The cells were monomorphous and lacked atypical features.

The patient underwent duplex ultrasonography of the thigh that showed normal superficial and deep venous systems without reflux, and a subsequent magnetic resonance angiogram excluded arteriovenous communications. After sclerotherapy, the papules did not resolve, and the patient reported increased pain of the treated areas; she elected to pursue surgical excision of 10 symptomatic papules on the thigh. Postoperatively and at last follow-up, she was pain free without evidence of recurrence.

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Article Information

Corresponding Author: Heron E. Rodriguez, MD, Division of Vascular Surgery, Northwestern University Feinberg School of Medicine, 676 N St Clair, Ste 650, Chicago, IL 60611 (herodrig@nmh.org).

Published Online: August 24, 2016. doi:10.1001/jamadermatol.2016.2746

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
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Barnes  L, Estes  SA.  Laser treatment of hereditary multiple glomus tumors.  J Dermatol Surg Oncol. 1986;12(9):912-915.PubMedGoogle ScholarCrossref
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Antony  FC, Cliff  S, Cowley  N.  Complete pain relief following treatment of a glomangiomyoma with the pulsed dye laser.  Clin Exp Dermatol. 2003;28(6):617-619.PubMedGoogle ScholarCrossref
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Parsi  K, Kossard  S.  Multiple hereditary glomangiomas.  Australas J Dermatol. 2002;43(1):43-47.PubMedGoogle ScholarCrossref
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Mounayer  C, Wassef  M, Enjolras  O, Boukobza  M, Mulliken  JB.  Facial “glomangiomas”.  J Am Acad Dermatol. 2001;45(2):239-245.PubMedGoogle ScholarCrossref
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