Faculty Bibliography

Acrokeratoelastoidosis is a rare palmoplantar keratoderma. We report the case of a 32-year old man with multiple keratotic papules and plaques on the peripheral margins of his hands and feet. Histopathologic evaluation showed compact orthokeratosis; Verhoeff van Gieson stain showed thin and fragmented elastic fibers. The differential diagnosis includes focal acral hyperkeratosis, which has a similar presentation but with normal elastic fibers. Treatment of acrokeratoelastoidosis is often disappointing, with minimal response to topical glucocorticoids, keratolytics, retinoids, cryotherapy, and lasers.

Purpose: The Penumbra RubyTM Coil system is a generation of novel large-volume platinum detachable coils designed for arterial and venous embolization in the peripheral vasculature. Recent literature describes the impact of packing density on the stability of recanalization.1 Herein, initial data from the multicenter Aneurysm Coiling Efficiency (ACE) registry aims to validate the system's safety and efficacy in relation to high packing density and sustainable occlusion in the periphery. Material and Methods: Patients were treated at 13 centers using the Ruby Coil system between March 2012 and January 2015. Data compiled from the first 68 cases included 7 splenic, 11 renal, 3 mesenteric, 1 iliac, and 1 hepatic artery aneurysms; 7 AVMs; 6 fistulae; 4 varices; and 28 vessel sacrifices. Results: For aneurysms only, median number of coils placed was 6 and mean packing density was 28% (N=40). Post-treatment distribution of Raymond occlusion scores were Class I (91.3%), II (4.3%), and III (4.3%). At 6 months, scores were Class I (92.9%) and II (7.1%). For vessel sacrifices, all 28 had successful coil embolization. Median number of coils placed was 2.5, and mean fluoroscopy time was 21 min. Thirteen patients with 6-month follow-up demonstrated stable or ongoing occlusion. For all 68 patients, no procedural SAEs were recorded. Follow-up is ongoing. Conclusion: Using Ruby resulted in a high mean packing density and complete post-procedure occlusion, which remained stable at the 6-month follow-up. 1JVIR 2013;24:1798

Purpose: The Penumbra RubyTM Coil system (Ruby) is a new generation of larger (.020) platinum detachable coils designed for peripheral arterial and venous embolization. Recent literature has shown the importance of packing density on occlusion stability and recanalization rates.1 Reported herein are preliminary results from the multicenter Aneurysm Coiling Efficiency (ACE) registry on the packing density and resulting long term outcomes of patients with peripheral embolizations by Ruby. Materials and Methods: Between Mar 2012 and Sep 2014, data from 62 Ruby cases at 12 centers were collected: 11 splenic artery aneurysms, 9 renal artery aneurysms, 1 hepatic artery aneurysm, 2 mesenteric artery aneurysms, 7 AVMs, 6 fistulae, 3 varices, and 23 vessel sacrifices. Results: Mean Ruby placed per aneurysm was 9 with a mean packing density of 26% in the aneurysms/malformations cases (N=39). Aneurysm volumes at the splenic and renal arteries were 110 to 21,500 mm³ and neck width was from 4 to 16 mm. Mean fluoroscopy time was 29 min. Among the 21 aneurysm cases with post-treatment occlusion data, 100% achieved Raymond Scale Class I occlusion. Of the 10 patients with 6-month follow up data, 100% displayed complete Class I occlusion with 0% recanalization. The remaining 11 patients are expected to complete their 6-month follow up at time of presentation.Of the 23 peripheral vessel sacrifices, 100% had successful coil embolization. Mean number of Ruby placed was 4 with a mean fluoroscopy time of 22 min. Out of the 11 patients with 6-month follow up data, 100% displayed stable occlusion. No procedural SAEs were recorded in any of the 62 cases. Conclusion: Using Ruby in the peripheral vasculature resulted in a high packing density and complete occlusion which remained stable for 6 months. Consistent with the published literature that 24% packing density is optimal to prevent recanalization, these results confirmed an association between high packing density and low recurrence of recanalization.1 Upon further validation, the use of packing density as an index for stable long-term outcome in the treatment of peripheral malformations should be considered, perhaps even emphasized

A 45-year-old man with hereditary hemorrhagic telangiectasia (HHT) presented with numerous mucocutaneous telangiectases, recurrent nosebleeds, and several first degree relatives with similar symptoms. HHT has been linked to mutations in the genes endoglin and activin receptor-like kinase-1 (ALK-1), which are located on chromosome 9q33-34 and 12q13, respectively. The resultant proteins mediate binding and signaling of transforming growth factor-beta. The clinical features, molecular basis, and management of HHT are reviewed

A 26-year-old man presented with a history of intermittent erythematous plaques on his hands and legs. A peripheral blood eosinophilia was noted. Histopathologic examination showed numerous eosinophils and characteristic flame figures. The clinical presentation and histopathologic alterations are consistent with the diagnosis of Wells' syndrome, which is also known as eosinophilic cellulitis. Wells' syndrome is a rare condition of unclear etiology. We discuss its diagnosis and possible association with other conditions that manifest peripheral eosinophilia

A 45-year-old man presented with lesions clinically and histologically consistent with telangiectasis macularis eruptiva perstans (TMEP). TMEP is a rare form of mastocytosis, which is localized to the skin, although systemic involvement has been reported. A mutation in the c-kit proto-oncogene is thought to be involved in mast cell hyperplasia in some patients with mastocytosis. Treatment depends on the presence of systemic involvement or clinical symptoms. In case reports, therapeutic benefit has been reported in TMEP with the use of the 585-flashlamp pumped dye lasers and of electron beam radiation