tempi
play

TEMPI Described 2011 Six patients with five shared findings - PDF document

Aug 23, 2023 •266 likes •352 views

New Diagnostic Considerations In Clinical Dermatology William D. James MD Paul R Gross Professor University of Pennsylvania I do not have any relevant relationships with industry TEMPI Described 2011 Six patients with five shared

  1. New Diagnostic Considerations In Clinical Dermatology William D. James MD Paul R Gross Professor University of Pennsylvania I do not have any relevant relationships with industry TEMPI • Described 2011 • Six patients with five shared findings – Telangiectasias – Elevated erythropoetin levels and erythrocytosis – Monoclonal IgG kappa gammopathy – Perinephric fluid accumulations – Intrapulmonary shunting NEJM 2011; 365: 475 TEMPI • Telangiectasias – Face, trunk and arms • Elevated erythropoetin levels and erythrocytosis – Reaches levels of greater than 5000 and hematocrit at presentation 58 to 73 • Monoclonal IgG gammopathy – MGUS (Bone marrow <10% plasma cells) – Smoldering myeloma NEJM 2011; 365: 475

  2. TEMPI • Perinephric fluid accumulations – Clear serous fluid • Intrapulmonary shunting – Hypoxemia • Dramatic responses to bortezomid, a proteasome inhibitor targeting the abnormal plasma cells NEJM 2012; 366: 1843 NEJM 2012; 367; 778 AESOP A denopathy and E xtensive S kin patch O verlying a P lasmacytoma AESOP Syndrome • Described in 2003 • 11 patients to date • Extensive red patch or plaque on the chest which overlies a plasmacytoma of the bone • Histology is of dermal mucin & reactive angiomatosis • Leads to the discovery of this condition & effective radiotherapy in some cases JAAD 2006; 55:909

  3. Coxsackievirus A6 • Between 11/11 to 2/12: 63 cases in U.S. – Alabama: 38; California: 7; Connecticut: 1; Nevada: 17 • 24% adults – 53% of adults had sick pediatric contact • 34/63 cases tested positive by PCR for A6 – 74% if those tested were positive MMWR 2012; 61: 213 Coxsackievirus A6 • Fever 76% • Eruption – Hand, foot, mouth 67% – Extremities 46% – Face 41% – Buttocks 35% – Trunk 19% – Nail shedding 4%

  4. Coxsackievirus A6 • 19% cases hospitalized – Dehydration – Severe pain • This year first outbreak of A6 in US • Prior outbreaks since 2008 in Taiwan, Finland, Japan, Singapore • U.S. strains closely related, but not from direct importation Hand-Foot-Mouth • Common viral illness • Children, < 5yo • Rare in immunocompetent adults • Most common cause is coxsackievirus A16 in US • Coxsackie A5, A7, A9, A10, B2, B5 • Enterovirus 71 • Transmitted via oral-oral, oral-fecal route Necrolytic Acral Erythema Hepatitis C • Described in 1996 • About 75 patients reported • Nearly equal men and women • Average age about 40 • All hepatitis C positive

  5. Necrolytic Acral Erythema Hepatitis C • Tender well-defined, velvety or scaly surfaced dusky red plaques • Occasional blisters and erosions • Usually involves the dorsal feet, but legs and hands also affected • May respond to interferon, ribaviron, or zinc treatment JAAD 2010; May 10: Epub Br J Dermatol 2010 Apr 23: Epub Necrolytic Acral Erythema • 300 patients with hepatitis C were screened • 5 patients (1.7% prevalence) had clinical and histologic findings of NAE • All were African-American men, over 40 with high viral load of HCV genotype 1 • Mean zinc levels in blood and skin are low JAAD feb (epub) Br J Dermatol 2010; 163: 476 Palisaded Neutrophilic and Granulomatous Dermatitis • Symmetrically distributed umbillicated, superficially eroded papules and nodules • Favor extensor knees, elbows, extensor digits, usually painful • Described in 1965, but majority of cases published in last ten years JAAD 2008; 58: 661

  6. PNGD • Biopsies of early lesions reveal leukocytoclastic vasculitis and collagen degeneration • Later reveal palisaded granulomas with dermal fibrosis and scant neutrophilic debris JAAD 2005; 53: 191 Palisaded Neutrophilic and Granulomatous Dermatitis • Occurs in patients with rheumatoid arthritis, systemic vasculitis, lupus erythematosus • Follows the course of the underlying disease • In a group of 215 patients with rheumatoid arthritis 14 (6.5%) had PNDG • Treat underlying disease Int J Dermatol 2008; 47: 894 Rheumatoid Neutrophilic Dermatosis • Chronic erythematous plaques on the trunk and fibrotic nodules on the extremities • Long standing rheumatoid arthritis • Intact neutrophils between collagen bundles • No vasculitis or leukocytoclasis • Two cases in 215 patients with RA • Treatement with steroids, dapsone or etanercept JAAD 2005; 52: 916 Int J Dermatol 2008;47:894

  7. Newer Variants of Sweet’s Syndrome Neutrophilic Dermatosis of the Dorsal Hands • Localized variant of Sweet syndrome • 27 patients: 23 women & 4 men • Dense neutrophilic infiltrate with leukocytoclasis and secondary vascular inflammation • Fever, leukocytosis, high ESR • Leukemia, lung cancer, inflammatory bowel disease • Prednisone, topical steroids, dapsone, methotrexate and SSKI effective Arch Dermatol 2006; 142: 361, 400 Br J Dermatol 2011; 36: 688 Chronic Lymphocytic Sweet Syndrome • 7 patients with recurring Sweet-like clinical syndrome • Biopsy showed myeloperoxidse (+) lymphocytic infiltrates • Long term follow up of 2-8 years revealed development of myelodysplasia of the bone marrow & typical neutrophilic Sweet Syndrome • Responds to prednisone, thalidomide Arch Dermatol 2006; 141:1170

  8. Histiocytoid Sweet Syndrome • Histiocytes may be abundant in the infiltrate of typical Sweet Syndrome • 41 patients, myeloperoxidase (+) histiocytes found in lesions typical of Sweet Syndrome • Responded to prednisone & did not recur Arch Dermatol 2005; 141:834 Arth Rheum 2008; 59: 1832

More recommend