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

William D. James MD Paul R Gross Professor University of Pennsylvania

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New Diagnostic Considerations In Clinical Dermatology

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

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

Adenopathy and Extensive Skin patch Overlying a Plasmacytoma 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

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%

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

• 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

Necrolytic Acral Erythema Hepatitis C

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

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

Newer Variants of Sweet’s Syndrome

Neutrophilic Dermatosis

• f 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

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