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Scleromyxedema. UNC Morning Report December 16, 2009 Christine Williams, MD. SCLEROMYXEDEMA. Also called lichen myxedematosus scleromyxedema of Groton papular mucinosis Epidemiology: quite rare, largest case series 26 Average age 55 3:1 Female to male ratio.

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Scleromyxedema

Scleromyxedema

UNC Morning Report

December 16, 2009

Christine Williams, MD


Scleromyxedema1
SCLEROMYXEDEMA

  • Also called

    • lichen myxedematosus

    • scleromyxedema of Groton

    • papular mucinosis

  • Epidemiology: quite rare, largest case series 26

  • Average age 55

  • 3:1 Female to male ratio


Physical findings scleromyxedema
PHYSICAL FINDINGS: Scleromyxedema

  • Flesh colored to yellow/red, papular skin eruption

  • Indurated with a cobblestone feel

  • Distribution includes head, posterior auricular area, neck, arms and trunk

  • Midportion of back can be involved

    • NOT involved in scleroderma

  • Sclerodactyly can be present but papular




Associated organ involvement
Associated Organ Involvement

  • Raynaud’s phenomenon

  • Esophageal dysmotility

  • Myopathy

  • Pulmonary hypertension has been reported

  • Neurologic symptoms

    • Encephalopathy, seizures, coma and psychosis


Laboratory data scleromyxedema
LABORATORY DATA: Scleromyxedema

  • Monoclonal gammopathy with lambda chains

    • Level of paraprotein does not decrease after effective treatment

    • Pathogenesis unclear

  • Mucinous deposition throughout the dermis

    • Thick collagen bundles

    • Increased fibroblast-like cells

    • Inflammatory infiltrate

  • Autoimmune markers typically negative


Treatment scleromyxedema
TREATMENT: Scleromyxedema

  • Variable immunosuppressants have been tried

    • IVIG 2g/kg monthly for 2-3 months then maintenance every 10-12 weeks

    • Melphalan

    • Cyclophosphamide

    • Cyclosporine

    • Thalidomide

  • Stem cell transplant has been used


Differential immunologic
DIFFERENTIAL: IMMUNOLOGIC

  • Scleroderma

    • Obliterative vasculopathy, extensive collagen deposition and fibrosis

  • Eosinophilic fasciitis (Shulman’s syndrome)

  • GVHD

  • Lichen sclerosus et atrophicus

  • POEMS Syndrome

    • Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes

  • SLE, dermatomyositis overlap syndromes


Differential metabolic genetic
DIFFERENTIAL: METABOLIC/GENETIC

  • Porphyria cutanea tarda

  • Hypothyroidism (myxedema)

  • Phenylketonuria

  • Stiff skin syndrome

    • congenital facial dystrophy

  • Progeroid disorders

    • Progeria, acrogeria


Differential deposition
DIFFERENTIAL: DEPOSITION

  • Systemic amyloidosis

  • Nephrogenic systemic fibrosis

  • Scleredema adultorum

  • Lipodermatosclerosis


Differential toxic occupational
DIFFERENTIAL: TOXIC/OCCUPATIONAL

  • Polyvinyl chloride

  • Organic solvents

  • Silica

  • Epoxy resins

  • Bleomycin

  • Pentazocine

  • Carbidopa

  • Eosinophilia-myalgiasydnrome (L-tryptophan)

  • Toxic-oil syndrome (aniline-denatured rapeseed oil)

  • Post-radiation fibrosis


Scleroderma
SCLERODERMA

  • 1-2:100,000

  • Ages 30-50, more common in women

  • Diffuse cutaneous systemic sclerosis

  • Limited cutaneous systemic sclerosis

    • Calcinosis cutis

    • Raynaud’s phenomenon

    • Esophageal dysmotility

    • Sclerodactyly

    • Telangiectasia


Scleroderma1
SCLERODERMA

  • Early in disease, arthralgias and soft tissue swelling may be more prominent

  • Heartburn/Dysphagia

  • DOE

  • Pulmonary HTN

  • Diarrhea w/malabsorption

  • Mucocutaneous telangiectasias

  • Digital infarctions

  • Hyperpigmentation

  • Calcinosis cutis

  • 10% do not have skin induration


Scleroderma autoantibodiess
SCLERODERMA: Autoantibodiess

  • ANA screening (95% +)

  • Anti-PM-Scl

    • Increased risk of myositis

  • Anti-Scl 70 – DNA topoisomerase I (dcSSc)

    • Increased risk of interstitial lung disease

  • Anti-centromere (lcSSc)

  • Anti-U3-RNP

    • Increased risk of Pulmonary HTN and myositis

  • Anti-RNA polymerase III

  • Anti-B2 glycoprotein I

    • Increased risk of thromboembolism, macrovasculardz

  • Specific 99%

  • Not sensitive 20-50%

  • High titers of RF, anti-CCP, anti-U1 RNP, anti-dsDNA or anti-Smith uncommon. ANCA not associated with systemic sclerosis.


Nephrogenic systemic fibrosis
Nephrogenic Systemic Fibrosis

  • History of renal failure and HD

    • Gadolinium exposure , high epo doses are associated

  • Rapidly developing, days to weeks

  • Confluent fibrotic skin induration ‘peaud’orange’  cobblestone

    • Nodular plaques which become woody

      • Tender, pruritic, burning

    • Brawny hyperpigmentation

    • Flexion contractures

  • Stocking/glove distribution, face usually spared

  • Muscles, myocardium, lungs, kidneys and testes

  • Fibroblast proliferation, thick collagen bundles and mucin deposition (very similar) but can extend into adipose and muscle layers

  • MRI – increased T1 signal in muscles, fascial and muscular edema

  • Rx-sodium thiosulfate?, imatinibmesylate, PT, pain management



Eosinophilic fasciitis
Eosinophilic Fasciitis

  • Scleroderma-like painful skin lesionsof extremities (forearms/calves)

  • Hands and face usually spared

  • Hypergammaglobulinemia (polyclonal)

  • Eosinophilia

  • Dermal/hypodermal sclerosis w/fibrotic thickening of subcutaneous adipose septa, superficial fascia and perimysium

    • Epidermis spared

  • Flexion contractures and peripheral nerve compression can complicate

  • Synovitisand constitutional symptoms possible

  • 2:1 male: female ratio

  • Preceded by vigorous exercise or trauma in 50%

  • 10-15% have underlying hematologic d/o or malignancy

  • Associated with spirochetes

  • Similar to symptoms from

    • rapeseed oil (Toxic oil sydrome, Spain ‘81)

    • L-tryptophan (eosinophilia-myalgiasydrome, US ‘89)

  • Rx: Corticosteroids, PT



Scleredema scleredema a dultorum buschke
SCLEREDEMA(ScleredemaAdultorum/Buschke)

  • Deposition of collagen and mucin causing thickening of the dermis

  • Non-pitting, doughy or woody induration

  • Neck, back, interscapular region, face, chest

  • Associated with

    • Post-infectious (strep), occurs in children also [type 1]

    • Monclonalgammopathies [type 2]

      • Multiple myeloma IgG, IgA, MGUS, Waldenstrom’s

    • Poorly controlled DM [type 3]

  • Treatment - treat underlying disease

    • Immunosuppressants (steroids, MTX) w/o clear benefit

    • PUVA therapy, UVA-1 treatment



Proposed approach to differential
Proposed approach to differential

  • History for risk factors, exposure to gadolinium

  • Raynaud’s phenomenon

  • Skin distribution

  • Presence of neurologic disease

  • ANA

  • SPEP

  • Full-thickness skin biopsy


References
REFERENCES

  • Boin F, Hummers LK. Scleroderma-like fibrosing disorders. Rheum Dis Clin North Am. 2008 Feb;34(1):199-220; ix.

  • Rey JB, Luria RB. Treatment of scleromyxedema and the dermatoneuro syndrome with intravenous immunoglobulin. J Am Acad Dermotol. 2009 Jun;60(6):1027-41. Epub 2009 Feb 26.

  • Varge, J. Diagnosis and differential diagnosis of systemic sclerosis (scleroderma) in adults. UpToDate. Online. (utdol.com) Accessed 14 December 2009.


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