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Robin Henry Dretler MD, FIDSA

3/16/2012

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CC: Painful leg woundHPI: 68 yom with chronic DVT in 1974 and 1982 on

chronic warfarin x 30 years.

Developed blistering, sloughing Left leg ulcers in1/2010

Seen DM and referred to ID/Wound Care

PMH: DVT 74, 84

Chronic Benign TremorNo HTN, DM, Followed by Rheumatology butno dx of vasculuitis

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FH: No vasculitis, clotting disorders

SH: No tob, alcohol or drugsROS: Denies fevers, chills, sweats, SOB, pleuritic sxs,

pain other sites, trauma, weight changes, arthralgia

PE: WNWD Slender, Tremulous M in NAD

VSS

HEENT: wnl

Neck: no TMG, no nodes

Lungs: clear, no ax nodes

Cor: RRR

Abd: no HSM, mass

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Left Leg ulceration

Draining, tender, weeping

Positive Nicolsky signNo edema

Palpable pulses

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9/2010 Evaluated for arterial insufficiency due to pain

Adequate

Venous insufficiency confirmed Profore wraps twice weekly with calcium alginate

Modest improvement

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Referred for Vein closure

Successful closureStill pain and very friableskin with Nicolsky sign

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Recurrent painful, ulcerated,

sloughing superficial skin

Lymphedema pumps added

Profores 3 x weekly

Contact layer to protect skin

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New Blister on

Forefoot, Elbow,

Scalp

Unable to tolerate pumps due

to painSoaking profores

Added Dapsone

Biopsy scalp blister

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Biopsy: Cicatricial

Pemphigoid

Dermatology Consult

Rheumatology Consult

Dapsone

Prednisone

NEJM CPC

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Pemphigus (Greek: pemphix-

bubble) Rare, chronic, potentially fatal

Autoimmune

Vesicobullous disease Blisters of skin and mucous membranes

Occur by acantholysis (loss of epidermal celladhesion)

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3 Types Pemphigus vulgaris

Pemphigus foliaceous

Paraneoplastic pemphigus

All uncommon, difficult to treat or cure

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DDX: Porphyrea cutanea tarda

Dermatitis herpetiformis

Erythema multeforme Toxic Epidermal necrolysis

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Pathogenesis and Clinical PatternAutoimmune antibodies vs. desmoglein (Dsg 1,2,3)

adhesion molecule

Intraepidermal blistering Usually mucosal with less often cutaneous lesions

Initially and usually oral, but may be entire alimentarytract, very painful

Cutaneous lesions also blistering, variable sized,characteristic Nicolsky sign

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Diagnosis Biopsy

Elisa Dsg 1 and 3 may correlate loosely with activity

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Treatment: 10 % go into remission

90% recurrent with year of suppression and risk of

fatal complications of immune suppression Dapsone 100mg

Prednisone 1 mg/kg/day

Cyclophosphamide 2-3 mg/kg/d

Mycophenolate 2-3 g/d

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Dapsone 100 BIDPrednisone 20 BID

Cyclosporine 150

BID

Monitor creatinine

Unable to taper prednisone

IVIG 400 mg/kg x 5 days

Only proven agent for steroidreduction, but still mayrelapse

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ClearedDapsone 100 mg BID Prednisone 10 mg daily