Robin Henry Dretler MD, FIDSA 3/16/2012

CC: Painful leg wound

HPI: 68 yom with chronic DVT in 1974 and 1982 on chronic warfarin x 30 years. Developed blistering, sloughing Left leg ulcers in 1/2010 Seen DM and referred to ID/Wound Care PMH: DVT 74, 84 Chronic Benign Tremor No HTN, DM, Followed by Rheumatology but no dx of vasculuitis

FH: No vasculitis, clotting disorders SH: No tob, alcohol or drugs

ROS: 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

Left Leg ulceration

Draining, tender, weeping Positive Nicolsky sign No edema Palpable pulses

9/2010
Evaluated for arterial insufficiency due to pain
Adequate Venous insufficiency confirmed Profore wraps twice weekly with calcium alginate Modest improvement

Referred for Vein closure Successful closure Still pain and very friable skin with Nicolsky sign

Recurrent painful, ulcerated, sloughing superficial skin

Lymphedema pumps added Profores 3 x weekly Contact layer to protect skin

New Blister on Forefoot, Elbow, Scalp

Unable to tolerate pumps due to pain Soaking profores Added Dapsone Biopsy scalp blister

Biopsy: Cicatricial Pemphigoid

Dermatology Consult Rheumatology Consult Dapsone Prednisone NEJM CPC

Pemphigus (Greek: pemphixbubble)

Rare, chronic, potentially fatal
Autoimmune Vesicobullous disease Blisters of skin and mucous membranes Occur by acantholysis (loss of epidermal cell

adhesion)

3 Types
Pemphigus vulgaris
Pemphigus foliaceous Paraneoplastic pemphigus

All uncommon, difficult to treat or cure

DDX:
Porphyrea cutanea tarda
Dermatitis herpetiformis Erythema multeforme Toxic Epidermal necrolysis

Pathogenesis and Clinical Pattern

Autoimmune 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 alimentary tract, very painful Cutaneous lesions also blistering, variable sized, characteristic Nicolsky sign

Diagnosis
Biopsy
Elisa Dsg 1 and 3 may correlate loosely with activity

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

Dapsone 100 BID Prednisone 20 BID Cyclosporine 150 BID

Monitor creatinine Unable to taper prednisone

IVIG 400 mg/kg x 5 days Only proven agent for steroid reduction, but still may relapse

Cleared
Dapsone 100 mg BID

Prednisone 10 mg daily