Internal Medicine

0521779407-13 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:15

Liver Transplantation Localized Scleroderma 915

➣recurrence of hepatitis C (100%)

➣biliary complications (10–25%)

➣opportunistic infections

Prognosis

■5-year graft and patient survival 80–85%

LOCALIZED SCLERODERMA

RAJANI KATTA, MD

history & physical

Signs & Symptoms

■Limited to skin and soft tissue involvement

■May be asymptomatic, itchy, or painful

■Several clinical variants

➣Localized morphea – single or few lesions, typically on trunk

➣Lesions of morphea may begin as red or violaceous patches

➣Progression to sclerotic, or firm, hypopigmented plaques

➣In active sclerotic lesions, red or violaceous border may be visible

■Generalized morphea – widespread, multiple plaques

■Linear scleroderma – linear unilateral plaques, usually on face or

extremities

➣Coup de saber – linear scleroderma on forehead; clinical appear-

ance similar to “blow from a sword”

➣Facial hemiatrophy (Parry-Romberg syndrome) is a variant asso-

ciated with atrophy of underlying muscle, bone and even brain

■Morphea profunda – subcutaneous bound-down plaques; sclerosis

may even affect fascia and muscle

tests

Laboratory

■Frequently positive anti-nuclear antibodies, including anti-single

stranded DNA and anti-histone antibody may be reflective of dis-

ease activity and may be associated with a poorer prognosis, but in

most cases do not alter management

Skin Biopsy

■Histology varies according to stage of disease

➣Lymphocytic inflammation in dermis

➣Sclerosis of collagen