Internal Medicine

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

Localized Scleroderma Low-Oxygen-Affinity Hemoglobins 917

■Phototherapy and photochemotherapy

➣UVA1 therapy, bath PUVA, topical PUVA (psoralen+UVA pho-

totherapy)

➣Must be administered in specialized centers

➣Treatments administered 2–4 times/week over months

➣Risk of burning, secondary skin malignancies

■Methotrexate with or without systemic corticosteroids has been

reported to be useful in a limited number of patients

follow-up

During Treatment

■Re-evaluate skin lesions every 1–3 months during treatment to mon-

itor efficacy of treatment and to note side effects

complications and prognosis

Complications

■Cosmetic concerns; disfigurement resulting from atrophy and pig-

mentary changes

■Contractures and limited mobility if involvement extends over a joint

■In severe cases, muscle atrophy

Prognosis

■Good, with spontaneous remission of disease in most

■May remit in few months or years

■Atrophy and pigmentary changes (hypo- or hyper-) may persist

LOW-OXYGEN-AFFINITY HEMOGLOBINS

AKIKO SHIMAMURA, MD, PhD

history & physical

History

■Positive family history (autosomal dominant inheritance)

■Generally asymptomatic (normal tissue oxygen delivery)

Signs & Symptoms

■Cyanosis may be present

tests

Blood

■Sometimes associated with mild hemolytic anemia