Recommended Link:

Scleroderma

Images

X-ray of both hands AP view

chest X-ray PA view


Clinical History:

H/o dyspnea for one year in a patient with known history of Scleroderma.


Findings:

1. X-ray of both hands: demonstrate resorption of all the terminal tufts of right hand and thumb,index and middle finger of left hand.
2. X-ray chest reveals fine reticul- nodular pattern in both the lungs suggesting Interstitial lung disease.


Diagnosis:

Scleroderma


Discussion:

Scleroderma is derived from the Greek words skleros (hard or indurated) and derma (skin)
The American College of Rheumatology (ACR) criteria for the classification of systemic sclerosis require one major criterion or two minor criteria, as follows:

•Major criterion: Proximal scleroderma is characterized by symmetric thickening, tightening, and induration of the skin of the fingers and the skin that is proximal to the metacarpophalangeal or metatarsophalangeal joints. These changes may affect the entire extremity, face, neck, and trunk (thorax and abdomen; see Images 1-2).
•Minor criteria
◦Sclerodactyly is characterized by thickening, induration, and tightening of the skin, limited to only the fingers.
◦Digital pitting scars or a loss of substance from the finger pad: As a result of ischemia, depressed areas of the fingertips or a loss of digital pad tissue occurs.
◦Bibasilar pulmonary fibrosis includes a bilateral reticular pattern of linear or lineonodular densities most pronounced in basilar portions of the lungs on standard chest roentgenography. These densities may assume the appearance of diffuse mottling or a honeycomb lung and are not attributable to primary lung disease.
Other entities that may also have Acro-Osteolysis include: Psoriatic arthritis, Reiter's disease, frost bite and Leprosy.


References / Suggested Reading:

References
1. MeSH Scleroderma,+Localized
2. MeSH Systemic+Scleroderma
3. a b http://content.nejm.org/cgi/content/full/360/19/1989 Gabrielli A, Avvedimento EV, Krieg T.] Scleroderma. N Engl J Med. 2009 May 7;360(19):1989-2003. PMID 19420368
4. Klippel, John H.. Primer On the Rheumatic Diseases 11ED. Atlanta, GA: Arthritis Foundation. ISBN 1-912423-16-2.
5. Harrison's Principles of Internal Medicine, 16th ed., Ch. 303, Systemic sclerosis (scleroderma) and related disorders, Bruce C. Gilliand
6.James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. Page 169-172. ISBN 0721629210.
7. a b c [1] The Free Dictionary, Medical terms, Scleroderma
8. Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum. 1980; 23:581-90.
9. Barnett A, Miller M, Littlejohn G. The Diagnosis and Classification of Scleroderma. Postgraduate Medical Journal. 1988; 64:121-125.
10. Fleischmajer,R., Sapadin, AN.Treatment of Scleroderma. Arch Dermatol. 2002; 138:99 - 104
11.Leighton, C. Drug Treatment of Scleroderma. Drugs. 2001; 61(3): 419-27.
12. [2] International Scleroderma Network, Lung, Oxygen therapy
13. A Brief History of Scleroderma, http://www.scleroderma.org/medical/other_articles/Coyle_2001_4.shtm


Author

Shalini Sharma,MD

Radiology Resident

NIMS Hyderabad

jdogra's picture
Offline
Joined: 05/23/2007
Great Case

Shalini:
Thank you for your submission.

Dr. Vikram Singh Dogra

Professor of Radiology, Urology & BME
Associate Chair for Education and Research.
Department of Imaging Sciences
University of Rochester School of Medicine