Crest, or limited form of systemic sclerosis

J. Becker, Norman Todd Ilowite, F. Bierman, E. Katz, M. Gandhi, I. R. Shenker, B. Gauthier, A. Battista

Research output: Contribution to journalArticle

Abstract

A 12-year-old girl developed a white lump on the third finger of her right hand two years earlier. The lesion recurred after excision and a similar lump developed on the left knee and was also excised. Significant findings on physical examination included dilated capillaries in the nail beds and raised scaly violaceous lesions over the dorsal surface of the metacarpophalangeal joints. The skin on the fingers was hard (sclerodactyly) and there were some telangiectasias around the face and mouth and on the lips. Nail bed capillaries showed 'drop out' and dilatation, findings suggestive of dermatomyositis and scleroderma. The sclerodactyly pointed toward scleroderma. The absence of significant abnormalities in the lungs or the kidneys suggested the limited form of systemic sclerosis otherwise known by the old term CREST. There is no definitively effective treatment for scleroderma. Penicillamine may be effective. Nifedipine may be used for severe Raynaud's phenomenon. Calcinosis may respond to low dose warfarin therapy. Emollients for the skin are also used. Patients with this disease are at risk for GI problems, skin problems and Raynaud's phenomenon and pulmonary hypertension later in life.

Original languageEnglish (US)
Pages (from-to)113-115
Number of pages3
JournalChildren's Hospital Quarterly
Volume6
Issue number2
StatePublished - 1994
Externally publishedYes

Fingerprint

Systemic Scleroderma
Raynaud Disease
Nails
Skin
Fingers
Emollients
Metacarpophalangeal Joint
Calcinosis
Telangiectasis
Penicillamine
Dermatomyositis
Warfarin
Nifedipine
Lip
Pulmonary Hypertension
Physical Examination
Mouth
Dilatation
Knee
Hand

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Becker, J., Ilowite, N. T., Bierman, F., Katz, E., Gandhi, M., Shenker, I. R., ... Battista, A. (1994). Crest, or limited form of systemic sclerosis. Children's Hospital Quarterly, 6(2), 113-115.

Crest, or limited form of systemic sclerosis. / Becker, J.; Ilowite, Norman Todd; Bierman, F.; Katz, E.; Gandhi, M.; Shenker, I. R.; Gauthier, B.; Battista, A.

In: Children's Hospital Quarterly, Vol. 6, No. 2, 1994, p. 113-115.

Research output: Contribution to journalArticle

Becker, J, Ilowite, NT, Bierman, F, Katz, E, Gandhi, M, Shenker, IR, Gauthier, B & Battista, A 1994, 'Crest, or limited form of systemic sclerosis', Children's Hospital Quarterly, vol. 6, no. 2, pp. 113-115.
Becker J, Ilowite NT, Bierman F, Katz E, Gandhi M, Shenker IR et al. Crest, or limited form of systemic sclerosis. Children's Hospital Quarterly. 1994;6(2):113-115.
Becker, J. ; Ilowite, Norman Todd ; Bierman, F. ; Katz, E. ; Gandhi, M. ; Shenker, I. R. ; Gauthier, B. ; Battista, A. / Crest, or limited form of systemic sclerosis. In: Children's Hospital Quarterly. 1994 ; Vol. 6, No. 2. pp. 113-115.
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