A definition of Juvenile Scleroderma in simple, easy to understand language
 

Raynaud Phenomenon And Digital Ulcers In Juvenile Scleroderma
Written by L. Nandini Moorthy, M.D., M.S.
Assistant Professor of Pediatric Rheumatology
Department of Pediatrics
UMDNJ-Robert Wood Johnson Medical School

and
James R. Seibold, M.D.
Professor & Director
UMDNJ Scleroderma Program
New Brunswick, NJ
2003

In 1862, Maurice Raynaud described a three-stage series of color changes of the fingers and toes occurring in response to environmental cold and emotional stress. Although not all persons with Raynaud phenomenon have all three color changes; the classic sequence is one of blanching or whitening followed by a dusky blue-purple phase and in some individuals a third phase of exaggerated redness as blood flow returns to the fingers. Associated symptoms include pain, numbness and burning.

Blood flow to the fingers and toes is ordinarily around 40-50 fold that required for nutrition and oxygen supply. It is ordinary to reduce blood flow to the hands when in cold environments and to increase hand blood flow when warm. In this way, the body can carefully regulate the core body temperature for optimum organ function. Due to the reduction of blood flow in the extremities of individuals with Raynaud phenomenon, they are unusually sensitive to even minor temperature changes and have difficulties with simple tasks like holding iced drinks or reaching into a freezer.

Raynaud himself recognized that the phenomenon could occur as an isolated clinical problem in persons who were otherwise healthy but that it was also a sign of systemic illness. Modern physicians view Raynaud phenomenon in much the same way. An estimated 10% or more of the healthy population including both men and women experience Raynaud phenomenon. In these persons said to have primary Raynaud phenomenon, the blood vessels are structurally normal but seem to have a heightened response to environmental stress. In this “haystack” of a very common complaint lies the “needle” of secondary Raynaud phenomenon. Secondary Raynaud occurs when the blood vessels have been damaged or narrowed. In this instance, normal levels of blood vessel closure in response to cold superimposed on the narrowing lead to blockage of blood flow to the finger tips. Prominent amongst the causes of secondary Raynaud phenomenon is scleroderma. Raynaud phenomenon is the most common presenting complaint in scleroderma and ultimately develops in nearly all patients, adult and children.

The pathogenesis of scleroderma remains poorly defined. The disease is named for its fibrotic features including the tightening and thickening of the skin. There is much evidence of immune system activation which may in part drive the fibrosis. It is nonetheless recognized that progressive scarring of the small artery is the most universal feature of scleroderma. When present in the fingers and toes, this leads to Raynaud phenomenon. Blood vessel injury and scarring are also the proximate cause of kidney, lung, heart and probably gastrointestinal involvement. Many researchers of scleroderma feel that a truly effective therapy for the global disease of scleroderma will be an agent or combination of agents that arrests and reverses the blood vessel injury.

Individual episodes of Raynaud phenomenon do not worsen the disease. Treatment of Raynaud phenomenon has the goals of 1) maximizing patient function 2) reducing symptoms and 3) preventing digital ulcers. Around 1/3 of persons with scleroderma will develop a finger tip ulcer at least once a year. Inadequate blood flow is the primary basis for the formation of ulcers although poor health of local skin tissue is also contributory. A patient with scleroderma who develops finger tip ulcers is well advised to treat their circulation and skin integrity with great care – much the way a diabetic would manage foot care.

If an ulcer occurs, infection and further tissue damage becomes the primary treatment agenda. Topical antibiotic creams or systemic antibiotics are necessary as well as specific guidance as to wound dressings. Your health care provider should be informed if the ulcer has redness around its margins; pain is increasing; there is drainage of pus; or the ulcer appears to be deepening or increasing in size.

The Table summarizes current options for treatment. Not all patients need medication. Keeping the central body warm with layered clothing; wearing hats to minimize heat loss through the scalp; careful planning of daily activities to avoid cold exposure are three simple recommendations that benefit all. In some ways, Raynaud severity is under the control of the patient. For example, if a medication is working, that individual may decide to risk more cold weather activities and thus risk more actual Raynaud attacks. Since quality of life is improved with medication, they are considered beneficial.

Not all patients respond to all or even any of the current recommended treatments. This does not mean that treatments shouldn’t be tried. We go by the simple rule that if the patient can’t tell if a therapy is working then it probably isn’t and something different should be attempted. None of the therapies should be expected to eliminate all attacks. Success can be defined as reduced number or severity of attacks, increased tolerance of cold, and/or reduction in the occurrence of digital ulcerations. Some patients do well by treating their Raynaud phenomenon in cold weather only.

TREATMENT OPTIONS FOR RAYNAUD PHENOMENON

General

  • Plan activities to avoid cold exposure
  • Dress central body in layers
  • Wear hats
  • Wear gloves and warm footwear
  • Avoid smoking and passive smoking
  • Keep skin soft and pliable with moisturizing creams

Blood Vessel Relaxants

  • Calcium channel blockers
  • Nifedipine (Adalat, Procardia and others)
  • Isradipine (DynaCirc)
  • Felodipine (Plendil)
  • Amlodipine (Norvasc)

Others

  • Block Angiotensin Effects
  • Angiotensin Receptor Blockers (Losartan)
  • Angiotensin Converting Enzyyme Inhibitors (Enalapril, Captopril, others)

Adrenergic blockers

  • Prazosin (Minipress)
  • Doxazosin (Cardura)

Centrally Acting Agents

  • Serotonin Reuptake Inhibitors (Prozac and others)
  • Antisympathetic Nervous System (Catapress, Clonidine)

Blood Thinners

  • Pentoxifylline (Trental)
  • Cilostazol (Pletal)
  • Low Dose Aspirin (81 mg daily)

Experimental

  • PDE-5 Inhibitors (Viagra and others)
  • Endothelin Antagonists (bosentan)
  • Prostacyclins (Flolan, Remodulin, Iloprost)
  • L-Arginine Supplements

Please keep in mind, this webpage is for your information only.
Please check with your child's physician for any treatments.


For more information on Juvenile Scleroderma, contact:

Juvenile Scleroderma Network, Inc.
1204 W. 13th Street, San Pedro, CA 90731

Tel: (310)519-9511 (Pacific Time)
24 Hour Support Line: 1-866-338-5892 (toll-free)

Speak to another JSD parent for emotional and logistical support provided by home-based JSD volunteers. For medical advice, please contact your child's physician.

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