White : Diseases of the Skin - Collagen Vascular Diseases

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Gary M. White & Neil H. Cox

Diseases of the Skin

13	Collagen Vascular Diseases

RAYNAUD PHENOMENON

Etiology and pathogenesis

Causes of Raynaud phenomenon are listed in Table 13.1. It is a vasospastic disorder that is relatively common as an isolated phenomenon, usually in women and mainly the young adult age group (2% of the overall population, but about 10–20% of young women). It may be familial, usually with a young age of onset (most below 30 years). Symptoms may vary with pregnancy, menopause, and menstrual cycle.

Five to ten percent of affected individuals will develop a collagen vascular disease, especially sclerodermas; this should be suspected especially in men who develop Raynaud phenomenon, as they less commonly suffer from idiopathic Raynaud disease. It is a feature in about 25% of patients with systemic lupus erythematosus (SLE) or Sjögren syndrome, and 95% of those with systemic sclerosis.

Within the microvasculature, the abnormal vasoconstriction appears to involve an imbalance between endothelium-derived constricting factors (endothelins) and relaxing factors (nitric oxide).

Table 13.1 CAUSES OF RAYNAUD PHENOMENON

Mechanism	Examples
Idiopathic	(= Raynaud disease)
Arterial: mechanical obstruction	Extravascsular causes (thoracic outlet, trauma), intravascular causes (thrombi, arteriosclerosis, stenosis, etc.)
Connective tissue disorders	Systemic sclerosis or scleroderma, polyarteritis nodosa, lupus erythematosus, vasculitis
Hematologic	Cold-precipitable proteins, other causes of hyperviscosity
Neurologic	Nerve compression, chronic disuse disorders (e.g. hemiplegia), reflex vasoconstriction
Trauma	Vibration white ﬁnger
Drugs	Beta-blockers, nicotine, ergot, bleomycin, vinyl chloride, methysergide

Clinical features

The characteristic lesion of Raynaud phenomenon is a sharply demarcated pallor of the digit (Fig.13.1), usually with a cut-off in the region of the proximal interphalangeal joint, which is of sudden onset and may be triggered by a cold environment or by handling cold objects.

Features that suggest an underlying collagen vascular disorder include elongated cuticles or nail fold telangiectasia, pulp atrophy of ﬁngertips producing pitted scars, and positive antinuclear antibody (ANA) tests.

Fig. 13.1 Raynaud phenomenon, demonstrating the sharply demarcated distal whiteness of affected digits. The affected ?ngers typically vary between episodes, although several may be affected at any one time.

Differential diagnosis

The classic sharp cut-off and color change sequence is diagnostic, in which case the issue is to determine whether the disorder is idiopathic or has an underlying cause. Disorders that may be confused with Raynaud phenomenon include the following.

• 'Poor circulation' - all fingers and the hands generally are cold, and may variably appear pale.

  •   Acrocyanosis - the hands and all ?ngers are dusky blue or purplish, and prone to perniosis.

  •   Perniosis (chilblains) - individual lesions are painful, inflamed, and last days or weeks.

  •   Achenbach syndrome - affects a solitary digit, which becomes painful and then swollen and blue; this is due to venous rupture, often after a degree of trauma (e.g. twisting off a tight jar lid) and does not have the vasoconstrictive phase of Raynaud phenomenon.

  •   Buerger disease - a smoking-associated severe form of peripheral vascular disease characterized by digital necrosis.

Treatment

Mild cases require no treatment other than gentle rewarming and sensible measures such as gloves and warm clothing to reduce the frequency of episodes. More complex clothing includes electrically heated gloves and socks.

A variety of vasodilator drugs may be helpful, especially calcium channel antagonists and pentoxifylline.

In the more severe cases with severe symptoms, ulceration, or vascular compromise (usually those with a collagen vascular disorder), additional treatments such as steroids, immunosuppressive agents, plasmapheresis, prostacyclin infusions, upper limb sympathectomy, and more recently digital sympathectomy may be applicable. Novel vasodilators such as calcitonin gene-related peptide (CGRP) have also been used.

PRACTICE POINTS

•	Raynaud phenomenon in a male patient is strongly suggestive that there may be an associated collagen vascular disease.
•	If all fingers are cold at the same time, the diagnosis is probably not Raynaud phenomenon.
•	A painful and dusky finger without a preceding pale vasoconstrictive phase is probably not due to Raynaud phenomenon.
•	Suggested screening tests in a patient with Raynaud phenomenon and no other clinical clues would be complete blood count, viscosity and autoantibody screen (to include antinuclear antibody, ribonucleoprotein, Scl-70, and anticentromere; see Table 13.4).

•	'Poor circulation' - all fingers and the hands generally are cold, and may variably appear pale.
•	Acrocyanosis - the hands and all ?ngers are dusky blue or purplish, and prone to perniosis.
•	Perniosis (chilblains) - individual lesions are painful, inflamed, and last days or weeks.
•	Achenbach syndrome - affects a solitary digit, which becomes painful and then swollen and blue; this is due to venous rupture, often after a degree of trauma (e.g. twisting off a tight jar lid) and does not have the vasoconstrictive phase of Raynaud phenomenon.
•	Buerger disease - a smoking-associated severe form of peripheral vascular disease characterized by digital necrosis.