What is the diagnosis and management for a middle-aged woman with symptoms of Raynaud's phenomenon, including hand discoloration in cold water, chillblains, and no joint pains?

Diagnosis: Primary Raynaud's Phenomenon with Chilblains

This middle-aged woman most likely has primary Raynaud's phenomenon with associated chilblains (perniosis), and the absence of joint pain is reassuring against secondary causes requiring immediate aggressive workup. 1, 2

Clinical Reasoning

The presentation strongly suggests primary rather than secondary Raynaud's based on several key features:

• Absence of joint pain makes connective tissue diseases (systemic sclerosis, lupus, rheumatoid arthritis) significantly less likely 3, 2
• Chilblains (perniosis) can occur with Raynaud's and represents a distinct but related cold-induced vascular response 4
• Middle age alone is not a red flag—primary Raynaud's typically manifests around age 40 (range 3-80 years) 5

Red Flags to Exclude (Critical Assessment)

You must actively look for these features that would indicate secondary Raynaud's requiring urgent workup:

• Digital ulcers, tissue necrosis, or gangrene—present in 22.5% of systemic sclerosis patients 2
• Asymmetric attacks or involvement of entire hand rather than individual digits 3, 2
• Severe, painful episodes beyond typical discomfort 1, 3
• Skin thickening (sclerodactyly), telangiectasias, or calcinosis suggesting systemic sclerosis 2
• Systemic symptoms: weight loss, malaise, fatigue, fever, photosensitivity, dry eyes/mouth 3
• Abnormal or absent peripheral pulses suggesting atherosclerosis or thromboangiitis obliterans 2

Diagnostic Workup

If physical examination is unremarkable (no digital ulcers, normal pulses, no skin changes, no joint findings), this supports primary Raynaud's and minimal laboratory testing is needed 2, 6:

• Complete blood count with differential 1
• Erythrocyte sedimentation rate (ESR) 1, 6
• Antinuclear antibodies (ANA) 1, 6
• Nailfold capillaroscopy—normal findings support primary Raynaud's 6, 4

If any red flags are present, expand workup to include:

• Rheumatoid factor, anticentromere antibodies, anti-Scl-70 antibodies 1
• Anticardiolipin antibodies and lupus anticoagulant if thrombotic history 1
• Ankle-brachial index if peripheral arterial disease suspected 1

Management Algorithm

First-Line: Non-Pharmacological Measures (All Patients)

• Absolute smoking cessation—tobacco is a critical trigger 1, 6
• Avoid cold exposure: wear mittens (not gloves), insulated footwear, hat, and coat in cold conditions 1
• Avoid abrupt temperature changes 6
• Discontinue offending medications: beta-blockers, ergot alkaloids, clonidine 1, 3
• Limit cold water immersion to maximum 10 minutes, 4 times daily to prevent tissue damage 7

Second-Line: Pharmacological Treatment (If Quality of Life Affected)

Start with calcium channel blockers as first-line pharmacological therapy:

• Nifedipine 30-90 mg daily is the most studied and effective medication, reducing both frequency and severity of attacks in approximately two-thirds of patients 1, 6
• Other dihydropyridine calcium channel blockers (amlodipine, felodipine) can be substituted if nifedipine is poorly tolerated 1

If inadequate response to calcium channel blockers, add or switch to:

• Phosphodiesterase-5 inhibitors (sildenafil or tadalafil) as second-line therapy 1

For severe, refractory cases:

• Intravenous iloprost (prostacyclin analogue) for severe symptoms unresponsive to oral therapies 1

Alternative Options (Limited Evidence)

• Fluoxetine (selective serotonin reuptake inhibitor) may help, though evidence is limited 1, 6
• Prazosin (sympatholytic agent) or losartan (angiotensin II receptor antagonist) are alternatives 6

Critical Pitfalls to Avoid

• Do not assume primary Raynaud's without thorough examination—missing secondary causes leads to delayed diagnosis and poor outcomes including digital ulcers 2
• Do not allow patients to use ice water immersion continuously—this causes immersion foot, trench foot, ulceration, and tissue damage 7
• Do not overlook subtle signs of connective tissue disease—carefully examine for skin changes, joint findings, and systemic symptoms 2
• Examine all digits for ulcers or pitting scars—digital complications are the most serious manifestation requiring aggressive treatment 2

Follow-Up Strategy

• Reassess in 3-6 months if symptoms are mild and controlled with non-pharmacological measures
• Earlier follow-up (4-6 weeks) if starting pharmacological therapy to assess response and tolerability
• Immediate reassessment if any digital ulcers, tissue necrosis, or new systemic symptoms develop 1, 2