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Confirming clinical suspicions - Clinical Case Studies - Vol. III

Livedo reticularis (blotchy 'corned beef' skin.

Mrs KU, aged 41, gave a three year history of ‘chronic fatigue syndrome’ as well as aches and pains, and a tendency to ‘irritable bowel’.

An initial diagnosis of ‘fibromyalgia’ (fatigues and aches and pains) was made.

A year later she developed slight shortness of breath, dry mouth, and frequent headaches. The headache became more severe, and frequently migrainous, so much so that on one occasion she was admitted to hospital in ‘status migrainosus’. A brain MRI was normal as was a chest x-ray. Subsequently investigations showed a positive ANA (anti-nuclear antibody) and a positive anticardiolipin test (strongly positive).

Clinical examination did not reveal any cause for the shortness of breath, but she did have two important critical signs. Firstly, she had a bone-dry Schirmer’s test (a simple test in which a strip of standardised blotting paper is hooked over the lower eyelid). The blotter is usually soaked in seconds. In Sjogren’s syndrome (see below) it can remain dry for the full five minutes of the test.

Secondly, she had prominent livedo reticularis (blotchy ‘corned beef’ skin) on the forearms and thighs.

She was started on Plaquenil with (after a month or so) improvement in her aches, pains and fatigue.

The headaches remained. Aspirin failed to help. She suffered a mini stroke despite being on aspirin. A three week trial of heparin helped to improve the headaches.

She ultimately started on warfarin, keeping the INR high at 3.5-4.

Six months later, she was a ‘changed person’. Not only had the headaches gone but, interestingly, the blotchy livedo skin had improved. As an added bonus, both the shortness of breath and the ‘irritable bowel’ had improved with the starting of warfarin anticoagulation.

What is this patient teaching us?

The story of this patient is a recurring theme: the association of Hughes syndrome (migraine, stroke and fatigue) with Sjogren’s syndrome (dry eyes and mouth, aches and pains, positive ANA).

The improvement of patients with this clinical combination after starting Plaquenil and anticoagulation is often striking. The disappearance of the livedo is something we often see following successful warfarin anticoagulation.

In this patient, the shortness of breath improved (it is conceivable that this patient was suffering from mini lung clots). More surprisingly, the ‘irritable bowel’ symptoms immediately improved. A coincidence? I have certainly seen irritable bowel symptoms in a small number of Hughes syndrome patients improve with anticoagulation. One possible explanation is that ‘bowel ischaemia’ (a sludgy blood supply to the bowel) might be contributing.

This article is intended to inform and give insight but not treat, diagnose or replace the advice of a doctor. Always seek medical advice with any questions regarding a medical condition.

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