Chronic chilblains
BMJ 2011; 342 doi: https://doi.org/10.1136/bmj.d2708 (Published 07 June 2011) Cite this as: BMJ 2011;342:d2708
- 1Middelie 91, 1472 GT Middelie, Netherlands
- 2Radboud University Nijmegen Medical Centre, Department of Primary and Community Care, Unit Women’s Studies Medicine, 6500 HB Nijmegen, Netherlands
- Correspondence to: IHSouweri.souwer{at}wxs.nl
- Accepted 7 March 2011
A woman visited her general practitioner in mid December with pain and reddish discoloration of her toes. Her symptoms had recurred every winter, but resolved completely in the summer. In previous years, she had had similar symptoms in her fingers.
What you should cover
Chronic chilblains are cold induced, painful or itching, red-blue lesions on the fingers, feet, ears, or thighs (fig⇓).1 The condition occurs throughout the world, more commonly in women than men. The average Dutch general practitioner reports four new cases a year (Continuous Morbidity Registration Nijmegen, The Netherlands; unpublished data). If patients consult a doctor at all, it will be their general practitioner.2
Symptoms usually start in early winter and vanish in spring, but often recur the next winter. Sometimes symptoms persist owing to continued exposure to cold, which is commonly associated with work conditions (cold storage work, for instance). Ulceration may also be present.
Patients may present with painful or itching lesions on the fingers, feet, ears, or thighs
In clinical settings, chronic chilblains are associated with connective tissue disease, particularly systemic lupus erythematosus, but the evidence for this association is not substantial. Although up to a third of cases of chronic chilblains are reported to be diagnosed as chilblain lupus erythematosus, the proportion of patients with chilblains in a group with systemic lupus erythematosus did not differ from patients in the control group.3 Persistence of lesions during the summer is probably associated with chilblain lupus erythematosus.4 Case reports have linked the disorder to rare forms of vessel disease and rare conditions such as Sjögren syndrome and chronic myelomonocytic leukaemia. The presence of cryoproteins, cold agglutinins, and hereditary protein C and S deficiencies are not associated with chronic chilblains.5
What you should do
The main aim is to differentiate chronic chilblains from chilblain lupus erythematosus and other mimics.
Ask about the typical symptoms of chronic chilblains, particularly the specific pattern of the course: did the symptoms start when average temperatures drop below 10-15 degrees Celsius? Do the symptoms decline in spring? And does relapse occur in consecutive winters? Any family history of chilblains supports the diagnosis, because a hereditary factor may be involved in the onset of chronic chilblains.2
Persistent symptoms during the summer, a rash associated with discoid lupus erythematosus, photosensitivity, arthralgia, arthritis, oral ulcers, seizures, or psychosis suggest chilblain lupus erythematosus.4
Chronic chilblains are commonly confused with Raynaud’s syndrome. If the patient reports a history of the characteristic paroxysmal and biphasic white, and later blue discoloration, after each exposure to cold, this will help to differentiate this syndrome from chronic chilblains.
Inspect the affected skin for red-blue lesions that are typical of the disorder. Such lesions are usually swollen and may be ulcerated.
Pulsation of the peripheral arteries should be normal; otherwise, peripheral vessel disease is a possibility.
For diabetic patients, the possibility of a diabetic foot should be considered, particularly if peripheral vessel disease or other ischaemic problems are present.
Additional tests are useful in some cases: If chilblain lupus erythematosus is suspected, a negative antinuclear antibody test makes systemic lupus erythematosus unlikely. Other laboratory investigations are not usually helpful. Skin biopsy does not usually help differentiate between chronic chilblains and its mimics.1
Various interventions have been suggested, including vitamin D3 (which has no therapeutic effect6), nifedipine, and a corticosteroid cream. Our study of the patient’s perspective shows that most patients visited a general practitioner, but were not given much useful information. Most patients tried a number of remedies without success.2
Simple advice for self management may be helpful (know that the disease is usually harmless and self limiting in spring, avoid exposure to cold, and wear adequate clothing).
The best evidence is provided by a randomised controlled trial of just 10 patients, which favoured nifedipine 60 mg/day over placebo.7
Notes
Cite this as: BMJ 2011;342:d2708
Footnotes
This is part of a series of occasional articles on common problems in primary care. The BMJ welcomes contributions from GPs.
Competing interests: All authors have completed the Unified Competing Interest form at www.icmje.org/coi_disclosure.pdf (available on request from the corresponding author) and declare: no support from any organisation for the submitted work; no financial relationships with any organisations that might have an interest in the submitted work in the previous three years; no other relationships or activities that could appear to have influenced the submitted work.
Provenance and peer review: Not commissioned; not externally peer reviewed.