Wilderness Medicine

Chilblains

Background

1. Definition
   • Cold-induced erythematous or ulcerative skin lesions
2. Synopsis
   • Derived from Anglo-Saxon "chill" and "blegn (sore)"
   • Usually a benign, self-limiting disorder not requiring extensive workup
     • Likely vasoconstrictive etiology
   • Primary treatment is protection of the affected area from cold

Pathophysiology

1. Etiology/Risk Factors/Predisposition
   • "Secondary" chilblains can occur as a manifestation of various systemic disorders including
     • Connective tissue/autoimmune disorders, particularly systemic lupus
     • Hematologic/malignant disorders such as chronic myelocytic leukemia
     • Viral infections
     • Cryoglobulinemia
2. Pathology
   • Exposure to cold, damp, non-freezing conditions triggers vasoconstriction and micro-vascular injury resulting in skin lesions
   • Symptoms worsen with sudden rewarming
3. Epidemiology
   • Mortality/Morbidity
     • If prolonged/recurrent symptoms - consider associated diseases (e.g., Lupus)
   • Incidence/Prevalence
     • True incidence unknown
     • Young and middle-aged women most commonly affected
     • Less common in children, older adults, males
     • Underweight people and those with poor circulation are at increased risk
     • Symptoms most common in early winter to spring

Diagnostics

1. History/Symptoms
   • Symptoms usually start 12-24 hours after exposure to cold
   • Causes itching, burning, pain in affected area
   • Typically occur on the toes or dorsum of fingers (View image)
   • Can occur on ears, nose, face, thighs, and buttocks
   • No systemic involvement
2. Physical Exam/Signs
   • Formation of single or multiple dusky red to purple macules, papules, plaques, or nodules
   • The lesions may blister or ulcerate
3. Labs/Tests
   • Diagnosis based on clinical history and physical examination
     • Laboratory tests only if suspect concomitant disease
     • CBC +Diff
     • ANA, antiphospholipid antibodies
     • Rheumatoid factor
     • Complement, cryoglobulins
4. Imaging
   • None indicated usually
5. Other Tests/Criteria
   • Skin biopsy if atypical presentation
6. Differential Diagnosis
   • Raynaud's phenomenon
   • Frostbite
   • Trench foot
   • Cold panniculitis
   • Cold urticaria
   • Erythema nodosum
   • Thromboembolic event

Treatment

1. Initial/Prep/Goals
   • Remove from cold environment
   • Gradually warm affected areas
2. Medicine/Pharmaceuticals
   • First-line
     • Avoid re-exposure to cold
     • Adequate clothing/footwear
     • Pain control with NSAIDs
     • Topical corticosteroids
       • Efficacy trials lacking
       • May be helpful for severe itch
     • Local wound care
     • Treat associated systemic illness
   • Second-line
     • Calcium channel blockers (e.g., nifedipine)
   • Recurrent disease
     • Improve circulation
     • Prazosin
     • Phenoxybenzamine
   • Alternative (isolated reports on efficacy)
     • Prednisone
     • Aspirin
     • Nicotinamide
     • Pentoxifylline
     • Prazosin
     • Hydroxychloroquine
     • Minoxidil
     • Topical tacrolimus
     • Topical nitroglycerin
3. Surgical/Procedural
   • None indicated

Complications

1. Untreated can lead to infection

Prevention

1. Avoid unprotected exposure to cold
2. Smoking cessation

Disposition

1. Admit if
   • Secondary infection
2. Consult
   • None indicated unless suspect other underlying etiology
3. Discharge/Follow-up instructions
   • Uncomplicated cases - symptoms resolve in 1-3 weeks
   • Recurrent acute episodes or prolonged symptoms for more than several weeks - consider association with systemic disease

Related Topics

• Pernio

References

1. Hedrich CM, Fiebig B, et al. Chilblain lupus erythematosus - a review of literature. Clin Rheumatol, 2008;27:949-954
2. Larkins N, Murray K. Major cluster of chilblain cases in a cold dry Western Australian winter. J Pediatr Child Health 2013;49:144-147
3. Prakash S, Weisman M. Idiopathic Chilblains. Am J Med 2009;122:1152-1155
4. Cribier B, Djeridi N, et al. A histologic and immunohistochemical study of chilblains. J Am Acad Dermatol, 2001;45:924-929
5. Cappel JA, Wetter DA. Clinical Characteristics, Etiologic Associations, Laboratory Findings, Treatment, and Proposal of Diagnostic Criteria of Pernio (Chilblains) in a Series of 104 Patients at Mayo Clinic, 2000 to 2011. Mayo Clin Proc 2014;89(2):207-215
6. Takci Z, Vahaboglu G, et al. Epidemiological patterns of perniosis, and its association with systemic disorder. Clin Exp Dermat 2012;37:844-849
7. Verma P, Singal A, et al. Pernio in an infant treated with topical nitroglycerine. Pediatr Dermat 2013;30(5):623-632
8. Simon TD, Soep JB, et al. Pernio in pediatrics. Pediatrics 2005;116:e472-475
9. Kliegman RM, St. Geme J. Nelson Textbook of Pediatrics, 21st ed, Elsevier, 2019;Chapter 93

Contributor(s)

1. Qureshi F, MD

Updated/Reviewed: July 2020