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Saturday, January 01, 2011

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Case Note

Case Note: Papular Eruption on the Toes

History

A 39-year-old healthy female, with no significant past medical history, presents for management of papular eruption on the toes.

Initial evaluation

  • Review of systems is non-contributory - notably negative for fever, joint pain, bleeding gums, or easy bruising
  • Social history is unremarkable
  • Recent trip to England to visit relatives
  • Presents in December for management of four tender papules on the toes
    • Papules are small (5 mm), purple-red, faintly violaceous, partly blanching, and are arranged symmetrically on the dorsal toes
    • Occurred in a similar distribution following a trip to England almost exactly 1 year ago
  • No other skin lesions present
  • Image 1
    Image 1

Enter your diagnosis

Diagnosis

  • A diagnosis of chilblains is rendered

Initial Treatment

  • It is recommended that the patient wears warm, layered clothing
    • Patient should carefully monitor temperature and clothing - including avoiding any damp clothes - both now and on future trips to England
  • Follow-up if patient fails to respond

Follow-up evaluation strategy

Follow-up evaluation:

  • Patient notes symptomatic relief and resolution of papules after 2-3 weeks
  • Patient advised to maintain warm attire in order to avoid cool, damp environments
  • Follow-up as required; report any systemic symptoms such as joint pain

Further recommendations:

  • Calcium channel blockers, particularly nifedipine (20 mg three times daily) may alleviate lesions and prevent recurrence
  • There are reports of ultraviolet light phototherapy leading to decreased numbers of and severity of lesions, particularly when instituted prophylactically at the beginning of the cool season

Subsequent treatment steps:

  • True idiopathic chilblain is a self-limiting disease whereby lesions should all resolve completely, although in some cases that may take weeks to months
  • Individual symptomatic lesions may benefit from topical over-the-counter antipruritic agents or weak anti-inflammatory agents, but there exists little evidence to support their use
  • Persistent lesions should prompt an evaluation for systemic diseases, particularly chilblains lupus erythematosus or chronic myelomonocytic leukemia. The presence of thromboses should prompt consideration of thrombotic disorders, including cryoglobulinemia or anti-phospholipid antibody syndrome

General discussion

Chilblains, also termed pernio, is a benign inflammatory condition of the distal skin which occurs in cold, damp environments. The skin findings of chilblains typically occur in subfreezing, moist climates - during the late fall and winter - and are due to persistent exposure to temperatures presumed to cause transient vascular changes in the periphery.

In the United States, most patients present with skin findings between December and January, though the disease is overall more common in England and northern Europe. Chilblains is rare in regions of extreme cold, as most people require warm houses with central heating and warm layered clothing during outdoor exposure. The condition is more common in women than men, with about a 2:1 ratio. Most reports are in white patients who are young to middle aged. Patients with low body mass index seem to be at increased risk.

Lesions of chilblains typically occur 1-5 hours after prolonged exposure to cool, damp air, and develop as single or multiple erythematous-to-purple macules, papules, plaques, or nodules; diffuse lesions over the digits may be seen. Lesions are more common on the dorsal aspects of the digits, and may be pruritic, painful (burning or tingling), and are often tender to palpation. Erosions, ulcers, and blisters may occur but are rare. Involvement of the feet is most common, followed by the hands. When the lesions appear they are often bilateral and affect cool extremities, particularly the digits. The nose, ears, lateral thighs, and buttocks may be involved. While biopsies are not often necessary, histological examination of chilblain lesions will show perivascular and/or peri-eccrine lymphocytic inflammation and superficial dermal edema.

The pathophysiology is uncertain, though it is believed that patients with chilblains have a disruption of neurovascular responses to skin temperature change. Vessels in the skin have multiple protective physiologic responses to dilate and constrict variably based on the need to conserve heat or to ensure tissue perfusion and prevent ischemia; in patients with chilblains some have postulated that there is either prolonged vasoconstriction leading to mild tissue hypoperfusion and localized inflammation in response to tissue injury. Vasospasm, hyperviscosity, or (in some patients) autoimmunity may alternatively be responsible for the disease.

Further reading

Brown PJ, Zirwas MJ, English JC 3rd. The purple digit: an algorithmic approach to diagnosis. Am J Clin Dermatol 2010;11:103-16.

Hedrich CM, Fiebig B, Hauck FH, et al. Chilblain lupus erythematosus: a review of the literature. Clin Rheumatol 2008;27:949-54.

Lutz V, Cribier B, Lipsker D. Chilblains and antiphosphlipid antibodies: report of four cases and review of the literature. Br J Dermatol 2010;163:645-6.

Page EH, Shear NH. Temperature-dependent skin disorders. J Am Acad Dermatol 1988;18:1003-16.

Prakash S, Weisman MH. Idiopathic chilblains. Am J Medicine 2009;122:1152-5.

Tlougan BE, Mancini AJ, Mandell JA, et al. Skin conditions in figure skaters, ice-hockey players, and speed skaters. Part II - Cold-induced, infectious, and inflammatory dermatoses. Sports Med 2011;41:967-84.

Yang X, Perez O, English JC 3rd. Adult perniosis and cryoglobulinemia: a retrospective study and review of the literature. J Am Acad Dermatol 201062:e21-2.

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