Discolored, Painful Toes in a 43-Year-Old Woman

Colin C. Edgerton, MD

Disclosures

September 03, 2015

Discussion

Chilblains (also called perniosis) are localized cutaneous inflammatory lesions associated with cold exposure. The diagnosis in this case was suggested by the characteristic location and appearance of the lesions, as well as their association with cold exposure. The benign medical history and general good health of the patient suggested that a more insidious diagnosis was unlikely.

Laboratory investigations were helpful in ruling out autoimmune or systemic inflammatory conditions known to cause similar lesions. The negative ANA and ANCA results suggested that such diagnoses as systemic lupus erythematosus and ANCA-associated vasculitides (eg, Wegener granulomatosis, Churg-Strauss syndrome, and microscopic polyarteritis) were unlikely. The normal inflammatory markers (ESR, CRP) and the absence of systemic symptoms, such as fever and weight loss, also made systemic vasculitis and pseudovasculitic conditions (such as bacterial endocarditis with emboli) unlikely causes.

Vascular surgery and endovascular catheterization may cause arterial embolization, resulting in symptoms similar to those described in this case; however, no such surgeries or procedural interventions had been performed in this case. A recent procedure involving the vascular system could be the inciting event of the cholesterol emboli syndrome. The normal lower-extremity arteriogram with run-off made large- and medium-vessel vasculopathy or vascular insufficiency unlikely diagnoses. The biopsy results revealed local inflammatory changes typical of chilblains: perivascular lymphocytic infiltrates with associated dermal edema and spongiosis.

Chilblains typically develop acutely. The lesions are either single or multiple, are erythematous to violaceous in color, and are characterized by pruritic or burning pain. Chilblains usually resolve within a few days; however, a more prolonged course, with blistering and ulceration, may occur with repeated cold exposure (as was seen in this case). Characteristic locations of the lesions include the distal phalanges of the fingers and toes.

Equestrian cold panniculitis, a condition that is probably related to chilblains, is characterized by erythematous papules and plaques located on the thighs. Young women are generally affected after exposure to cold during equestrian activities. The histology of equestrian cold panniculitis and chilblains show some overlapping features. Chilblains occur primarily in women aged 15-30 years but may occur in children and older adults as well.[1]

Chilblains are categorized as primary or secondary, on the basis of the absence or presence of an associated underlying condition. Primary chilblains (also called "banal chilblains") occur in the absence of an underlying disorder. Primary chilblains are milder than secondary disease, with complete resolution typically occurring within 3 weeks; however, chronic cases have been described with repeated exposure to cold.

Secondary chilblains occur in association with several systemic conditions, including systemic lupus erythematosus (chilblain lupus), cryoproteins, antiphospholipid antibodies, and myelomonocytic leukemia,[2] and as a reaction to certain medications (eg, infliximab [Remicade®], sulindac [Clinoril®], or terbinafine [Lamisil®]).[3,4,5] Clinical features found to correlate most closely with the diagnosis of secondary chilblains include female sex and the persistence of lesions during periods of hot weather.[6]

The leading differential diagnosis of chilblains is Raynaud phenomenon. The two can be distinguished by the shorter duration of Raynaud disease attacks (hours rather than days), as well as the characteristic triphasic pattern of Raynaud disease (pallor and cyanosis, followed by erythema.) Other considerations in the differential diagnosis include vasculopathies; vasculitis; and embolic diseases, such as the cholesterol emboli syndrome (also known as the "blue toe syndrome").

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