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

Colin C. Edgerton, MD

Disclosures

September 03, 2015

The pathophysiology of chilblains is poorly understood, but it appears to involve increased cutaneous vasoreactivity leading to microvascular injury, with an ensuing inflammatory response. Cutaneous biopsy findings in primary chilblains include epidermal spongiosis, variable papillary dermal edema, and both superficial and deep lymphocytic inflammatory infiltrate (which can also extend to the perieccrine areas).

Other epidermal changes can also occur in primary chilblains. When associated with an underlying connective tissue disorder, secondary chilblains, aside from exhibiting the aforementioned histologic findings seen in primary chilblains, may also demonstrate the histopathologic findings of an interface dermatitis, including epidermal keratinocyte basal layer vacuolation, dyskeratotic keratinocytes, and lymphocytes approximating the dermal/epidermal junction. The perivascular inflammatory cellular infiltrates in both primary and secondary chilblains contain primarily T cells, with fewer macrophages and B cells. Because chilblains and chilblains-like lesions can be seen in association with various underlying conditions, patients with the clinical or histologic features of chilblains should be screened for any underlying conditions.

In addition to skin biopsy, useful diagnostic studies include those that suggest secondary causes, as well as those that rule out diagnoses causing similar-appearing lesions. A complete blood count may reveal leukocyte abnormalities associated with leukemia. Anemia and thrombocytosis may suggest a systemic inflammatory condition, such as vasculitis. Thrombocytopenia may be associated with antiphospholipid antibodies and infiltrative leukemias.

Elevations in the ESR and the CRP level may indicate systemic inflammation that suggests secondary causes, such as leukemia, vasculitis, cryoproteins, and systemic lupus erythematosus. ANA testing is useful in ruling out systemic lupus erythematosus. ANCA testing is useful in detecting ANCA-associated vasculitides in the proper clinical setting. Assays for antiphospholipid antibodies, including the lupus anticoagulant, anticardiolipin antibodies, and antibodies to beta-2 glycoprotein 1, may detect the presence of pathologic titers of these antibodies.

Angiography may detect vasoocclusion secondary to inflammatory vasculitis or noninflammatory vasculopathy. Angiography and noninvasive vascular laboratory testing is normal in chilblains.[7]

Nail-fold capillaroscopy, a relatively simple examination of the nail-fold capillaries under magnification, is normal in chilblains, but it may reveal dilated vessels and vessel drop-out in systemic autoimmune conditions, such as systemic lupus erythematosus and systemic sclerosis.

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