Necrolytic acral erythema.
DISCUSSIONNecrolytic acral erythema (NAE) is a chronic, hyperkeratotic skin condition of the distal extremities first described in 1996. 1 NAE is classically associated with chronic hepatitis C based on concomitant hepatitis C infection in nearly all of the earliest reports. 1 However, more recent reports of NAE arising in other conditions have challenged this dogma, including evidence of NAE arising in response to certain medications, autoimmune diseases, and other chronic conditions in the absence of coexistent hepatitis C infection. [2][3][4][5] As far as we are aware, this is the first known case of NAE arising in the setting of autoimmune hepatitis.The acute stage of NAE presents as erythematous papules, plaques, and/or flaccid bullae, most commonly in an acral distribution. In the chronic phase, lesions become annular, sharply defined, hyperkeratotic, and erythematous or violaceous plaques. The lesions may be pruritic or have an aching or burning sensation. 3 Histologically, early lesions of NAE are characterized by necrosis of the superficial epidermis with detachment from the underlying epidermis. In chronic lesions, the development of psoriasiform epidermal hyperplasia, superficial epidermal pallor, areas of basal cell layer vacuolar degeneration, scattered foci of necrotic keratinocytes, and associated parakeratosis become apparent. 3 Of note, our case lacked significant epidermal pallor as classically seen in NAE and other nutritional deficiencies (such as necrolytic migratory erythema), which demonstrates there can be some variability in From the