Discussion
To our knowledge, we report the first case of EED with skin lesions
involving the scalp, and the first case of EED improved with the
treatment of IVIg. According to the current reports, EED skin lesions
mostly occur in the limbs, especially in the
joints(4). Recently, it has been reported that EED
lesions can occur in some atypical sites such as the trunk and palm, and
these EED cases have been reported to be related to non-Hodgkin
lymphomas and IgA gammopathy(5, 6). These cases have a
good response to the therapy. However, our case is more peculiar. This
case shows a history of pulmonary tuberculosis and abnormal levels of
IgG and IgA. In addition, lesions of this patient were distributed not
only on the trunk and plantar surface but also on the scalp.
Furthermore, this case showed an ineffective response to dapsone and
corticosteroids. Therefore, we speculate that EED with multiple
underlying diseases as well as the lesions involving the atypical sites
has a poor response to traditional treatments such as dapsone and
corticosteroids.
In addition, this patient showed a sensitive response to IVIg with no
obvious side effects. IVIg is thought to interrupt the steps of the
complement activation cascade and down-regulate B cell
proliferation(7). It has been proved to have a good
curative effect in patients with pemphigus, dermatomyositis, and
scleroderma. In the literature we retrieved so far, this is the first
case of IVIg in the treatment of EED. A large number of literature have
reported that dapsone has been successfully used in the treatment of
EED, but still showed poor efficacy in about 20% of
patients(8). Treatment for refractory EED should also
be considered. For those patients with a poor response to dapsone and
corticosteroids or intolerant to dapsone, this case provides another
option for the treatment of EED. However, because this is the first case
of EED treated with IVIg, its efficacy should be confirmed by more
patients with longer follow-up.