Infantile Bullous Pemphigoid Treated Using Intravenous Immunoglobulin: Case Report and Review of the Literature

Abstract

We report a 5-month-old girl diagnosed with bullous pemphigoid who initially did not respond to systemic corticosteroids and dapsone but rapidly improved after the addition of intravenous immunoglobulin (IVIG) infusions. A literature search revealed anecdotal cases of infantile bullous pemphigoid treated with IVIG, although variable treatment regimens were used, and some resistant cases required additional medications such as rituximab for clinical remission.

Infantile bullous pemphigoid successfully treated with combined pulse corticosteroids and high-dose IVIG

“…IVIG is known to act not only as steroid‐sparing agents but was proven to be also highly efficient in refractory cases of autoimmune bullous diseases in adults (Chee & Murrell, ). Our case along with reported ones (Tekin & Yücelten, ) confirm that IVIG is secure and valuable treatment modality in infantile pemphigoid. It should be especially considered in resistant instances unresponsive to systemic corticosteroids.…”

Autoimmune bullous disease in childhood

“…In one of these patients (case 3), who has previously been described in greater detail, a rapid response was obtained using IVIg infusions after treatment failure with methylprednisolone and dapsone. [4] The another patient (case 4) had hyper-immunoglobulin E (IgE) syndrome and was being treated with monthly IVIg infusions and methylprednisolone at the time of onset of vesiculobullous lesions [Figure 1b]. In addition to ongoing treatment for hyper-IgE syndrome, the patient received a long course of oral dapsone to obtain remission after 72 months of treatment.…”

“…In the literature, a favorable response was obtained in most of the patients with infantile or juvenile BP using topical or systemic corticosteroids;[210] however, IVIg has been utilized with success in refractory cases, such as case 3. [4] Notably, the other patient was being followed up with a genetically confirmed diagnosis of hyper-IgE syndrome and treated with monthly IVIg infusions at the age of 2 years when he developed blisters clinicopathologically compatible with BP. Several monthly courses of systemic corticosteroids, dapsone and methotrexate, were introduced while continuing IVIg and slowly tapering corticosteroids, allowing remission to be achieved 72 months after initial diagnosis.…”

Two infants with blistering rashes originating on acral sites as a presenting sign of infantile bullous pemphigoid

“…1,3,8 intravenous immunoglobulin may also be considered in infantile BP treatment, especially in recalcitrant disease. 4,9,10 Although infantile BP is considered a rare disease, its incidence has been growing. Whether this is caused by increased disease incidence or better diagnostic techniques is uncertain, but clearly defined diagnostic and treatment guidelines would aid clinicians in identifying and treating patients.…”