We have analyzed the clinical variety of 13 patients with bullous pemphigoid (BP) who developed BP after oral administration of DPP4i between December 2009 and April 2016. The mean age at inclusion in the case series was 76.3 years (64-96 years). Patients developed BP between 3 months and over 3 years after the introduction of DPP4i. Of the 13 patients, 10 were on vildagliptin. According to the prognosis, we classified them into 3 groups; good after stopping DPP4i (group G), poor after stopping DPP4i (group P), and continuous DPP4i administration (group C). Group G presented non-inflammatory skin lesions, and both anti-BP180NC16a (CLEIA) and anti- full-length BP180 antibodies were negative or weekly positive. Skin lesions and antibody titers in Group P varied according to the case. Only a few cases in Group C were refractory to conventional therapy.
A 43-year-old woman was admitted to our hospital for the treatment of acute lymphocytic leukemia. After venipuncture of the right forearm for peripheral intravenous catheter placement, swelling and pain occurred in the same region. She was referred to our department 9 days after the venipuncture, because her symptoms worsened. A vascular murmur was heard with a Doppler stethoscope. Doppler ultrasonography revealed pulsation of the vein running parallel to the right radial artery that was similar to the pulsation in the artery. The diagnosis was right radial arteriovenous fistula. Local compression was applied for 4 weeks, and her symptoms improved thereafter.
An 8-year-old Japanese boy presented with a hemispherical, smooth, and flexible, 25×23 mm nodule at the back of his head after a concussion one year previously. Our initial diagnosis was a mesenchymal tumor, so it was surgically removed for excisional biopsy. A pathological examination revealed that spindle cell proliferated from the dermis to the subcutaneous tissues. The immunohistochemical examination was positive for α-SMA, D2-40, and Ki-67 and negative for CD34. Chromogenic in situ hybridization analysis for USP6 rearrangement revealed positive results. Therefore, the patient was diagnosed with nodular fasciitis. No recurrence was observed to date. Recently, it has been found that USP6 rearrangement such as MYH9-USP6 gene fusion are specific to nodular fasciitis, which is often misdiagnosed as sarcoma. Therefore, the diagnosis of nodular fasciitis is sometimes confusing. However, identifying USP6 rearrangement is useful in confirming the diagnosis of nodular fasciitis in cases of insufficient results from a pathological examination alone.