A 66-year-old Japanese woman presented with a three month history of nodules on the right buttock, thigh and lower leg. She had been diagnosed with rheumatoid arthritis which had been treated with methotrexate and prednisolone, and the administration of abatacept. The nodule was about 2 cm in diameter with 10 day history of pain and linear erythema. After taking antibiotics, the nodule ulcerated. On the right side of the lesion, a 5 mm bulla was noted. Biopsies were taken from both lesions. Histopathology: On the nodular lesion, many granulomas had formed. On the bullous lesion, an intra-epidermal blister with epidermal necrosis and ballooning degeneration was detected. Both lesions had perivascular fibrin deposition, nuclear dust and red blood cell extravasation. Immunohistochemical findings of VZV were positive in both lesions. By real time PCR, only VZV was detected.
We diagnosed the nodular lesions as cutaneous granulomatous vasculitis associated with herpes zoster infection. This disease is a non-necrotizing granulomatous vasculitis that may occur in VZV reactivation, particularly in an immunocompromised host. It can be severe when it affects the central nervous system. In the skin, however, it is rare. This is a first case of granulomatous vasculitis associated with herpes zoster infection in Japan.
The parents are both Asian Indian and consanguineous. Their first child was healthy. Their second and fourth children developed blisters and erosions around their body when they were three-days-old and one-day-old, respectively. Pathological examination showed subepidermal bullae, and immunohistochemical staining revealed deficiency of laminin332, which led to the diagnosis of generalized severe junctional epidermolysis bullosa (formerly named Herlitz type). Despite the topical therapy, general medication, and antibiotics, they both died of dyspnea at the age of fifteen-months and thirteen-months, respectively. Genetic analysis revealed homozygosis of the p.Arg569* mutation for both children. This mutation has been reported from the USA, India, and the UK so far. In Japan, 15 cases of generalized severe junctional epidermolysis bullosa have been reported, but none of them had this mutation.