Scleroderma renal crisis (scleroderma renal crisis: SRC) is characterized by severe hypertension that suddenly develops. It is one of the most severe complications in patients with systemic sclerosis (SSc). There have been only a few reports of prodromal symptoms of SRC. Therefore, we retrospectively examined these symptoms in 12 patients who visited the Department of Dermatology, Kanazawa University Hospital, between 2001 and 2014. We included general malaise, fever, muscle pain, and headache as physical findings and decreases in hemoglobin and platelets and serum creatinine elevations as laboratory findings. The average age of onset of SRC was 51.5 (24-79) years and the period from diagnosis of SSc to diagnosis of SRC was 1.95 (0.3-25) years [median (range) ]. The frequencies of prodromal symptoms of SRC were as follows; general malaise was found in 9 patients (75%), fever in 3 (25%), muscle pain in 5 (42%), and headache in 2 (17%). These symptoms were often seen from one week to five months before onset. Laboratory findings detected decreased hemoglobin in 2 patients (17%), decreased platelets in 5 (42%), and elevated serum creatinine in 6 (50%). Eleven of the 12 patients had one or more prodromal symptoms; only one did not. These prodromal symptoms were correlated with thrombotic microangiopathy (TMA)-like pathology. These results indicate that SRC often shows some prodromal symptoms and that careful follow-up is necessary in patients with SRC risk factors such as early stage, diffuse form of skin thickness and anti-RNA polymerase antibody.
A 61-year-old man presented with erythema, erosion, and crusting in the genital area and lower limbs, which had spread gradually to his face and thighs during the 6 months prior to his visit. An histological examination of the erythema showed vacuolation of keratinocytes and a reduced Langerhans cell count. A blood examination showed hypoaminoacidemia, a low serum zinc level, and an extremely high serum glucagon level. An abdominal computed tomography scan revealed pancreatic tumors and multiple liver metastases. Based on the findings of a needle biopsy, glucagonoma was diagnosed. The erythema markedly improved following intravenous drip administration of amino acids. In this article, we discuss the nomenclature of necrolytic migratory erythema, reemphasizing that necrolytic migratory erythema and similar nutritional deficiency disorders can be unified under the single term, "erythema dystrophica."
A 39-year-old woman was bitten on the right thigh by a spider, identified as Loxosceles rufescens by an experienced entomologist. Six hours later, the bite became painful with fever and generalized erythema. Twenty hours later, the "red, white, and blue sign" became evident. On day 30, the necrotic lesion had developed into an ulcer with a diameter of 10 cm. Four months later, the wound was almost healed with initiation of scar formation. Loxosceles rufescens can be found in Japan and can cause necrotic skin lesions. This is the first reported case of proven loxoscelism in Japan.