[in Japanese]

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A 29-year-old man presented with wheals, nausea, and vomiting, and he was diagnosed with anaphylaxis by a nearby clinic. Thereafter, he suffered from five anaphylactic episodes with symptoms including abdominal cramps, wheals, nausea,vomiting, and decreased blood pressure. Since he had ingested“natto (fermented soybeans)”the day before each of the fourth and fifth anaphylactic episodes, late-onset anaphylaxis against natto was suspected. A skin prick-to-prick test for natto was positive, and after an oral challenge with natto, he developed pharyngeal discomfort and wheals accompanied by a severe decrease in blood pressure six hours after ingestion. Therefore, this case was diagnosed as anaphylaxis due to natto. No anaphylactic or allergic symptoms have occurred after he stopped eating natto.

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A 29-year-old woman underwent abdominal liposuction 13 days before her first visit to our hospital. Nine days later, she had fever and a painful erythema and swelling at the operation sites that rapidly became exacerbated and formed bullae the next day. On admission to the emergency room, she was suspected to have a pulmonary embolism. The abdominal lesion became necrotic and the laboratory findings revealed leukocytosis, elevated C-reactive protein, anemia, and a coagulation abnormality. Echocardiography and a pulmonary CT scan showed pulmonary artery dilatation without an overt thrombus,leading to a diagnosis of pulmonary embolism due to fat embolism. Septic shock was ruled out because of a negative bacterial culture result for the necrotic tissue obtained by surgical debridement. Despite the subsequent development of acute respiratory distress syndrome, she was successfully treated and survived. Fat embolism syndrome is a rare but serious complication after liposuction. Careful observation is needed after this procedure.

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An 83-year-old woman presented with blisters on both feet, the lower legs, and the buttocks. Histopathological examination demonstrated subepidermal bullae and mild lymphocytic perivascular infiltration in the upper dermis. Direct immunofluorescence examination showed the linear deposition of IgG at the epidermal basement membrane zone. Indirect immunofluorescence examination of normal human skin split using 1M NaCl showed IgG reactivity on the dermal side. Enzyme-linked immunosorbent assays detected IgG autoantibody against type Ⅶ collagen recombinant protein. The patient was diagnosed as epidermolysis bullosa acquisita based on the clinical and pathological findings. Although the patient was treated with oral prednisolone 25 mg (0.5 mg/kg)/day, the symptoms did not improve. We added cyclosporine 50 mg (1 mg/kg)/day and increased the dose to 75 mg (1.6 mg/kg)/day after one week. One week later, the patient improved dramatically with the cessation of new blister formation. We report a case with epidermolysis bullosa acquisita that was successfully treated with low-dose cyclosporine. This report indicates the efficacy of cyclosporine in treating epidermolysis bullosa acquisita.

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A 75-year-old Japanese woman was treated with bevacizumab and docetaxel combination chemotherapy for multiple bone metastases of lung cancer. Two months later, chemotherapy was temporarily ceased due to loss of appetite and febrile neutropenia. At 2 months after the re-administration of bevacizumab, she was referred to our department complaining an ulcer on her right ankle. The wound shrank during a suspension of the chemotherapy. However, the re-administration of bevacizumab markedly exacerbated the ulcer again. A 62-year-old Japanese woman visited our department with an ulcer on her dorsal right or left foot. She had been administered with bevacizumab and paclitaxel combination therapy for relapsing breast cancer. The wound shrank after the discontinuation of chemotherapy. Histopathologically, epidermal necrosis,infiltration of inflammatory cells such as lymphocytes and neutrophils, and fibrinoid degeneration of capillaries with hemorrhage were observed. The administration of bevacizumab, an anti-vascular endothelial growth factor antibody, was suspected to have caused the leg ulcers in both cases.

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A 42-year-old Japanese man presented with numbness of his left face and pain on his left side of the forehead 1 year prior to his initial visit to our clinic. At the first medical examination, the left cheek and nose wing were clearly depressed. The left side of the forehead presented pigmentation and an atrophic sclerosing plaque, while the contiguous region on the left frontal side of the head appeared atrophic and hairless. Computed tomography demonstrated a decreased amount of fat tissue at the left cheek. A skin biopsy from the left frontal side of the head showed a thickened dermis composed of abundant collagen bundles. We diagnosed Parry-Romberg syndrome together with scleroderma en coup de sabre. These two disorders frequently overlap ; thus, they are thought to be on the same spectrum of localized scleroderma. Both often accompany central nervous system imaging abnormalities without central nervous system symptoms. Since neurologic findings are not always revealed in correlation with skin lesions, neuroimaging studies should be considered in Parry-Romberg syndrome cases at the time of diagnosis.

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A 45-year-old Japanese man presented to our department with multiple calli on the soles. He has a past medical history of hyperparathyroidism, bilateral ocular cataracts, dyslipidemia, and osteoporosis. He had a lifelong history of short stature with muscle atrophy from childhood. He developed scleroderma on the fingers in his teenage years. Around his 20s, he suffered from hair loss as well as gray hair. In his 40s, the soles of his feet became severely sclerotic. At presentation, he had a short stature associated with a bird-like face and gray hair. We presumed Werner syndrome because such features are typical of this condition. Laboratory studies revealed primary hypogonadism and an X-ray examination showed flame-like calcification of the Achilles' tendon. Ultrasonography demonstrated fatty liver, a thyroid cyst in the right lobe measuring 6 mm, and a nodule in the left lobe measuring 9 mm. A genetic test revealed a c.3139-1G＞C mutation in both alleles of the WRN gene. We diagnosed him with Werner syndrome based on the diagnostic criteria, which consisted of 3 major parts, namely cardinal signs and symptoms, other signs and symptoms, and genetic testing. Dermatologists may play an important role in diagnosing Werner syndrome because the patients are susceptible to dermatological symptoms.

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A 62-year-old woman had hit her head on a garage door. Subsequently, a nodule developed 3 months prior to her first visit to our department. On gross examination, there was a tumor presenting as a purplish macule on the parietal region of the head. Histopathological examination of the tumor showed a proliferation of endothelial cells with large nuclei in the dermis, which formed vascular-like structures with distinct lumens. On immunohistochemistry, the tumor cells were positive for CD31, CD34, and D2-40 in the cytoplasm, and ERG in the nucleus, which led us to the diagnosis of angiosarcoma. The tumor was irradiated with an electron ray (70 Gy/35 Fr), followed by chemotherapy using paclitaxel. Due to severe bone marrow suppression, paclitaxel was discontinued after the 3rd course ; subsequently, docetaxel was started and has been continued monthly. At 6 years after the first visit, docetaxel was discontinued because the tumor disappeared clinically ;however, during a suspension of treatment for 10 months, angiosarcoma recurred on the head. The tumor disappeared rapidly after the resumption of docetaxel and the patient has subsequently been treated with docetaxel monthly. The patient is still alive and has experienced no distant metastasis for more than 8years. Angiosarcoma is a high-grade vascular malignant neoplasm. We discuss the usefulness of taxanes for patients with angiosarcoma.

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A 61-year-old woman with cancer of the right breast presented with diffuse erythema on her right breast during preoperative chemotherapy. Histopathological assessment of the erythema indicated no invasion of breast cancer cells ;therefore, treatment with topical steroid was conducted. One month later, radical right mastectomy and right axillary lymph node dissection were performed. During postoperative ionizing radiotherapy, the erythema spread over the upper abdomen and left chest, i.e., on the opposite side to the primary lesion. Histopathology of the erythematous papules revealed tumor cells in the lymphatic vessels and the diagnosis of inflammatory breast cancer (IBC) was made. Although chemotherapy initially improved the erythema, diffuse annular erythema later reappeared on her left breast. Histopathological and positron emission tomography/computed tomography examinations indicated a relapse of the IBC, and the chemotherapy was restarted. The erythema disappeared 6 months later and she has been doing well until now at over 4 years since the initial diagnosis of mammary cancer. IBC is a rare condition that is defined as a form of breast cancer with symptoms of swelling and reddening of the skin. The histologic hallmark of IBC is cancer cell emboli in the dermal lymphatic vessels. In the present case, we consider that embolic cancer cells invaded over the midline of the trunk through interconnecting lymphatic vessels to develop skin lesions in the contralateral breast. If patients with breast cancer develop inflammatory cutaneous symptoms, IBC should be suspected.

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