Hiromi Shibuya

Churg‐Strauss Syndrome (CSS) Manifested as Necrosis of Fingers and Toes and Liver Infarction

We report a case of Churg‐Strauss syndrome (CSS) with necrosis of the fingers and toes and liver infarction. A 59‐year‐old man with asthma suddenly noticed that his fingers and toes felt unusually cold. This condition worsened progressively, and some digits became necrotic within several weeks. Laboratory studies revealed hypereosinophilia and an extremely elevated serum level of IgE. Digital subtraction angiography of the extremities revealed extensive irregular narrowing of small and medium‐sized arteries in the extremities. Abdominal computed tomography (CT) revealed an area of low density at the periphery of the right lobe of the liver. Angiography revealed irregular narrowing of small arteries that corresponded to the ischemic area. A nerve conduction study suggested sensory nerve neuropathy. The preceding asthma, acute onset of digital necrosis, liver infarction, neuropathy, and hypereosinophilia strongly suggested a diagnosis of CSS. The patient was treated with 40 mg of prednisolone and 120 μg of intravenous prostaglandin E1 daily, and all the digits that had turned black and necrotic were amputated. After the amputation, the dose of prednisolone was gradually reduced, and no new lesions appeared on the skin or in the liver. The rare possible complications of CSS, including necrosis of digits and liver infarction, should not be ignored.

Use of groin flap and anterolateral thigh adipofascial flap of tensor fascia lata for reconstruction of a wide lower abdominal wall defect.

We describe a patient in whom a wide defect of the lower abdominal wall was repaired by an anterolateral thigh adipofascial flap with tensor fascia lata and groin flap.

Three cases of 'mechanic's hands' associated with interstitial pneumonia: possible involvement with foot lesions.

We describe three cases of the rare combination of lateral erythema and hyperkeratosis of the fingers that typify a condition known as ‘Mechanics Hands’. The first and the third cases were unusual in that the condition was associated with cutaneous involvement of the feet and interstitial pneumonia but not with myositis, or with only mild muscular involvement, while the second case was typical, being accompanied by myositis and detectable antibodies against histidyl transferase. We propose that Mechanics Hands can occur in association with foot lesions and interstitial pneumonia, even if it is not accompanied by myositis.

A Case of Subacute Necrotizing Fasciitis

We report a 48‐year‐old woman who developed necrotizing groin fasciitis with insidious onset. Before she visited us, she had been unsuccessfully treated with several kinds of antibiotics by other doctors for one month, because of a small ulcer covered by blackish necrotic tissue. She was referred to us because of high fever, an ulcer on the left labium majus, and a cellulitis‐like lesion with severe pain on the lower abdomen. Methicillin‐resistant Staphylococcus aureus (MRSA), Streptococcus intermedius, and Bacteroides uniformis were isolated from the wound. After aggressive debridement on the eighth day after admission of the whole indurated area and the fascia of the underlying muscle, healthy granulation tissue covered the defect, and the wound was finally closed with a skin graft. Long‐term administration of antibiotics along with insufficient and delayed surgical treatment were considered to have caused the full development of this disease.

Gas gangrene following sacral pressure sores.

We report two cases of gas gangrene developed from sacral pressure sores. The first case was clostridial and the second, non‐clostridial gas gangrene. Both patients died within two months. The first patient, a 56‐year‐old woman suffering from palsy of the lower half of the body for 3 weeks, developed a sacral pressure sore. One month later, crepitus by palpation and gas formation in the X‐ray film were detected in the hip and right thigh. A culture of odoriferous pus yielded Clostridium limosum in addition to Staphylococcus intermedius, Enterococcus faecalis, Pseudomonas aeruginosa, and Bacteroides fragilis. Blood culture yielded Bacteroides fragilis. The patient died 50 days after admission in spite of surgical debridement and aggressive therapy with high doses of antibiotics and hyperbaric oxygen. The second patient, a 70‐year‐old man suffering from diabetic nephropathy, arteriosclerosis obliterans of the lower limbs, and cerebral infarction, developed a large decubitus ulcer covering the whole sacral area. Crepitus and gas were detected in the soft tissue of the left gluteal region. Almost the entire gluteus maximus muscle was necrotic. Bacteroides fragilis, methicillin‐resistant or ‐sensitive Staphylococcus aureus and Escherichia coli were isolated from the muscle. Bacteroides fragilis was also obtained by blood culture. The patient died on the 72nd day after admission.

Frontonasal Flap for Reconstruction of Complete Alar Defects

background It is difficult to reconstruct a satisfactory ala. Axial frontonasal flap has been common in reconstruction of nasal tip. We modified this flap to reconstruct nasal ala. objective A modified axial frontonasal flap was applied for reconstruction of complete unilateral alar defects in two patients. methods Skin from an intact nasal tip covered the alar defect. The resulting defect in the nasal tip was covered with dorsal skin from the nose. Extended mucosa or a hinged nasolabial flap was used to line the mucosal side of the reconstructed ala. results The outcome judged by shape, and texture, was satisfactory. This technique can be employed under field block. conclusion The modified frontonasal flap is one of the ideal techniques to reconstruct an entire nasal ala.

Analysis of 256 cases of basal cell carcinoma after either one‐step or two‐step surgery in a Japanese institution

Basal cell carcinoma (BCC) is a common skin cancer that arises from the cells of the basal layer of the epithelium or from the external root sheath of the hair follicle. In the present report, 256 cases treated surgically between 1999 and 2008 in our department were retrospectively analyzed. The most frequent BCC locations included the face (77.8%), especially the nose (26.9%) and eyelids (21.5%). Incomplete excisions occurred in 21 cases. Two patients experienced local recurrence; one of these patients exhibited a bone metastasis while the other had a metastasis of the parotid gland without the local recurrence. The rate of local BCC recurrence was 0.78%, which is lower than that described in previous reports. We categorized BCC into four histological types: superficial, solid, adenoid and infiltrative. The solid type was the most frequent histological type (62.1%). For preventive recurrence, we treated BCC patients with two‐step surgery when the tumor was large or histologically invasive. At the first step, we excised the tumor with a sufficient safety margin, and at the second step, we performed reconstruction after the histological confirmation that no remnant malignant cells were in the tumor margins. In the present report, no local recurrence occurred in patients following the two‐step surgery. Therefore, two‐step surgery is recommended for tumors at locations and with histological types related to frequent recurrence.

Bacteroides fragilis Pyomyositis in a Patient with Multiple Myeloma

Pyomyositis is a bacterial infection with abscess formation affecting large skeletal muscles. It is predominantly caused by Staphylococcus aureus. The disease is common in tropical areas, but rare in temperate climates. We report a patient with multiple myeloma who developed a giant elastic tumor on the right thigh and a hen egg‐sized tumor on the right upper arm. MR imaging revealed cystic spaces in the femoral quadriceps and brachial biceps muscles. A large amount of pus with foul smell was removed by incision, drainage and aspiration of the two tumors. The lesions were successfully treated with intravenous administration of antibiotics. Repeated bacterial cultures yielded only Bacteroids fragilis. To our knowledge, this is the first report of pyomyositis due to Bacteroides fragilis.

A Case of Werner Syndrome with Three Primary Lesions of Malignant Melanoma

Three primary lesions of malignant melanoma developed in a 44‐year‐old Japanese woman with Werner syndrome. One lesion was on the right large pudental lip and the others in distinct locations on her left sole. After the wide local excision of these tumors, the wound of the large pudental lip was sutured, and the defects on the sole were covered with skin grafts. After one course of chemotherapy consisting of dacarbazine, nimustine, vincristine sulfate and local injection of Interferon β were performed, severe myelosupression occurred and continued for two months. Defective production of WRN protein was confirmed by Western blotting, although the three representative mutations in Japanese patients, mutations 1, 4 and 6, which include over 90% of the Japanese patients, were not detected. We also reviewed 26 cases of malignant melanoma associated with Werner syndrome (WS), including ours.

Intracranial infiltration by recurrent scalp dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) of the scalp is a rare soft tissue sarcoma. A 22-year-old male with DFSP, who had undergone several surgical excisions and radiotherapy, presented with a mild left lower-limb paresis. An MRI scan demonstrated a huge mass extending throughout the frontoparietal convexity. A macroscopically complete removal of the mass was performed. Despite this, there were several recurrences; thereafter, the patient underwent surgery, stereotactic radiosurgery and chemotherapy. The histological findings demonstrated a DFSP that both abutted and occasionally invaded the adjacent brain cortex, while extending along the Virchow-Robin spaces. Due to the highly infiltrating characteristics of DFSP, another approach, such as the use of molecular-targeted agents, will be required to significantly improve the clinical outcome.