Saburo Anzai

Apocrine hidrocystoma : a case report and analysis of 167 Japanese cases

Correspondence Apocrine hidrocystoma: a case report and analysis of 167 Japanese cases An 81-year-old woman presented with a deep-purple cystic tumor, measuring 15 mm × 15 mm, on her right cheek (Fig. 1). Puncture of the tumor resulted in the extravasation of a clear but straw-colored fluid. The histopathologic examination showed cystic spaces in the mid-dermis, with some papillary projections. The epithelial lining of the cyst was composed of columnar cells showing a “decapitation” secretion indicative of apocrine secretion (Fig. 2). Periodic acid–Schiff-positive, diastase-resistant granules were found in the secretory cells. An outer layer of spindle-shaped cells was partially S-100 protein positive. Although total excision is the most common treatment, this would have been difficult in our patient, who had severe senile dementia. We therefore excised, by scissors, only the roof of the tumor. A faint scar remained on her cheek at her 2-month follow-up visit. Apocrine hidrocystoma (cystadenoma) commonly occurs as a solitary cystic lesion on the head and neck. In 1968, Mehregan described 17 cases of a benign nevoid tumor as apocrine cystadenoma, and noted that the tumor was considered as an adenomatous cystic proliferation of apocrine glands and not a simple retention cyst. The first Japanese case was reported in 1968. So far, several review articles on Japanese cases of apocrine hidrocystoma have been published. Hosaka and Shishiba summarized 141 cases that appeared in the Japanese literature between 1968 and 1998. In the 5 years from 1999 to 2003, 25 new cases were reported in Japan. We counted a total of 167 Japanese cases of apocrine hidrocystoma, including the present case. Of these, 86 occurred in men and 80 in women (one was not described). The age distribution was as follows: < 10 years, four cases; 10–19 years, three cases; 20–29 years, 12 cases; 30–39 years, 28 cases; 40–49 years, 31 cases; 50–59 years, 35 cases; 60– 69 years, 30 cases; 70–79 years, 19 cases; > 80 years, four cases (one was not described). The tumor seems to occur in any age group. In 123 cases (73.7%), the tumor was found on the face or scalp (Table 1). The periorbital location seems to be the favored site. In only 15 cases (9.0%) was the tumor found in apocrine gland-bearing regions, such as the axilla and groin. Multiple apocrine hidrocystomas seem to be uncommon, as reported previously. Only two cases presented

Hidroacanthoma simplex: a case report and analysis of 70 Japanese cases.

Hidroacanthoma simplex (HS) is a rare benign eccrine adnexal tumor, which is often clinically misdiagnosed as seborrheic keratosis, Bowen’s disease or other adnexal tumors [1]. Here we report a case of HS that had been treated as chronic dermatitis for some years, and we analyze a total of 70 Japanese cases of HS. A 73-year-old woman presented with a 15-year history of a gradually growing cutaneous lesion on her left lower leg. The lesion had been treated with topical ointments under the diagnosis of chronic dermatitis for some years at another dermatologic clinic. At that visit the lesion was a well-defined, brownish but irregularly colored plain plaque with a few papules, measuring 20 ! 40 mm (fig. 1). A biopsy specimen was taken from the lesion under a diagnosis of Bowen’s disease. Histological examination revealed well-circumscribed intraepidermal nests of clear-looking basaloid cells (fig. 2a, b). A few small ductal structures and no clumping cells were observed. In an immunohistochemical study, epithelial membrane antigen and S100 protein were both negative, and keratin (polyclonal rabbit antihuman cytokeratin; Dako) and carcinoembryonic antigen were partially positive. We diagnosed this tumor as HS. Although a wide excision was recommended, our patient refused an operation. The patient has been carefully examined periodically. Hidroacanthoma simplex was first described by Smith and Coburn in 1956 [2]. The tumor is expressed as intraepidermal poroma, since nests of tumor cells are seen within the normal epidermal cells [3]. We counted a total of 69 Japanese cases appearing in the Japanese literature between 1983 and 2003. Among 70 cases, including ours, 19 occurred in men and 47 in women (4 cases were not described). The male-to-female ratio was 1:2.5. HS seemed to occur more often in females than in males. This differs from the previous report, which suggested there was no sex predilection in HS [1]. Ages ranged from 24 to 91 years in our study, with an average age of 65.8 years, and 80% of the patients were more than 50 years old. In the present study, HS occurred more often on the trunk (back 10, thorax 4, abdomen 6, buttock 10; 42.8%) and the lower extremities (thigh 16, lower leg 11, foot 5; 45.7%) than on the head and neck (2 cases; 2.9%) or the upper extremities (2 cases; 2.9%). Both the trunk and lower extremities seemed to be predilection sites. This result also differs from the previous report, which suggested that the limbs were more likely to be involved than the trunk or head [1]. The tumors varied in size from 4 ! 7 mm to 80 ! 90 mm, and they were colored light red, dark red, brown or dark brown. Their clinical appearance was reported as verrucous plaque, plain plaque, an aggregation of papules or a mixture of these. Most lesions were clinically diagnosed as seborrheic keratosis, Bowen’s disease, basal cell carcinoma or other adnexal tumors. No cases were diagnosed as HS clinically. It seems difficult to make a diagnosis of HS clinically, and this is suggestive of the existence of some other cases of HS that have been treated insufficiently like our case.

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.

Dermatomyositis associated with primary intramuscular B cell lymphoma.

A rare case of dermatomyositis associated with primary intramuscular malignant lymphoma is described. A 40‐year‐old Japanese woman noticed swelling of the right thigh during the treatment of dermatomyositis with prednisolone, azathioprine and cyclophosphamide. A biopsy specimen taken from the right quadriceps muscle revealed infiltration of lymphoma cells which were positive for CD20. We reviewed 12 cases of dermatomyositis associated with malignant lymphoma reported in Japanese literature between 1984 and 1996.

Postoperative Hyponatremia in a Patient with ACTH‐Producing Merkel Cell Carcinoma

Merkel cell carcinoma is characterized by specific neuroendocrine features and the expression of several neuropeptides. We report a case of Merkel cell carcinoma with post‐surgical hyponatremia in an 85‐year‐old Japanese woman. A tumor on the left cheek histopathologically showed the characteristics of Merkel cell carcinoma together with Bowens disease. Although an increased level of ACTH was found both in the tumor and in the peripheral blood, the postoperative hyponatremia in our patient seems more likely to have been caused by the stress of the operation and indapamide, considering that the ACTH level in the tumor was much lower than those in other ectopic ACTH‐producing tumors in previous reports.

Nucleotide Sequence and Phylogenetic Analyses of Dengue Type 2 Virus Isolated in the Dominican Republic

Dengue virus infection has been recognized as an important public health problem in the Dominican Republic in the last decade. Complete genomic sequences of three strains of dengue type 2 (DEN-2) virus, DR23/01 and DR31/01 isolated from dengue fever (DF) patients, and DR59/01 isolated from a dengue hemorrhagic fever (DHF) patient, all with primary infection, in the Dominican Republic in 2001, have been established. This achievement constitutes the first genomic characterization of DEN-2 strains from the Dominican Republic. No amino acid differences were observed between the strains isolated from DHF and DF patients. They exhibited extensive homology with the strain from La Martinique, French West Indies. Although phylogenetic analysis was suggestive of their Southeast Asiatic origin, Dominican Republic strains and other Caribbean strains from La Martinique and Jamaica showed 26 amino acid changes that differed from both the Southeast Asia and native American genotypes.

A case of rheumatoid nodulosis successfully treated with surgery.

We report the case of a 76 year‐old male with multiple subcutaneous nodules, but without joint symptoms or deformities, who was diagnosed histopathologically with rheumatoid nodulosis after resection of the nodules. Rheumatoid nodulosis is a disease characterized by multiple subcutaneous nodules that are histopathologically similar to rheumatoid nodules, a high titer of rheumatoid factor, and radiologically detectable cystic bone lesions, but with none or few of the systemic manifestations or joint activity of rheumatoid arthritis (RA). It is considered to be a benign variant of RA. This rare disease must be considered when a case of multiple subcutaneous nodules is encountered, even in aged males without joint symptoms or deformities.

Elevation of IL‐6 in ATL Patient with a Pathological Fracture

Hypercalcemia and osteolytic bone lesion are important complications in the prognosis of patients with adult T cell leukemia/lymphoma (ATL). We report a 61‐year‐old Japanese woman who died of ATL and had multiple osteolytic lesions and pathological fractures of her extremities. Highly increased serum levels of Interleukin‐6 (IL‐6) and a parathyroid hormone‐related protein (PTHrP) together with a high level of serum calcium observed at the time of fractures suggested their contribution to the formation of the bone lesions.

Skin Cancer Screening on a Fishing Island and in an Inland Agricultural Area of Japan

We performed skin cancer screening from 2000 to 2004 at two locations in Japans Oita Prefecture: Himeshima, a small fishing island, and Naoiri, an inland agricultural area. We found 108 and 21 cases of AK in Himeshima and Naoiri, respectively. None of the AKs transformed into SCC, and 21.7% of the AKs underwent spontaneous remission during our observation period. The prevalence and incidence of AK in Himeshima were five times higher than in Naoiri: 1,399 and 826 per 100,000 population, respectively, in the fishing village, vs. 261 and 164 in the agricultural community. Seven and three cases of BCC were observed in Himeshima and Naoiri, respectively. There were two cases of SCC in Himeshima. The highest risk ratio of skin types I to III was 9.2 in Himeshima. Although people engaged in outdoor occupations are thought to be more prone to skin cancer and precancerous skin lesions, our results suggested different potentials for AK in people engaged in different outdoor occupations.

Ga-67 citrate scintigraphy in Merkel cell carcinoma

Ga-67 citrate scintigraphy was performed on two patients with biopsy-proved Merkel cell carcinoma. Distinctly increased uptake was observed in the tumors in both patients. The tumor of one patient decreased strikingly in size after 50 Gy preoperative radiotherapy. Ga-67 scanning after radiotherapy did not demonstrate abnormal uptake in the residual tumor. Histologic examination of the resected specimen revealed no malignant cells but did show radiation-induced dense fibrous tissue.