Ken-ichi Kayashima

Two Cases of Pilomatrixoma in Turner’s Syndrome

Multiple and familial cases of pilomatrixomas have been reported in myotonic dystrophy. We report 2 cases of pilomatrixoma in Turner’s syndrome. Between 5 and 19 years of age, 1 patient developed 6 pilomatrixomas including atypical occurrences on the chest and the posterior aspect of the leg. This is the first report of multiple pilomatrixomas in Turner’s syndrome.

Regressing Merkel cell carcinoma-a case showing replacement of tumour cells by foamy cells.

Merkel cell carcinoma (MCC) is a malignant neuroendocrine tumour with a high rate of recurrence and metastasis. However, some cases of spontaneous regression have recently been reported. We describe the clinical course of an 80‐year‐old Japanese woman with regressing MCC. We also report histological findings of the regressing tumour for the first time. After the patient’s first visit to our hospital, the lesion was a rapidly progressive tumour, but suddenly began decreasing in size, and rapidly regressed. The surface of the tumour flattened, the colour changed from red to dark red, and finally the lesion appeared as a small yellowish plaque. Histopathological analysis of the completely regressed tumour revealed that the tumour cells were completely replaced by numerous foamy cells. This is the first report demonstrating the histopathological features of regressing MCC.

Expression of neuronal nitric oxide synthase in dermal infiltrated eosinophils in eosinophilic pustular folliculitis

Nitric oxide (NO) is produced by a wide variety of human cells and affects physiological and pathophysiological processes. In this study, we demonstrated for the first time that neuronal NO synthase (NOS) is expressed in eosinophils infiltrating into the dermis and follicular epidermis in eosinophilic pustular folliculitis (EPF). EPF is a cutaneous inflammatory follicular disorder first described in Japan. The clinical and histological features of EPF are characterized by erythematous papules, infiltration of numerous eosinophils, and a spongiotic appearance of the follicular epidermis, but the pathophysiology of EPF remains unclear. Our results suggest that NO produced from eosinophils plays an important part in the pathogenesis of EPF. Furthermore, we speculate that NOS inhibitors may be useful in the management of EPF.

Late onset dermal melanocytosis: an upper back variant.

Seven elderly Japanese men with blue macules on their upper backs are reported. Histological examination showed this pigmentation to be melanosis due to the presence of spindle‐shaped or oval pigment‐laden cells in the dermis. Electron microscopy revealed the cells to be compatible with dermal melanocytes. We assume that this condition belongs to the category of late onset dermal melanocytosis and is an upper back variant. This concept has not been proposed before.

Giant Basal Cell Carcinoma Associated with Systemic Amyloidosis

A large basal cell carcinoma, 39 times 26 cm in size, is presented as second in size only to the largest basal cell carcinoma documented (40 times 30 cm), reported by Beck and co‐workers (1). A 61‐year‐old Japanese male visited our clinic with a huge ulcerating tumor on the back. He had hidden the tumor for the previous 30 years. The tumor was histologically confirmed as basal cell carcinoma. The condition was associated with anemia, hypoproteinemia, and dyspnea, and with systemic amyloidosis in the skin, in the lymph nodes, and in the intestinal canal. On admission, the tumor had metastasized to the regional lymph nodes, and, about two years after the first operation, there were metastases to bone and lung, leading to death due to respiratory failure.

Hereditary perioral pigmented follicular atrophoderma associated with milia and epidermoid cysts.

Eight members of a single family all presented the characteristic changes of facial, especially perioral, pigmented follicular atrophoderma, with numerous milia and epidermoid cysts. For this condition, diagnosis at a glance may be possible because of the perioral cutaneous manifestations. Histopathological examination of follicular atrophoderma revealed proliferation of basaloid cells continuous with the epidermis and coarse collagen fibres, with a decreased density of elastic fibres around the basaloid cells. Two of the eight individuals also showed generalized hypohidrosis. The eight affected persons were the proband, her son, mother, uncle, two younger sisters, cousin and nephew; an autosomal dominant mode of transmission was suggested from this family tree. The patients’ symptoms resembled those of Bazex–Dupre´–Christol syndrome, except for the different distribution of the follicular atrophoderma and the absence of basal cell carcinoma and hypotrichosis. This disease may be an entirely new syndrome characterized by perioral pigmented follicular atrophoderma associated with milia and epidermoid cysts.

Metastatic skin cancer : a case with signet ring cell histology

Summary We report a 64‐year‐ old Japanese man who developed metastatic skin cancer, in the form of 1–3 cm diameter dome‐shaped tumours on his face and head. Histopathological examination demonstrated diastase‐resistant periodic acid‐Schiff and Alcian blue‐positive signet ring cells, suggesting gastric carcinoma. Immunohistochemical staining showed that these cells were positive for carbohydrate antigen CA19‐9.

A Case of Superficial Leiomyosarcoma, and a Review of Japanese Cases

We report a case of leiomyosarcoma in a 55‐year‐old Japanese female. She developed an erythematous nodule, 1.5 cm in diameter, on her right thigh. The totally excised specimen contained tumor cells with variously sized, chromatin‐rich nuclei. Gitter staining revealed the so‐called encased picture, immunohistopathological staining was positive for smooth muscle actin, and electron microscopy revealed filaments with focal densities. A diagnosis of leiomyosarcoma was made based on these findings, which suggested a muscle cell origin. Normal arrector pili muscles were continuous from the tumor, further suggesting the tumors origin. The presence of Epstein‐Barr virus‐encoded RNA (EBER) was examined by in situ hybridization with negative results.

Larva currens: The Usefulness of the Agar Plate Method

Creeping eruption caused by Strongyloides stercoralis is called larva currens, while those caused by other parasites are called larva migrans. To detect the parasite, the direct smear method was used. However, this method is not very sensitive in detecting S. stercoralis. A 62-year-old male with polyarteritis nodosa cutanea had an erythematous creeping eruption in various regions, such as his lumbar region, abdomen and thigh. The diagnosis of larva currens was made by the clinical appearance and the detection of S. stercoralis in his feces using the direct smear method and the agar plate method. This is the first reported case of larva currens in Japan. The agar plate method was useful and more sensitive than the usual direct smear method for detecting the S. stercoralis in feces and for elucidating the effects of treatment with antiparasitic drugs.

BCC-associated amyloidosis with a peculiar pattern of deposition.

An 83‐year‐old Japanese woman with lepromatous leprosy had been treated in a leprosarium. More than 10 years ago, she developed a dome‐like brown tumor on the dorsum of the nose which showed the histology of basal cell carcinoma. The lesion was a well bordered, rounded tumor with tumor cell nests on its periphery extending toward the center as cords of tumor nest and with amyloid depositions between the cords. The tumor nests and amyloid lessened toward the center of the tumor, being replaced by collagen fibers. Amyloidosis showing this peculiar pattern of transition was reported with a review of published cases.