[A case of chromomycosis caused by Fonsecaea pedrosoi presenting as a small plaque on the left upper arm: a review of reported cases of dematiaceous fungal infection in Japan].

We report a case of a 67-year-old woman with chromomycosis on the left upper arm. The plaque was a very small, erythematous and scaly lesion with a diameter of 1.5 cm. Fonsecaea pedrosoi was isolated as the causal fungus, and a number of Phialophora type conidia, the formation of which is considered rare, were observed. Treatment involved surgical excision of the lesion with a 5 mm margin. Follow up three years later revealed no recurrence. In Japan, 536 patients with chromomycosis were reported from 1955 to 2004. This consisted of 296 cases from 1955 to 1981 as reported by Fukushiro, and 240 cases from 1982 to 2004 as reviewed by us. Our examination of data showed that the most common causal fungi was F. pedrosoi with 137 cases (57.15%), followed by Exophiala jeanselmei with a total of 41 cases (17.15%), other fungal species comprised of 16 cases (6.7%), Phialophora verrucosa in 9 cases ( 3.8%) and E. dermatitidis in 4 cases (1.7%). Compared to the previous report by Fukushiro, the incidence of infection with E. jeanselmei had increased. Of the 235 cases we reported, the site of infection involved: upper extremities in 91 (38.7%), face and neck in 42 (17.9%), buttocks in 41 (17.4%), lower extremities in 33 (14%) and body in 23 (9.8%). Compared to Fukushiro's report, cases affecting the lower extremities had decreased, whilst cases involving the buttocks had increased. Overall, the treatment for chromomycosis was either oral administration of antifungal agents, excision, thermotherapy, or a combination of these methods.

A case of diffuse plane normolipemic xanthomatosis associated with pancytopenia and monoclonal gammopathy.

We report a case of diffuse plane normolipemic xanthomatosis (DPNX) which showed poorly demarcated, uncommon, yellow macules symmetrically distributed on the nape, axillae and inguinal folds accompanied by severe, persistent itching. Histopathological and ultrastructural studies of skin biopsy specimens revealed the existence of some foamy cells and the deposition of neutral fat in the upper papillary dermis. Laboratory investigations and bone marrow aspirate smears showed that our patient had myelodysplastic syndrome (MDS) associated with pancytopenia and monoclonal gammopathy of undetermined significance. Because our patient had neither a malignant hematological disorder nor a severe systemic disease, monoclonal gammopathy might explain the pathogenesis of DPNX in the present case.

Electron microscopic study in a case of solitary mastocytoma.

The authors report a solitary mastocytoma with a solitary red infiltrated plaque on the dorsum of the right foot for 2 months. Histologically there were numerous mast cells infiltrating the dermis. Electron microscopy revealed CLCs located in phagosomes of activated macrophages as well as in the stromal tissue, close association between CLCs formation and damaged eosinophils was documented Charcot-Leyden crystals (CLCs) have been found in many conditions associated with eosinophilia, but their occurrence in skin diseases is very rare. These occurrences showed the evidence that the formation of CLCs in a mastocytoma correlated to the individual and related to the biology of mast cells, basophils, eosinophils and macrophages. Phagosomes probably acted as the localization of CLCs formation. The pathological role of CLCs in a mastocytoma needs further investigation.

A case of unilateral, systematized linear hair follicle nevi associated with epidermal nevus-like lesions.

Multiple hair follicle nevi are an extremely rare condition. In 1998, a case of unilateral multiple hair follicle nevi, ipsilateral alopecia and ipsilateral leptomeningeal angiomatosis of the brain was first reported from Japan. Very recently, hair follicle nevus in a distribution following Blaschko's lines has also been reported. In this paper, we observed a congenital case of unilateral, systematized linear hair follicle nevi associated with congenital, ipsilateral, multiple plaque lesions resembling epidermal nevi but lacking leptomeningeal angiomatosis of the brain. These cases implicate the possibility of a novel neurocutaneous syndrome. Additional cases should be sought in order to determine whether this condition is pathophysiologically distinct.

Immunohistochemical localization of cathepsin L and cystatin A in normal skin and skin tumors.

Cathepsin L, a cysteine proteinase, and cystatin A, an inhibitor of cysteine proteinases, are thought to regulate the invasion and metastasis of malignant cells. In this study, the expression of cathepsin L and cystatin A in skin tumors was investigated immunohistochemically in order to examine the relationship between these two enzymes in the pathophysiology of malignant cells. Formalin-fixed and paraffin embedded specimens from normal skin, seborrheic keratoses, and squamous cell carcinomas were reacted with polyclonal antibodies against rat cathepsin L or cystatin a which cross-react to human cathepsin L and cystatin A, respectively. The consequent immunostaining of these enzymes was observed to be strong in normal skin (4 cases) and seborrheic keratosis (6 cases). In well-differentiated squamous cell carcinoma (SCC) (9 cases), staining for cathepsin L and cystatin A was moderately positive in differentiated tumor cells and negative in undifferentiated SCC (5 cases). The degree of staining of these enzymes was inversely correlated with the differentiation of the malignant cells. These results suggest that the immunohistochemical analysis of cathepsin L and cystatin A is a useful indicator for an aspect of malignancy in human epidermal keratinocytes.

The ultrastructural study in a case of Fabry disease.

An investigation of the ultrastructural study was conducted on specimens from a typical patient with Fabry disease. Numerous characteristic cytoplasmic inclusions were observed in the vascular endothelial cell, pericyte, smooth muscle cell, nerve and eccrine sweat glands, the lamellar pattern of which were considerably variable in various types of gland cells. Large vacuolar inclusions predominated in clear cells of the secretory coil; lesser vacuoles were also seen in the coiled duct, and the basal cells of the straight duct toward the coiled duct displayed mulberry-like figures. There were some clear cells showing cell damage and necrosis in the secretory coil. Lamellated bodies were noted in the axons and schwann cells around the eccrine sweat glands. The small blood vessels around the eccrine glands were narrowed by swollen endothelial cells with heavy inclusions. These intracytoplasmic deposits may be responsible for the decreased sweating ability in Fabry disease. The factors related to hypohidrosis are also discussed.