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Extramammary Paget's disease (EMPD) is an uncommon intraepithelial adenocarcinoma, primarily affecting the apocrine-bearing skin. Bowen disease is an intraepithelial squamous cell carcinoma having the potential to become invasive carcinoma. The histopathological concomitant features between EMPD and Bowen disease have been described. One theory is that primary EMPD arises multicentrically, within the epidermis from the pluripotent stem cells. Herein, we describe a case of EMPD that had bowenoid features, and review the previous cases associated with the origin of EMPD.

Darier's disease is a genetic disorder of keratinization with autosomal dominant inheritance. Its appearance is usually in the form of greasy, crusted, keratotic yellow-brown papules and plaques found particularly on seborrheic areas of the body. However, there are some clinical variants showing atypical skin lesions. Here we report an unusual case of Darier's disease, which mainly showed prominent comedonal papules over the face.

Granular cell tumors (GCTs) can be divided into neural type with S-100 reactivity and non-neural type without that. The latter has not been widely recognized and there are only fewer reports available when compared to conventional GCT. A 65-year-old man was presented with the presence of a painless mass on his back. The mass had developed into a small nodule on the scar developed because of previous surgery carried out 2 years ago. The tumor consisted of large, polygonal cells comprising of an enormous number of faintly eosinophilic small granules in the cytoplasm. The cytoplasmic granules were stained positively for periodic acid-Schiff stain. Immunohistochemical stains for S-100 protein and neuron-specific enolase were found to be negative. Herein, we report the appearance of a very rare case of non neural GCT developed on the surgical scar in support with relevant literature reviews.

Compared to cutaneous benign fibrous histiocytoma (BFH), deep-seated BFH is very rare and poorly recognized. Both cutaneous and deep-seated BFH are usually asymptomatic. We herein report a 25 year-old woman who presented with a painful mass in her foot that was poorly controlled by analgesics and associated with walking difficulty. After preoperative ultrasonographic evaluation, the mass was completely excised and histologic exam showed spindle cells loosely arranged in storiform architecture, with CD34(-), desmin(-), S-100(-), focal CD68(+), vimentin(+), smooth muscle actin(+), and factor XIIIa(+). The patient was diagnosed with deep-seated BFH based on the histologic, radiologic and intraoperative findings.

Desmoid tumors are uncommon benign neoplasm of the fibroblasts. They occur rarely in the general population, but they are comparatively common in patients with familial polyposis coli with or without other elements of Gardner's syndrome. Herein, we report a 16-year-old woman with Gardner's syndrome complicated by desmoid tumors on the right subscapular area.

Neurofibromatosis is a systemic hereditary disorder with varied manifestations in bone, soft tissue, the nervous system and skin. Cutaneous manifestations of neurofibromatosis are characterized by café-au-lait macules, multiple neurofibromas, Lisch nodules and intertriginous freckling. Some benign or malignant tumors such as juvenile xanthogranuloma, pheochromocytoma, and malignant melanoma can accompany neurofibromatosis. But, in the English literature, no case of eccrine spiradenoma associated with neurofibromatosis has been reported. Eccrine spiradenoma is a benign uncommon neoplasm of skin adnexa. It presents as a painful, slow-growing and solitary nodule on the head or upper trunk. Here, we report a rare case of eccrine spiradenoma in a patient with neurofibromatosis.