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.
Darier Disease ; Keratins ; Skin ; Wills

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 cafe-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.
Head ; Humans ; Melanoma ; Nervous System ; Neurofibromatoses ; Neurofibromatosis 1 ; Pheochromocytoma ; Skin ; Xanthogranuloma, Juvenile

Localized scleroderma is a connective tissue disorder characterized by thickening and fibrosis of the skin due to excessive collagen deposition in the dermis. Dystrophic calcinosis occurs when calcium is deposited in the previously damaged skin tissue, and calcium and phosphorous levels in the blood are within normal limits. It occurs secondary to connective tissue disease, infection, inflammatory processes, chronic venous stasis, cutaneous neoplasm, and trauma. We report a rare case of dystrophic calcinosis cutis with transepidermal elimination secondary to localized scleroderma.
Calcinosis ; Calcium ; Collagen ; Connective Tissue ; Connective Tissue Diseases ; Dermis ; Fibrosis ; Scleroderma, Localized ; Skin

Primary cutaneous adenoid cystic carcinoma is a rare, slow-growing malignancy that consists of basaloid cells. It characteristically follows an indolent course but has a high tendency to recur locally after excision. We experienced a recurrent primary adenoid cystic carcinoma of the abdomen in a 69-year-old female. It had recurred locally three times over 13 years despite repeated excisions. Wide spreading and perineural invasion of tumor cells were identified during Mohs micrographic surgery, and it seemed to be related to the repeated recurrences. Herein, we report this rare case with clinical and histological features of a recurrent nature.
Abdomen ; Adenoids ; Aged ; Carcinoma, Adenoid Cystic ; Cicatrix ; Female ; Humans ; Mohs Surgery ; Recurrence

Trichorrhexis nodosa is the most common hair shaft anomaly in which there is a chemical or physical traumatic injury, characterized by the development of node-like swelling, fragility of the shaft and paint brush effect on the side of the fracture. We report two cases of trichorrhexis nodosa in 15-year-old girl and 16-year-old-boy, occurring as results of repetitive hot-comb straightener and excessive scratching. Scanning electroscopic findings are typical to trichorrhexis nodosa.
Adolescent ; Hair ; Humans ; Paint

Skin cancers usually occur on the face and so successive cutaneous reconstruction after surgery is very important for the patients' quality of life. Cutaneous reconstruction is especially difficult when the defects after surgery involve the nasolabial fold. The nasolabial folds run from each side of the nose to the corners of the mouth, separating the cheeks from the upper lip. This area has complex contours, unique skin color and texture and the limited availability of mobile adjacent skin. We reconstructed such a defect after Mohs micrographic surgery for basal cell carcinoma of the nasolabial fold area, and we used a subcutaneous island pedicle flap and periosteal suspension suture. Herein we report on the details with the pictures, and we discuss the cosmetic effect and limitations of this method.
Carcinoma, Basal Cell ; Cheek ; Cosmetics ; Lip ; Mohs Surgery ; Mouth ; Nasolabial Fold ; Nose ; Quality of Life ; Skin ; Skin Neoplasms ; Sutures

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.
Analgesics ; Female ; Foot ; Histiocytoma, Benign Fibrous ; Humans ; Muscle, Smooth ; Walking

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.
Aged ; Cicatrix ; Coloring Agents ; Cytoplasm ; Cytoplasmic Granules ; Eosinophils ; Granular Cell Tumor ; Humans ; Phosphopyruvate Hydratase ; S100 Proteins

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.
Adenocarcinoma ; Bowen's Disease ; Carcinoma, Squamous Cell ; Epidermis ; Genitalia ; Genitalia, Male ; Male ; Paget Disease, Extramammary ; Pluripotent Stem Cells ; Skin

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.
Adenocarcinoma ; Bowen's Disease ; Carcinoma, Squamous Cell ; Epidermis ; Genitalia ; Genitalia, Male ; Male ; Paget Disease, Extramammary ; Pluripotent Stem Cells ; Skin