Extramammary Paget's Disease of External Genitalia with Bowenoid Features

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.

Extramammary Paget's Disease of External Genitalia with Bowenoid Features

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.

A Clinical Study of Cutaneous Adverse Reactions to Nonionic Contrast Media in Korea

BACKGROUND: The use of intravenous contrast media (CM) has increased for the diagnosis of several diseases. The newly developed low osmolar nonionic contrast agents cause significantly decreased adverse reactions than the higher osmolar ones. However, adverse reactions may still occur, ranging in severity from minor side effects to severe complications. However, there have been few reports about cutaneous adverse reactions (CARs) to nonionic monomer CM. OBJECTIVE: The purpose of this study was to evaluate clinical features of CAR to intravenous nonionic monomer CM. METHODS: A total 47,338 examinees underwent intravenous iodinated contrast-enhanced computed tomography scan using nonionic monomer CM. Among the adverse reactions to the CM, we divided them into cutaneous or noncutaneous and immediate ( or =1 hr) adverse reactions. RESULTS: Adverse reactions were noted in 62 cases out of the total 47,338 cases; 50 cases (80.7%) were categorized CARs. Among them, there were 24 male and 26 female patients. There was no significant difference between the sexes, and CARs occurred in all age groups. The highest occurrence was in the age range of 50~59 years. CARs included urticaria (78%), angioedema (10%), maculopapular rash (8%), erythema (2%), and pruritus without rash (2%). Immediate reactions were 92% (46 cases), while late reactions were 8% (4 cases). CONCLUSION: CARs to nonionic monomer CM accounted for most of the adverse reactions (80.7%) and urticaria was the most common.

Bilateral Collagenous Fibroma Occurred on Both Anterior Superior Iliac Spine / 대한피부과학회지

Collagenous fibroma is a recently described rare benign tumor that mainly affects those in the fifth through seventh decades. It is a painless solitary mass, which grows slowly for more than 6 months, up to 20 cm. Several cases were reported chromosomal rearrangements with a breakpoint at 11q12. Histopathologically, it shows well-circumscribed mass that consists of scattered stellate cells in hypovascular collagenous matrix without atypia or mitosis. Management method is excision with functional preservation. In Korea, only two cases of collagenous fibroma have been reported so far. A 69-year-old woman was presented to our clinic with two painless well-demarcated hard masses, 4.5x4.5 and 4.5x2.5 cm in size, on both anterior superior iliac spine. Traumatic injury or a previous event was not noticed by the patient. After histopathologic examination, those masses were diagnosed as collagenous fibroma.

Bilateral Collagenous Fibroma Occurred on Both Anterior Superior Iliac Spine / 대한피부과학회지

Collagenous fibroma is a recently described rare benign tumor that mainly affects those in the fifth through seventh decades. It is a painless solitary mass, which grows slowly for more than 6 months, up to 20 cm. Several cases were reported chromosomal rearrangements with a breakpoint at 11q12. Histopathologically, it shows well-circumscribed mass that consists of scattered stellate cells in hypovascular collagenous matrix without atypia or mitosis. Management method is excision with functional preservation. In Korea, only two cases of collagenous fibroma have been reported so far. A 69-year-old woman was presented to our clinic with two painless well-demarcated hard masses, 4.5x4.5 and 4.5x2.5 cm in size, on both anterior superior iliac spine. Traumatic injury or a previous event was not noticed by the patient. After histopathologic examination, those masses were diagnosed as collagenous fibroma.

Systematized Inflammatory Linear Verrucous Epidermal Nevus / 대한피부과학회지

Inflammatory linear verrucous epidermal nevi (ILVEN) were first described in the literature, in 1971, by Altman and Mehregan. Most cases were reported as solitary lesions. In contrast, systematized ILVEN, involving wide areas of the integument, has only rarely been reported. A variety of treatment modalities has been reported, ranging from topical medications, such as potent corticosteroids or tretinoin 0.1% to variable procedures, including CO2 and pulsed dye laser, cryotherapy, and surgical excision. However, multifocal skin lesion is more difficult to treat. An 8-year old boy was presented with an extensive thick scaly plaques and patches, affecting the trunk and the four extremities sparing face. It developed when he was 3 years old, and he complained pruritus. On physical examination, linear, or whirl-like scaly plaques were seen, along with Blaschko lines. Pathologic finding was in accordance to ILVEN. We treated him with topical calcipotriol, tacrolimus and systemic acitretin. After 8 months, the lesion and symptoms improved.

Recurrent Primary Cutaneous Adenoid Cystic Carcinoma along an Abdominal Surgical Scar after Repeated Simple Excisions / 대한피부과학회지

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.

Deep Penetrating Benign Fibrous Histiocytoma of the Foot Associated with Throbbing Pain

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.

Large Dermal Non Neural Granular Cell Tumor on the Surgical Wound Site

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.

Non-familial Generalized Hypotrichosis Simplex / 대한피부과학회지

Hypotrichosis simplex is a descriptive term of hair loss without other ectodermal or systemic abnormalities. Hypotrichosis simplex with non-familial and generalized types has been seldom reported. A 30-year-old man visited our clinic complaining of scalp hair loss since birth. There was no hair on the arms, axillae and legs. He had relatively scanty eyebrows and pubic hairs. None of his family members had known health problems or any hereditary disease. Hair shaft examination based on electron microscopy did not reveal any structural abnormalities. Microscopic examination of a scalp biopsy specimen showed a reduced number of hair follicles with vellus hairs replacing terminal hairs. Herein we report a rare and interesting case of non-familial generalized hypotrichosis simplex.

A Case of Familial Comedonal Darier's Disease

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.

A Case of Gardner's Syndrome Associated with Desmoid Tumor

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.

Dystrophic Calcinosis Cutis with Transepidermal Elimination Secondary to Localized Scleroderma / 대한피부과학회지

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.

Using a Subcutaneous Island Pedicle Flap and Periosteal Suspension Suture Following Mohs Surgery for Basal Cell Carcinoma Involving the Nose and Nasolabial Fold / 대한피부과학회지

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.

A Case of Eccrine Spiradenoma in a Patient with Neurofibromatosis

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.

A Case of Generalized Granuloma Annulare That Clinically Presented as Erythema Annulare Centrifugum / 대한피부과학회지

Granuloma annulare (GA) is usually a benign, self-limiting, granulomatous, chronic dermatosis of an unknown cause, characterized by skin colored papules that often have an annular configuration. Generalized granuloma annulare (GGA) is an uncommon variant of GA, and this is characterized by disseminated papules on the trunk and limbs. We report here on an interesting case of GGA that clinically presented as erythema annulare centrifugum.

Two Cases of Trichorrhexis Nodosa / 대한피부과학회지

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.