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Arteriovenous malformation (AVM) is a structural vascular abnormality with no proliferation of cellular components. We report on a 53-year-old man who presented with a 15-year history of a progressively enlarging nodule on his lower lip. A dark-reddish, easy-bleeding nodule diagnosed as AVM was resected to reduce the volume and troublesome bleeding. Histologically, the nodule revealed that the proliferating cellular area was composed of endothelial cells and pericytes in addition to the area of dilated vessels. We speculated that the cell proliferation developed secondary to AVM. We also discuss the histological differential diagnosis of spindle cell hemangioma and pseudo-Kaposi's sarcoma.

Livedoid vasculopathy (LV) is thought to be a thrombogenic disorder. Here, we report a case of LV presenting livedo reticularis with leg ulcers clinically and many thrombogenic cutaneous vessels histologically. These features strongly suggested the presence of thrombogenic factors involved with the development of the lesions. After examination of various possible thrombogenic factors including phosphatidylserine-dependent anti-prothrombin antibody, we failed to detect any responsible thrombogenic factors for this case of LV. Recently, diverse thrombogenic factors have been reported to be involved in LV. This case may suggest that unknown thrombogenic factors are additionally related to LV pathogenesis.

We report a 58-year-old man with mycosis fungoides (MF) and occupational systemic sclerosis (SSc) induced by silica exposure. He was engaged in tunnel construction from the age of 18 to 33 years. He developed MF at the age of 30. Diagnosis of silicosis was made at the age of 52 and SSc at the age of 58. Physical examinations revealed sclerotic skin changes on his forearms and fingers and poikiloderma on the left popliteal fossa and inguinal region. Both antinuclear antibody and antitopoisomerase-I antibody were positive. We could find no apparent difference between his clinical features and those of idiopathic SSc except for the presence of silicosis and MF. Systemic therapy with interferon-gamma for MF did not improve the skin sclerosis. We discuss the relationship of silica exposure to both MF and SSc.

Bullous pemphigoid (BP) is an acquired autoimmune blistering disease which predominantly affects the elderly. It is rare in children and infants. We reported a 14-year-old girl presenting with a month history of relapsing tense bullae on the face and extremities. Histopathological examination of the lesional skin revealed a subepidermal bulla with infiltration of eosinophils, neutrophils, and lymphocytes. Direct immunofluorescence showed linear deposits of IgM and C3 at the basement membrane zone. Indirect immunofluorescence using normal human skin sections as a substrate detected IgG anti-basement membrane zone antibodies in the patient's serum and that using 1M NaCl split skin sections showed that the patient's antibodies bound to the epidermal side of the split skin. Immunoblot analysis using normal human epidermal extracts demonstrated the presence of autoantibodies against the 230-kDa BP antigen. Furthermore, the patient's serum reacted with the recombinant protein of the NC16a domain of the 180-kDa BP antigen by immunoblot analysis and enzyme-linked immunosorbent assay. Our patient showed significant improvement of the skin lesions with systemic administration of diaminodiphenyl sulfone.

We present a 79-year-old man who suffered from extramammary Paget's disease (EMPD) with bowenoid histological features accompanied by an ectopic EMPD lesion on his abdomen. He had had an erythematous plaque on his genital region for three years. Based on a biopsy specimen, he was referred to our hospital with the histological diagnosis of Bowen's disease. The histological findings of the genital lesion obtained by surgical resection showed typical areas of Paget's cells adjacent to areas characteristic of Bowen's disease. Immunohistochemical findings showed CEA and CK7 positive tumor cells in both areas, so the atypical cells showing the bowenoid pattern could be regarded as tumor cells of Paget's disease. Immunohistochemical staining for CEA and CK7 along with multiple biopsies can be helpful in making the diagnosis of Paget's disease with bowenoid histologic features.

Dermatofibroma is a common benign cutaneous tumor that usually appears as a slowly growing firm nodule. Polypoid nodular dermatofibroma is a variant type that is rarely encountered. We reported a case of polypoid dermatofibroma with a review of the previously reported cases. Polypoid dermatofibroma tends to arise on the leg, especially below the knee. Its size is often larger than that of common dermatofibroma. It is speculated that both the underlying firm tissue and long-term development may lead the tumor to form a polypoid appearance.

We report a 58-year-old man with mycosis fungoides (MF) and occupational systemic sclerosis (SSc) induced by silica exposure. He was engaged in tunnel construction from the age of 18 to 33 years. He developed MF at the age of 30. Diagnosis of silicosis was made at the age of 52 and SSc at the age of 58. Physical examinations revealed sclerotic skin changes on his forearms and fingers and poikiloderma on the left popliteal fossa and inguinal region. Both antinuclear antibody and antitopoisomerase-I antibody were positive. We could find no apparent difference between his clinical features and those of idiopathic SSc except for the presence of silicosis and MF. Systemic therapy with interferon-gamma for MF did not improve the skin sclerosis. We discuss the relationship of silica exposure to both MF and SSc.

The surgical treatment of large, deep defects on the midline of the lower back or lumbar area comprises difficult reconstructive challenges. Various flaps have been designed to reconstruct the defect area. We herein report a 70-year-old Japanese woman with a large, deep ulcer in the midline of the lower back, caused by postoperative radiation therapy for eccrine porocarcinoma. The ulcer was successfully treated with surgical debridement followed by reverse latissimus dorsi musculocutaneous flap. This flap is reliable and useful for reconstruction of a large, deep midline defect of the lower back, having large and bulky tissue with a sufficient blood supply.

A 34-year-old woman with adult-onset Still's disease (AOSD) developed prurigo pigmentosa-like lesions on her chest and upper back in addition to the typical rash of AOSD. A biopsy specimen taken from the upper back showed characteristic features of prurigo pigmentosa. The eruption and fever subsided immediately after the administration of 40 mg/day prednisolone, but arthralgia persisted even after intravenous pulse methylprednisolone therapy in combination with immunosuppressive drugs. Various atypical skin rashes, including prurigo pigmentosa-like lesions, have been reported in association with AOSD. Therefore, one should carefully follow the clinical course of a patient in order not to overlook these atypical cutaneous manifestations of AOSD.

Cholesterol crystal embolization (CCE) is characterized by tissue ischemia secondary to occlusion of small arteries. It may occur spontaneously but more often follows radiological interventional procedures or vascular surgery. This systemic disease affects multiple organs, including skin, kidney, brain, eye, and gastrointestinal tract. We reported a Japanese male CCE patient with cutaneous manifestations of livedo reticularis, diarrhea, clouding of consciousness, and acute renal failure. Histopathological examination demonstrated multiple biconvex clefts in a vessel of the subcutis. Corticosteroid administration improved his consciousness, diarrhea and skin lesions. Awareness of the skin manifestations of CCE is essential for dermatologists to make an early diagnosis and prescribe appropriate treatment.

Bowen's disease developing on mucous or mucocutaneous regions is clinically called erythroplasia of Queyrat. We report herein a 56-year-old male with Bowen's disease extending from the penis shaft to the glans penis, and urethral meatus. Physical examination revealed bright red velvety plaques on the prepuce and glans penis and an irregularly pigmented scaly lesion on the dorsum of his penis shaft. Histopathological findings of both lesions were compatible with those of Bowen's disease, supporting the concept that erythroplasia of Queyrat and Bowen's disease should be regarded as one clinicopathologic entity. A partial penectomy was finally performed, because tumor cells were pathologically observed in the mucous epithelium of the urethra. Although several therapeutic modalities exist for Bowen's disease on the external genitalia, treatment options are limited when Bowen's disease extends to the urethral meatus. We discussed the recent therapeutic modalities in genital Bowen's disease.

The report describes a woman with primary antiphospholipid antibody syndrome complicated with Grave's disease. Developing symptoms included a small cutaneous nodule on her finger and subsequently ecchymotic purpura on the cheeks, ears, buttocks and lower legs. Histological examinations showed thrombosed vessels in the dermis without or with hemorrhage, respectively. Laboratory investigation revealed positive lupus anticoagulant and immunogenic hyperthyroidism due to Grave's disease. There is a close relationship between the cutaneous manifestation of antiphospholipid antibody syndrome and the activities of Grave's disease and a possible link of antiphospholipid antibody syndrome with Grave's disease was suggested both by the etiology of the disease as well as the disease activity.