Dermatofibrosarcoma protuberans is a rare, slowly growing, locally invasive spindle-cell tumor characterized by a protuberant cutaneous mass with a pronounced tendency to recur and by a prominent storiform histopathologic pattern'-'. We present a case of dermatofibrosarcoma protuberans with myxoid area on the chest of a 57-year-old man. The histopathological study showed a dermal tumor of uniform spindle-shaped cells with storiform pattern. Immunohistochemically, the tumor was stained positively to anti-CD34 antibody and negatively to anti-factor XIIIa antibody.
Dermatofibrosarcoma* ; Factor XIIIa ; Humans ; Middle Aged ; Thorax

Papular xanthoma is a persistent normolipemic, nonconfluent, eruptive xanthomatosis located on the face, trunk and rnucous m,mbranes with no internal involvement, which exclusively occurs in adults. Histologically there is an infiltration of foamy histiocyte-like cells and a few Touton giant cells in the upper dermis and middermis without inflammatovy cells or a pure histiocytic component. Papular xanthomas in the childhood age group were recently described and characterized by their self-healing course, leaving, anetoderma-like scars. We report a case of papylar xanthoma in a 21-month-old infant with typical clinical, histopathologic and immunohistochemical findings. The positive immunohistochemical staining with factor XIIIa confirms that papular xanthorna is included in the group of dermal dendrocytic disorder.
Adult ; Cicatrix ; Dermis ; Factor XIIIa ; Giant Cells ; Humans ; Infant* ; Xanthomatosis*

Multiple histiocytomas are rare and defined when more than 15 lesions are present according to Baraf and Shapiro. It has been reported occurring in patients with autoimmune disease, who were receiving immunosuppressive treatment. Reports in the literature of these multiple lesions occurring in a healthy person are very rare. A 45-year-old man had 26 asymptomatic nodules on the trunk and upper extremities of two years duration. The lesions varied from 5mm to 2cm in size, were brownish, round, firm, nontender and positive for dimple signs. Microscopic examination of the lesions were indistinguish able from those seen in dermatofibroma. Most cells react intensively with factor XIIIa.
Autoimmune Diseases ; Factor XIIIa ; Histiocytoma* ; Histiocytoma, Benign Fibrous ; Humans ; Middle Aged ; Upper Extremity

BACKGROUND: Dermatofibroma (DF) comprises a heterogeneous group of mesenchymal tumors, with fibroblastic and histiocytic elements present in varying proportions. The cell of origin of DF has been investigated, but remains unclear. OBJECTIVE: The present study attempted to investigate the expression of leukocyte-specific protein 1 (LSP1), a marker of fibrocytes, in DF. Additionally, we evaluated the effectiveness of LSP1 in the differential diagnosis of DF from dermatofibrosarcoma protuberans (DFSP). METHODS: Immunohistochemical staining was performed on 20 cases of DF using antibodies against LSP1, CD68, and factor XIIIa (FXIIIa). In addition, the expression of LSP1 and FXIIIa was evaluated in 20 cases of DFSP. RESULTS: Eighteen of 20 cases (90%) of DF stained positive for LSP1, with variation in the intensity of expression. CD68 was positive in 10 cases (50%), and FXIIIa was expressed in all cases of DF. There were differences between the regional expression patterns of the three markers in individual tumors. In contrast, only 2 of 20 cases of DFSP expressed LSP1, and none of DFSP cases stained positive for FXIIIa. CONCLUSION: The LSP1-positive cells in DF could potentially be fibrocyte-like cells. FXIIIa and CD68 expression suggests that dermal dendritic cells and histiocytes are constituent cells of DF. It is known that fibrocytes, dermal dendritic cells and histiocytes are all derived from CD14+ monocytes. Therefore, we suggest that DF may originate from CD14+ monocytes. Additionally, the LSP1 immunohistochemical stain could be useful in distinguishing between DF and DFSP.
Antibodies ; Dermatofibrosarcoma ; Diagnosis* ; Diagnosis, Differential ; Factor XIIIa ; Fibroblasts ; Histiocytes ; Histiocytoma, Benign Fibrous* ; Langerhans Cells ; Monocytes

Dermatofibrosarcoma protuberans (DFSP) is a dermal spindle cell neoplasm of intermediate malignancy. It typically forms a brown indurated plaque on which firm nodules subsequently arise, sometimes with ulceration. Atypical DFSP presentations are not unusual, including atrophic, pedunculated, morphea-like and angioma-like forms. However, subcutaneous variant of DFSP that may either arise without dermal involvement or with minimal dermal involvement is very rare. A 36-year-old man presented with a palpable nodule without surface change around the right nipple. Microscopically, the neoplasm was composed of spindle cells with monomorphic storiform arrangement. The superficial part of the neoplasm was located in the subcutaneous tissue. Immunohistochemical staining showed strong cytoplasmic positivity for CD34, but not for factor XIIIa. Dermatologists should pay careful attention to these unusual variant of DFSP, which can be confused with other soft tissue tumors.
Adult ; Breast ; Cytoplasm ; Dermatofibrosarcoma ; Factor XIIIa ; Humans ; Nipples ; Subcutaneous Tissue ; Ulcer

Bednar tumor is a variant of dermatofibrosarcoma protuberans (DFSP). The clinical and histopathological findings of Bednar tumors are identical to DFSP except for the presence of melanin- containing cells scattered within the lesion, so called pigmented DFSP. There are no known precipitating or predisposing factors associated with this tumor. A 30-year-old woman was presented with a tumor on the right upper arm arising after trauma at the site of prior vaccination. Histologically, it shows large uniformed spindle shaped cells arranged in a cartwheel or storiform pattern mixed with scattered pigmented cells. On immunohistochemical staining, the tumor cells were positive for vimentin and CD34, but negative for factor XIIIa and S-100 protein. The patient was treated with a wide excision and skin graft. We report a case of Bednar tumor occuring after trauma at the site of prior vaccination.
Adult ; Arm ; Causality ; Dermatofibrosarcoma ; Factor XIIIa ; Female ; Humans ; S100 Proteins ; Skin ; Transplants ; Vaccination* ; Vimentin

A Bednar tumor is a variant of dermatofibrosarcoma protuberans(DFSP). The clinical and histopathological findings of Bednar tumors are identical to DFSP except for the presence of melanin-containing cells scattered within the lesion, so called pigmented DFSP. The majority of Bednar tumors are seen as DFSP present as multinodular protuberant masses in the skin. They can also present as an atrophic depressed scar-like lesions without any nodularity. We report an uncommon clinical presented case of a Bednar tumor on the back in a 22-year-old female. She presented with an asymptomatic, firm, bluish, chestnut sized, depressed and atrophic lesion on the back which had been present for 6 years. A Histopathological examination revealed massive proliferation of spindle-shaped cells arranged in a tight storiform pattern mixed with scattered pigmented cells. On immunohistochemical staining, the tumor cells were positive for vimentin, a -l-antitrypsin, and CD34, but were negative for cytokeratin, neurofilament, and factor XIIIa. The majority of the tumor cells was negative and the pigment cells were positive for the S-100 protein. The patient was treated by a wide local excision of the lesion. There has been no evidence of recurrence after 20 months post-operative follow up.
Dermatofibrosarcoma* ; Factor XIIIa ; Female ; Follow-Up Studies ; Humans ; Keratins ; Recurrence ; S100 Proteins ; Skin ; Vimentin ; Young Adult

BACKGROUND: Dermatofibrosarcoma protuberance (DFSP) must be differentiated from dermatofibroma (DF). However, especially in cases of superficial biopsy and cellular dermatofibroma, this is difficult by using histopathology alone since both are composed of neoplastic spindle cells. Although a panel of immunostains is useful, the expressions of conventional markers often overlap. A previous study showed that novel D2-40 immunostain may be useful for differentiating between DF and DFSP. OBJECTIVE: To evaluate the usefulness of D2-40 immunohistochemical staining for differentiating DFSP from DF and compare the results with other commonly used immunostains (CD34 and factor XIIIa). METHODS: Twenty-eight cases of DF and 15 cases of DFSP were selected from clinicopathologically proven cases reviewed by the Department of Dermatology at our medical center and Daegu Catholic University Medical Center. D2-40, CD34, and factor XIIIa immunohistochemical staining was performed. The immunopositivity was measured throughout the entire lesion. RESULTS: Seventeen cases (60.7%) of DF and no cases of DFSP showed immunoreactivity to D2-40 in the spindle cells. Three (10.7%) cases of DF and 13 (86.7%) cases of DFSP showed immunoreactivity to CD34 in the spindle cells. Twenty-five (89.3%) cases of DF and four (26.7%) cases of DFSP showed immunoreactivity to factor XIIIa in the spindle cells. A total of 60.7% of cases of DF were positive on D2-40 staining, 89.3% were negative on CD34 staining, and 89.3% were positive on factor XIIIa staining. All cases (100%) of DFSP were negative by D2-40 staining, 86.7% were positive by CD34 staining, and 73.3% were negative by factor XIIIa staining. CONCLUSION: D2-40 immunostaining may be useful for distinguishing between DF and DFSP since the immunoreactivity of DF was significantly higher than that of DFSP (p=0.001). However, the results of our study were not as useful as those of a previous study. Therefore, further studies are needed to address this issue.
Academic Medical Centers ; Biopsy ; Daegu ; Dermatofibrosarcoma* ; Dermatology ; Factor XIIIa* ; Histiocytoma, Benign Fibrous*

We report a case of AIDS-related Kaposi's sarcoma in a 47-year-old male. He was diagnosed as HIV-positive 3 years ago. He developed two asymptomatic purple-colored papules on the nose and right elbow. The histopathological finding showed abnormally proIiferated and dilated vessels, vascular slits, spindle shaped cells, and extravasated erythrocytes. In immunohistochemical studies, the cryptic vessels was positive with CD34, but negative with factor XIIIa and factor VIII-related antigen, Human herpesvirus-8 was found by PCR. We think that this is the first reported case of AIDS-related Kaposi's sarcoma in Korean dermatologic literature.
Elbow ; Erythrocytes ; Factor XIIIa ; Humans ; Male ; Middle Aged ; Nose ; Polymerase Chain Reaction ; Sarcoma, Kaposi* ; von Willebrand Factor

BACKGROUND: Dermatofibroma (DF) is one of the most common benign soft tissue tumors, and its diagnosis is not difficult if clinicopathologic features are typical. However, DF occurring on the face is very rare; therefore, it is usually missed clinically. OBJECTIVE: This study was conducted to obtain better understanding of the clinicopathologic features of dermatofibroma of the face. METHODS: This is a retrospective study of fibrous histiocytoma of the face at our center over a 23-year period (1989~2011). Clinicopathologic features of 13 patients were evaluated. RESULTS: Of the 13 patients, ten were female and three were male. The neoplasms presented with various and atypical features, such as nodule, ulceration and papules. Low-power examination revealed that most of the cases were extended beyond the subcutaneous fat layer, showing ill-defined diffuse infiltrative pattern. The most common histologic type was typical fibrocollagenous type, but some cases presented features of cellular or angiomatous type. Mitotic activity was not definite in majority of cases, and usually ranged 0~1 mitoses per 10 HPF and a few atypical cells were shown in 2 cases, but not accompanied by recurrence. Tumor cells in all cases tested were negative for desmin and CD34, but positive for Factor XIIIa and CD68 in majority of the cases. CONCLUSION: Because of its rare development on face and diverse clinical presentation, correct diagnosis with differential diagnosis is thought to be important. DF of the face usually presents with infiltration of deeper structures and still shows a benign behavior.
Desmin ; Diagnosis, Differential ; Factor XIIIa ; Female ; Histiocytoma, Benign Fibrous ; Humans ; Male ; Mitosis ; Recurrence ; Retrospective Studies ; Subcutaneous Fat ; Ulcer