1/22. Expression of CD34 in sclerotic ("plywood") fibromas.CD34 antigen is expressed in normal human skin on endothelium, in spindle cells located around adnexal structures, and in a subset of interstitial cells in the reticular dermis. CD34 expression has also been identified in a number of fibrohistiocytic neoplasms, such as dermatofibrosarcoma protuberans and solitary fibrous tumors of soft tissue. CD34 expression has not previously been described in sclerotic, or "plywood" fibromas. Here presented are three lesions from three patients, in which histologic examination revealed a well-circumscribed dermal nodule composed of spindled cells with focal nuclear pseudo-inclusions. There was extensive fibrosis with hypocellular, storiform areas, characteristic of sclerotic fibroma. The spindled cells strongly expressed CD34, but not factor xiiia or markers of melanocytic, neural, or muscular differentiation. A diagnosis of Cowden syndrome was considered in one of the cases. These cases provide evidence that CD34 expression can occur in sclerotic fibromas, either solitary or associated with Cowden syndrome. When diagnosing a sclerotic fibroma, one should comment in the report regarding the possibility of Cowden syndrome.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
2/22. Solitary sclerotic fibroma of the skin: degenerated sclerotic change of inflammatory conditions, especially folliculitis.Two cases showing changes of sclerotic fibroma developed in association with an inflammatory process, especially folliculitis. The lesion in the first case showed a well-circumscribed, nonencapsulated nodule in the dermis, which consisted of a perifollicular fibrotic area and a peripheral sclerotic area. In addition to the usual findings of sclerotic fibroma, spindle cells were heavily infiltrated in a storiform and fascicular pattern around the degenerated hair follicle, suggestive of dermatofibroma. The lesion in the second case showed the typical findings of sclerotic fibroma in association with folliculitis and hair follicle remnants. Our observations suggest that solitary sclerotic fibroma of the skin may be a degenerated or sclerotic end stage of other fibrous conditions, such as dermatofibroma, and that it may be induced by inflammation, especially folliculitis.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
3/22. Sclerosing perineuriomas affecting bilateral hands.Sclerosing perineurioma is a rare, recently described variant of perineurioma that tends to affect the fingers and palms of adolescents or young adults. The process typically presents as a painless, well-circumscribed, unencapsulated nodule involving subcutis or reticular dermis. To our knowledge, all of the previously reported sclerosing perineuriomas were solitary. Hence, we report a 16-year-old boy, without stigmata of neurofibromatosis, who developed one sclerosing perineurioma in the right palm and another on the left index finger. Microscopically, tumours at both sites revealed small epithelioid and plump spindle cells with trabecular, whorled, or Indian file growth pattern enmeshed within extensively hyalinizing stroma. Immunohistochemically, the tumour cells showed strong reactivity for EMA but negative staining for S-100 and neurofilament. Ultrastructually, the tumour cells in both lesions exhibited features of perineurial cells, including discontinuous external basal lamina, occasional pinocytotic vesicles, and encirclement of abundant collagen fibrils by the interdigitating cytoplasmic processes. This case confirms the fact that sclerosing perineurioma is not always solitary.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
4/22. Giant cell collagenoma: case report and review of the literature.BACKGROUND: Giant cell collagenoma (GCC) is a recently described cutaneous fibrous neoplasm that usually affects young to middle-aged adults. Despite its similar histological appearance with circumscribed storiform collagenoma, no association of GCC with Cowden's syndrome has been described so far. To the best of our knowledge only five cases of this rare fibrous tumor have been reported so far. methods: We report a case of a 79-year-old male patient presenting with a slow growing flesh-colored dome-shaped lesion in his left forearm, with a clinical diagnosis of fibroma. No stigma of Cowden's syndrome was depicted. RESULTS: The histological analysis showed a symmetrical and well-circumscribed flat-dome-shaped lesion covered by an atrophic overlying epidermis. The neoplasm was composed of hyalinized collagen bundles disposed in a whorled storiform pattern. Admixed with the collagen matrix, there were two distinct cell populations, one composed by spindle-shaped mononuclear cells, and the other composed by bizarre multinucleated giant cells. Immunohistochemical analysis showed positivity for vimentin and actin HHF35 in the mononucleated. The multinucleated cells only immunoexpressed vimentin. CONCLUSION: GCC is an unusual cutaneous fibrous tumor that should be differentiated from circumscribed storiform collagenoma, pleomorphic fibroma, regressive forms of dermatofibroma, and solitary myofibroma based on its histological features.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
5/22. nephrogenic fibrosing dermopathy: an unusual skin condition associated with kidney disease.We report the case of a patient who, while on long-term hemodialysis (HD), developed nephrogenic fibrosing dermopathy, a newly described sclerosing skin disorder. This disorder is characterized by thickened, hardened skin with brawny hyperpigmentation and raised plaques. The most common patient complaints are pruritus and dysesthesia. The extremities are predominantly involved with sparing of the torso and face. Dysfunction of internal organs has not been described, distinguishing it from other fibrosing conditions such as scleroderma. The skin biopsy is characterized by haphazardly arranged dermal collagen spindle cells in the reticular dermis. Extensive mucin deposits are interposed between collagen bundles and there are an increased number of fibroblast-type cells. In contrast to scleroderma, inflammatory cells are generally absent. Corticosteroid therapy can be tried, but in our patient was of no benefit.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
6/22. nephrogenic fibrosing dermopathy with systemic involvement.BACKGROUND: There is a growing literature regarding sclerotic and panniculitic cutaneous conditions seen in patients with end-stage renal disease (eg, calciphylaxis and soft tissue calcification). nephrogenic fibrosing dermopathy (NFD) is a recent designation to describe cutaneous findings in patients with end-stage renal disease who developed sclerotic plaques with scleromyxedema-like histologic features. Soft tissue calcification is rare in patients with NFD and systemic involvement has not been reported. OBSERVATIONS: We describe a patient with end-stage renal disease who developed diffuse indurated woody plaques consistent with NFD in association with soft tissue calcification with catastrophic sequelae. A deep excisional biopsy specimen from the patient revealed thickened collagen bundles in the reticular dermis, plump bipolar spindle cells, and increased mucin. Focally, there were zones of calcium deposition in dermal collagen without vessel calcification. autopsy of the patient revealed extensive fibrosis and calcification of the diaphragm, psoas muscle, renal tubules, and rete testes. The patient died 11 months after developing NFD. CONCLUSION: A subset of patients with NFD may have significant systemic involvement.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
7/22. Sclerotic fibroma-like dermatofibroma: an uncommon distinctive variant of dermatofibroma.Dermatofibroma (DF) is a common benign cutaneous tumor with many variants based on alterations in the morphology and composition of its diverse elements. One very infrequent type is sclerotic fibroma-like DF (SF-DF). We report 7 new cases of SF-DF. In addition, their main clinicopathological and immunohistochemical features were compared with 14 unselected common DFs and with 3 sclerotic fibromas (SFs). Microscopically, the 7 cases of SF-DFs showed an unencapsulated, well-circumscribed, hypocellular central nodule with thick collagen bundles arranged in a storiform pattern with prominent clefts. The overlying epidermis was attenuated. The periphery of this nodule was more cellular with histopathologic features of common DF. The 7 SF-DFs patients were 4 women and 3 men with a mean ( /-SD) age of 44.8 ( /-15.5) years. These 7 patients were younger than those suffering from SFs [71.0 ( /-17.3) years; (p=0.04)] and older than those presenting common DFs [30.5 ( /-12.3) years; (p=0.03)]. Immunohistochemically, spindle cells in all 7 SF-DFs were negative for CD34 and CD99. On the contrary, the 3 cases of SF were positive for CD34 and CD99. All of the common DFs were negative for CD34 and only 4 (28.6%) of them were positive for CD99. In conclusion, SF-DF is an uncommon variant of DF with similar clinicopathological and immunohistochemical features. SF-DF shares certain histopathologic features with SF but they are immunophenotypically different. Therefore, both entities should be differentiated.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
8/22. Sclerosing dermatofibrosarcoma protuberans (DFSP): an unusual variant with focus on the histopathologic differential diagnosis.A 59-year-old man presented with a 10-cm x 8-cm tumoral plaque with a superficial nodule in the interscapular region of the back (Fig. 1). The lesion had been growing for 25 years. As a cystic lesion was suspected, the superficial nodule was biopsied. The histopathologic diagnosis was low-grade sarcoma with sclerosis. Two months after the initial biopsy, the lesion was completely excised, reaching the muscular fascia, with a 2-cm margin and with a free graft. Formalin-fixed paraffin-embedded samples were submitted to histologic and immunohistochemical study (4-microm paraffin sections); frozen tissue was submitted to electron microscopy. For histopathology, sections were stained with hematoxylin and eosin. immunohistochemistry was performed following standard avidin-biotin immunoperoxidase procedures with primary antibodies for vimentin, CD34, smooth muscle-specific actin, bcl-2, S-100, desmin, myoglobin, factor viii, p53 (all from DAKO, Copenhagen, denmark), HHF-35 (Enzo Diagnostics, Farmingdale NY), cytokeratin (AE1/AE3) (Biogenex, San Ramon, CA), and factor xiiia (Calbiochem Novabiochem Corporation, La Jolla, CA). At low magnification, the histologic study of the initial tumoral nodule revealed a poorly circumscribed mesenchymal proliferation, with fibroblastic-like neoplastic cells arranged in a fascicular and storiform pattern, admixed with extensive areas of sclerosis. At higher magnification, tumoral cells were spindle-shaped with hyperchromatic nuclei and scant cytoplasm. In some areas, sclerosis was so evident that a keloid-like pattern was seen (Fig. 2a). The surgical specimen showed a fibroblastic neoplastic proliferation infiltrating the dermis and hypodermis. In the dermis, cells were arranged in a storiform pattern, whereas in the hypodermis there was a honeycomb or lace-like pattern (Fig. 2b). There were also cellular areas alternating with sclerotic areas, with transitional zones in between, in both the dermis and hypodermis. The immunohistochemical study of the initial tumoral nodule and the surgical specimen showed that tumoral cells expressed vimentin, CD34 (Fig. 3), bcl-2, HHF-35, and smooth muscle actin. Neoplastic cells failed to show positivity with desmin, myoglobin, factor xiiia, factor viii, S-100, cytokeratin (AE1/AE3), and p53. An ultrastructural study revealed spindle cells having an irregular contour with a well-developed granular reticulum endoplasmic (REG) system in their cytoplasm, as well as some Golgi complexes and mitochondria. Also visible was the presence of many actin filaments and some myosin condensations (Fig. 4), characteristics of a fibroblastic cell with myofibroblastic differentiation. The final histopathologic diagnosis of the surgical specimen was sclerosing dermatofibrosarcoma protuberans. Two years after surgery, the patient is alive and well.- - - - - - - - - - ranking = 6keywords = dermis (Clic here for more details about this article) |
9/22. Diabetic (lymphocytic) mastopathy with exuberant lymphohistiocytic and granulomatous response: a case report with review of the literature.We report a case of a 66-year-old woman who presented with multiple painless masses in both breasts. Prior bilateral biopsies were diagnosed as Rosai-Dorfman disease (Sinus histiocytosis with Massive Lymphadenopathy). A recent lumpectomy specimen revealed a gray-white smooth cut surface with a discrete masslike lesion. The histopathology demonstrated a fibrotic breast parenchyma with foci of dense fibrosis and scattered inconspicuous breast epithelium surrounded by lymphocytes that formed aggregates and follicles with germinal centers. The inflammation was in a periductal, perilobular, and perivascular distribution. In addition, an exuberant inflammatory response with histiocytes and fibroblasts was present. This inflammatory response focally surrounded areas of fat necrosis and formed noncaseating granulomas with rare multinucleated giant cells. This process had infiltrative, ill-defined edges and involved the subcutaneous tissues. The overlying epidermis was normal. The final diagnosis was diabetic mastopathy with an exuberant lymphohistiocytic response. The differential diagnosis included Rosai-Dorfman disease, inflammatory myofibroblastic tumor, granulomatous mastitis, sclerosing lipogranulomatous response/sclerosing lipogranuloma, lupus panniculitis, and rheumatoid nodules. Immunohistochemical studies and flow cytometry confirmed the polyclonal nature of the lymphoid infiltrate. After the histologic evaluation, we inquired if the patient had a history of diabetes mellitus, and learned that she did have type 2 noninsulin-dependent diabetes mellitus. In conclusion, we report a case of diabetic mastopathy that presents with bilateral tumorlike masses and an unusual exuberant lymphohistiocytic response with granuloma formation. The pathologist may not be provided with a history of diabetes mellitus, but the characteristic fibrosis, lymphocytic ductitis/lobulitis, and sclerosing lobulitis with perilobular and perivascular lymphocytic infiltrates should provide clues for an accurate diagnosis, even when an exuberant and an unusual lymphohistiocytic response is present. A timely accurate diagnosis can help limit repeat surgeries in this vulnerable group of patients.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
10/22. An ultrastructural study of a sclerosing epithelial hamartoma.The histological and ultrastructural features of a sclerosing epithelial hamartoma are described. By electron microscopy, epithelial cords formed by basal-like cells and epithelial cysts with a pattern of keratinization similar to that of normal epidermis or of follicular infundibula were seen. By conventional light microscopy, continuities were found between the epithelial cords and the overlying epidermis. The stroma was found to be fibrous and contained some fibroblasts or possibly myofibroblasts with bundles of microfilaments. The data are interpreted with respect to the origin of the neoplasm, its line of differentiation, and its sclerosing behavior.- - - - - - - - - - ranking = 2keywords = dermis (Clic here for more details about this article) |
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