Cases reported "Skin Diseases"

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1/140. Hypergranulotic dyscornification: a distinctive histologic pattern of maturation of epidermal epithelium present in solitary keratoses.

    Hypergranulotic dyscornification is an appellation proposed to designate a newly recognized distinctive pattern of epidermal maturation that is analogous to other epithelial reaction patterns such as epidermolytic hyperkeratosis, focal acantholytic dyskeratosis, cornoid lamellation, pale-cell acanthosis, and follicular mucinosis. The name "benign hypergranulotic keratosis with dyscornification" is proposed to specify solitary keratoses with digitated epidermal hyperplasia that exhibit this exceptional pattern of cornification. This abnormal type of cornification is characterized by hypergranulosis. A pale-staining basophilic substance is present intercellularly within the upper spinous layer and the hyperplastic granular layer. Overlying the thickened granular layer in foci at tips of epidermal papillations are orthokeratotic mounds of large, dull, eosinophilic staining corneocytes that are sharply demarcated from the thickened granular layer. Basophilic keratohyalin granules are focally retained within these corneocytes. There is overlying compact orthokeratosis that extends across the entire lesion. The compact orthokeratosis is slightly basophilic, and lies below a laminated and basket-weave orthokeratotic stratum corneum. There is a predominantly lymphocytic infiltrate at the base of these neoplasms with some spongiosis. There is parakeratosis focally present in the stratum corneum overlying these individual areas of abnormal cornification. The histopathologic and clinical findings in eight lesions that exhibit hypergranulotic dyscornification, a heretofore undescribed unique pattern of epidermal cornification, are presented.
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ranking = 1
keywords = neoplasm
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2/140. A case report including EM and dna repair investigations in a dermatosis associated with multiple skin cancers: epidermodysplasia verruciformis.

    This report describes the clinical, histological and electron microscopic observations in a 51-year-old male with epidermodysplasia veruciformis (EV). cells with early signs of malignant transformation were found closely connected with virus infected epidermal regions. skin cancers appeared initially on sun-exposed areas, such as the face and ear lobes. The UV-induced dna repair synthesis was therefore studied, utilizing peripheral leukocytes. The patient had 40% lower UV-induced dna repair synthesis than the mean of nine healthy subjects of the same age. These results suggest that a decrease in UV-induced dna repair synthesis in combination with a possibly oncogenic viral infection may enhance the disposition for somatic mutations and malignant transformation in patients with EV.
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ranking = 7.1559124360824
keywords = cancer
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3/140. Cutaneous waldenstrom macroglobulinemia in transformation.

    waldenstrom macroglobulinemia is a low-grade B-cell lymphoproliferative disorder of the elderly with characteristic monoclonal IgM-producing neoplastic infiltrates of the bone marrow, lymph node, and spleen. Cutaneous manifestations are usually nonspecific such as purpura, ulcers, and urticarial lesions. These lesions are caused by hyperviscosity of the blood, immune complex-mediated vascular damage, paraprotein deposition, and amyloid deposition. Specific skin lesions occur rarely and generally consist of translucent, flesh-colored papules composed of monoclonal IgM deposits. Rarely, there may be violaceous lesions composed of low-grade lymphoplasmacytic infiltrates characteristic of waldenstrom macroglobulinemia. Both cutaneous manifestations of the disease, as well as disease transformation to high-grade, large cell lymphoma are rare. We report two very unusual cases of waldenstrom macroglobulinemia with documented skin disease that demonstrated transformation to high-grade lymphoma. Both patients were elderly men with long-standing waldenstrom macroglobulinemia involving the bone marrow, who subsequently developed skin involvement by the disease. waldenstrom macroglobulinemia can rarely manifest as cutaneous disease, sometimes as a high-grade transformation of low-grade waldenstrom macroglobulinemia elsewhere. Distinction of cases of transformed waldenstrom macroglobulinemia from de novo cutaneous large cell lymphoma may be important, because the two entities are likely biologically different.
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ranking = 0.42892654255766
keywords = spleen
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4/140. Microcystic adnexal carcinoma: collaborative series review and update.

    BACKGROUND: Microcystic adnexal carcinoma (MAC) is a malignant appendageal tumor first described in 1982. It can be clinically and histologically confused with other malignant and benign cutaneous neoplasms, leading to inadequate initial treatment. This neoplasm is locally aggressive and deeply infiltrating, characterized by high morbidity and frequent recurrence. Mohs micrographic surgery has been used to conserve tissue and improve the likelihood for cure. OBJECTIVE: We report our experience using Mohs micrographic surgery for the treatment of MAC and compare with earlier reports in the literature. In addition, we review the epidemiology, clinical and histologic characteristics, and optimal treatment of this rare neoplasm. We also describe a 15-year-old white male patient with MAC on the scalp occurring only 7 years after radiation exposure. methods: The medical records of 11 patients with MAC who were treated by Mohs micrographic surgery were reviewed at both departments, and follow-up data were obtained. RESULTS: In all patients treated with Mohs micrographic surgery, there were no recurrences after a mean follow-up of 5 years. CONCLUSION: Mohs technique enables the detection of clinically unrecognizable tumor spread and perineural invasion often encountered with MAC. Aggressive initial treatment by microscopically controlled excision appears to offer the greatest likelihood of cure for this neoplasm, while providing conservation of normal tissue. In addition, we describe the second youngest patient with MAC and readdress the issue of previous radiotherapy as an important predisposing factor.
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ranking = 4
keywords = neoplasm
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5/140. Subcutaneous calcification as a late effect of orthovoltage chest wall irradiation.

    Adjuvant radiotherapy to the breast or chest wall is given to some patients with breast cancer, to reduce the risk of local recurrence. It is known to be associated with various late sequelae, including subcutaneous fibrosis, telangiectasia and pulmonary fibrosis. Delivering radiotherapy to the chest wall and nodal drainage areas presents the technical problem of matching the glancing and anterior supraclavicular fields. Overlap between these fields will result in underlying tissues receiving a larger dose than intended; similarly, a gap results in an inadequate dose. We present the case history of a patient with subcutaneous calcification occurring as a late sequela of radiotherapy to the chest wall and anterior supraclavicular field. This has not been previously reported and is thought to have arisen from a high dose region in an area of overlap between fields.
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ranking = 1.4311824872165
keywords = cancer
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6/140. muir-torre syndrome: case report of a patient with concurrent jejunal and ureteral cancer and a review of the literature.

    BACKGROUND: muir-torre syndrome is a rare autosomal dominant genodermatosis, first described in 1967, characterized by the presence of sebaceous tumors and an internal malignancy in the absence of other predisposing factors. OBJECTIVE: Our purpose was to review and update published literature on muir-torre syndrome. methods: We describe a 66-year-old white man with a history of sebaceous tumors and newly diagnosed transitional cell cancer of the right ureter and adenocarcinoma of the jejunum. The literature on muir-torre syndrome is reviewed by means of medline search and available published reports and updated. RESULTS: Only 205 cases of muir-torre syndrome with 399 internal malignancies have been reported. The common presentation is the presence of sebaceous tumors along with a low-grade visceral malignancy. Sebaceous tumors appeared before the internal malignancy in 45 cases (22%), concurrently in 12 (6%), and after the internal malignancy in 114 (56%). In 33 (16%) of 205 patients, a temporal relationship was not reported. The total number of sebaceous gland carcinomas reported is 44; 17 of 44 were neoplasms of the meibomian gland. Keratoacanthomas have been noted in 48 (23%) of 205 patients. Gastrointestinal cancers are the most common internal malignancies (61%), followed by genitourinary (22%). CONCLUSION: The presence of sebaceous tumors warrants a search for an internal malignancy. In patients with muir-torre syndrome, regular follow-up and search for new malignancy is mandatory. Evaluation and monitoring of the family members of patients are also necessary. patients and their families should be counseled for genetic testing. Genetic analysis of the primary tumor and skin lesions should be arranged as an added research tool if possible to better understand the disease.
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ranking = 9.5870949232989
keywords = cancer, neoplasm
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7/140. Spiradenoma arising in a nevus sebaceus of Jadassohn: case report and literature review.

    nevus sebaceus (NS) of Jadassohn is usually a verrucous plaque on the scalp or face that arises secondary to disordered development of epithelial, pilar, sebaceous, and apocrine structures. The emergence of neoplasia is a late stage in the natural history of NS. Although most neoplastic proliferations are benign, several malignant tumors have arisen in this lesion. We describe the first case of a benign spiradenoma arising in an NS on the scalp in a 72-year-old Caucasian woman. Reexcision was recommended to prevent the development of a second neoplastic process and to avoid the rare occurrence of a malignant transformation of the existing neoplasia. The patient declined reexcision and remains under observation. The spectrum of tumors arising in NS are described and are categorized according to behavior. Syringocystadenoma papilliferum is the most commonly observed benign growth, whereas basal cell carcinoma is the most frequently seen malignant process. The signs of tumor development (benign or malignant) within an NS are reviewed, and treatment recommendations are provided. The clinical course of rare and unique aggressive neoplasms originating in NS is summarized.
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ranking = 1
keywords = neoplasm
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8/140. A solitary cutaneous tumor with distinct areas of verruca and seborrheic keratosis-like lesion.

    A single, exophytic, cutaneous tumor on the thigh of a 52-year-old man was examined by light microscopy, in situ hybridization and immunohistochemistry. It demonstrated distinct areas of verruca and of seborrheic keratosis-like morphology simultaneously. Focally, architectural abnormalities were noted in some deeper parts of the tumor, but there was no morphological evidence of malignancy. The patient has remained disease-free for two and a half years after surgery. Biotinylated full genomicDNA probes of HPV confirmed the presence of types 6/11 exclusively in the verrucous portion of the neoplasm. In the verrucous component p53 protein was overexpressed and, additionally, increased Ki-67 immunopositive signals were detected, being localized below the HPV-DNA-expressing spinous cells.
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ranking = 1
keywords = neoplasm
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9/140. Trichoblastoma and sebaceoma in nevus sebaceus.

    We report two cases of an unusual combined adnexal neoplasm arising in a nevus sebaceus (NS). Clinically, both neoplasms presented in two women (46 and 78 years) as single, partially ulcerated nodules within NS situated on the scalp. Histopathologically, each neoplasm showed distinctive aggregations of basaloid cells with features of trichoblastoma adjacent to aggregations of neoplastic cells exhibiting features of sebaceoma. In both cases, typical features of NS were present. To the best of our knowledge, this unusual combined adnexal neoplasm comprised of trichoblastoma and sebaceoma could not be assigned to any previously described histopathologic entity. This "complex" adnexal neoplasm should be distinguished histopathologically from basal cell carcinoma with sebaceous differentiation and trichoblastoma with sebaceous differentiation.
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ranking = 5
keywords = neoplasm
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10/140. Topical 5-fluorouracil as primary therapy for keratoacanthoma.

    keratoacanthoma is a common, benign cutaneous neoplasm that displays rapid growth on sun-exposed skin. Keratoacanthomas usually involute spontaneously after several months but rarely progress to squamous cell carcinoma. Because this is a benign, self-limited lesion of exposed skin, effective treatment should emphasize patient comfort and cosmetic results in addition to effectiveness. The authors present 2 patients with keratoacanthomas treated with topical 5-fluorouracil. Both patients had complete resolution of their lesions within 8 weeks. The cosmetic result was superb in both patients. patient satisfaction with this therapy was excellent. Treatment can be instituted based on a clinical diagnosis; no diagnostic biopsy is necessary. Most keratoacanthomas respond to topical 5-fluorouracil therapy within 3 weeks, whereas squamous cell carcinomas respond poorly. Any lesion that shows a poor response after 3 weeks of therapy or that does not resolve within 8 weeks should undergo prompt excisional biopsy for definitive diagnosis and treatment. Topical 5-fluorouracil is an effective, convenient, relatively inexpensive treatment for keratoacanthoma that produces excellent cosmetic results. It should be added to the therapeutic armamentarium of all physicians who treat keratoacanthoma.
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ranking = 1
keywords = neoplasm
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