Cases reported "Skin Diseases"

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1/871. 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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2/871. Clear cell acanthoma presenting as polypoid papule combined with melanocytic nevus.

    We report on a case of clear cell acanthoma that developed above a preexisting melanocytic nevus. Although melanocytic nevi can be complicated by the presence of several different types of epithelial or appendageal tumors, there have been no reported case of clear cell acanthoma combined with a nevus. Interestingly, in this case the clear cell acanthoma had a polypoid configuration, an unusual finding.
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3/871. Cell-mediated immunity in epidermodysplasia verruciformis.

    Investigations were performed in 6 cases of epidermodysplasia verruciformis and 2 healthy family members. Nonspecific cell-mediated immunity (CMI) was studied by measuring response to phytohemagglutinin (PHA) and concanavalin a (Con A), percentrages of E- and EAC-rosette-forming lymphocytes, bacterial skin tests, and allergic reactions to dinitrochloro-benzene (DNCB). Impairment of CMI was manifested by reduction in the percentage of E rosettes, and lowered response to PHA, and- to a lesser degree- to Con A. The immune response to DNCB sensitization was invariably negative. Impairment of CMI was greater in cases of long duration and with extensive lesions. The cases of similar duration and extent of lesions, which never showed tendency to tumor formation, were not different in CMI in comparison with cases with numerous tumors. Only in cases with very advanced tumors CMI was impaired parallel to the gravity of the patient's general condition.
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4/871. abscess formation as a complication of parenteral methylphenidate abuse.

    case reports of five patients suffering from either skin abscesses or cellulitis following parenteral methylphenidate abuse are presented. Four patients had similar lesions consisting of abscesses or cellulitis which demonstrated typical signs and symptoms of an infective process and were treated with local symptomatic therapy, incision and drainage if indicated, and systemic antibiotics. The fifth patient suffered from a circular, necrotic, nonpurulent ulcer on the dorsum of the right foot which produced no local or systemic toxic effects. Attempts to culture a responsible organism yielded streptococcus viridans, an organism which is normal flora of the skin and, although opportunistic, is generally considered nonpathogenic. These cases further substantiate the belief that local vasospasm, chemical irritation, or both, produced by the methylphenidate solution may primarily cause a necrotic ulcer susceptibble to secondary bacterial infection.
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5/871. Systemic sarcoidosis presenting with multiple tattoo granulomas and an extra-tattoo cutaneous granuloma.

    We describe a 29 year old Caucasian man who developed cutaneous sarcoidosis manifesting itself as a tumour at the left outer canthus clinically mimicking a basal cell carcinoma and nine tattoo granulomas. Subsequent investigation revealed that the patient was also suffering from systemic sarcoidosis.
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6/871. An unusual cutaneous manifestation of myelodysplastic syndrome: "pseudo-Koebner phenomenon".

    An unusual and hitherto unreported complication of myelodysplastic syndrome is reported: the "pseudo-Koebner phenomenon." The skin lesions were characterised by exuberant "fleshy" masses at the sites of intravenous cannulation and skin trauma, and by histological evidence of chronic inflammation with focal necrosis and abscess formation. No evidence of dermal infiltration by malignant haemopoietic cells was seen. The exact aetiopathology of the phenomenon is unclear but an inappropriate and exaggerated inflammatory response owing to aberrant mediator mechanisms that are known to occur in some cases of myelodysplastic syndrome may be implicated.
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7/871. Cutaneous necrosis as a terminal paraneoplastic thromboembolic event in a patient with non-Hodgkin's lymphoma.

    Thrombotic complications in non-Hodgkin's lymphoma often originate in the large veins. We describe a patient with refractory advanced high-grade lymphoma who presented with the rare complication of extensive cutaneous necrosis due to thrombosis of dermal vessels; there was also a recent new peak of monoclonal IgM-kappa protein. Direct immunofluorescence demonstrated immune deposits with complement in the dermal vessel wall. Based on these observations and on published data, we suggest that these complexes were the trigger for the thrombotic events and that the monoclonal IgM acted as xenoreactive antibodies, initiating a cascade of events. The first step of this cascade was activation of the complement and the membrane attack complex, which caused secretion of IL-1 alpha by endothelial cells, followed by overexpression of tissue factor on the surface of the dermal vessel wall endothelium. Dermal vessel thrombosis was the final event in this cascade.
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8/871. immunoblastic lymphadenopathy-like T-cell lymphoma complicated by multiple gastrointestinal involvement.

    We report a rare case of immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma complicated by multiple gastrointestinal involvement, which appeared to be ameliorated by chemotherapy but resulted in perforative peritonitis. A 66-year-old Japanese woman who had generalized lymphadenopathy and eruptions was admitted to our hospital because of bloody stool. Colonoscopic examination revealed hemorrhagic ulcers in the terminal ileum and a saucer-like ulcer in the cecum. Gastrointestinal endoscopy revealed several ulcerative or elevated lesions in stomach and duodenum. biopsy specimens of these lesions and of a lymph node showed characteristic histological features of IBL-like T-cell lymphoma. The initial treatment with prednisolone (PSL) and cyclophosphamide (CPA) was effective. Six months after the treatment, however, she developed bloody stool again caused by multiple ulcerative lesions in the large intestine. The recurrence of the disease was determined histologically, and four courses of CPA, PSL, vinblastine sulfate and doxorubicin hydrochloride (CHOP) therapy were administered. One month after completing the CHOP therapy, she developed intestinal obstruction and then acute peritonitis resulting from perforation at an ulcer scar in the jejunum. Surgical treatment was successful, and histological examination demonstrated no lymphoma cells in the resected specimen. A gastrointestinal perforation should be recognized as a potential complication of IBL-like T-cell lymphoma, even during remission.
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9/871. 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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10/871. Squamous cell carcinoma arising in a cutaneous epidermal cyst: case report and literature review.

    Although cutaneous epidermal (infundibular) cysts are extremely common lesions, neoplastic transformation of their epithelium is quite rare. We describe a patient with a squamous cell carcinoma arising in the wall of an otherwise conventional epidermal cyst. In addition, we review the literature concluding that most of the previously reported cases may not correspond to this rare occurrence.
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