Cases reported "Mouth Diseases"

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1/15. oral manifestations of idiopathic lenticular mucocutaneous pigmentation (Laugier-Hunziker syndrome): a clinical, histopathological and ultrastructural review of 12 cases.

    OBJECTIVE: To present a clinical, histopathological and ultrastructural study on a group of patients affected by idiopathic mucocutaneous pigmentation (Laugier-Hunziker syndrome: LHS). MATERIALS AND methods: Twelve patients were investigated: clinical examination, laboratory tests, and X-ray studies together with light microscopy and electron microscopy were performed in order to diagnose LHS. RESULTS: All cases showed acquired, benign, macular hyperpigmentation of buccal mucosa lips and nails. Histologically, pigmentations are due to an accumulation of melanin in the basal layer keratinocytes and an increase in the number of melanophages in the submucosa and/or papillary dermis. Ultrastructurally there were increased numbers of normal-appearing melanosomes in keratinocytes of the lower epithelium. No evidence of malignant changes were detected. CONCLUSIONS: The importance of this condition relates to it being included in the differential diagnoses of pigmentary disorders of the oral mucosa with associated nail involvement. It is important to recognize this acquired benign disorder to avoid unnecessary investigations and treatments.
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keywords = dermis
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2/15. Acral keratotic graft versus host disease simulating warts.

    A 55-year-old man experienced persistent oral graft versus host disease after receiving an HLA-matched allogeneic stem cell peripheral blood transplant for chronic myeloid leukaemia. Twelve months post-transplant, multiple keratotic lesions resembling warts developed on his fingers, on his palms and on the soles of his feet. Skin biopsy showed a lichenoid reaction under a hyperplastic and hyperkeratotic epidermis lacking signs of papillomavirus infection. The lesions partially regressed when the patient's oral prednisone was increased. This instance of acral keratotic lesions may represent an unusual expression of chronic graft versus host disease; however, the the hydroxychloroquine which had been used for 8 months to control the patient's oral graft versus host disease cannot be excluded as a cofactor.
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keywords = dermis
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3/15. Oral acantholytic itching disease responding to dapsone. dermatitis herpetiformis, pemphigus, or a new disease?

    A patient had a blistering and severely itching disease confined solely to the oral cavity. The histopathologic findings had the features of both pemphigus and dermatitis herpetiformis. There were no gastrointestinal symptoms, no IgA could be found in uninvolved skin, and no intercellular or basement membrane antibodies were present in the serum or epidermis. The disease responded favorable to dapsone (diaminodiphenylsulfone), which could be discontinued after 2 1/2 years. The literature is briefly reviewed, and there is a discussion of whether the patient was suffering from pemphigus or dermatitis herpetiformis. The conclusion is drawn that the diagnosis cannot be established with certainty and that we are perhaps dealing with a new disease. It is stressed that in such aberrant cases a therapeutic trial with dapsone should be made.
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keywords = dermis
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4/15. Oral psoriasis in a patient with hepatitis c virus infection.

    We report a case of 65-year-old patient with psoriasis vulgaris who developed a psoriatic manifestation on his lower lip, along with typical features of psoriasis on his trunk and extremities. Mucous membranes, palate and tongue were not affected. A biopsy specimen from lip showed acanthosis of the epidermis with parakeratosis, and mild cellular infiltrates in the upper dermis. He was also suffering from type C hepatitis, however, he had not been treated with interferons. Oral psoriasis involving the lip is extremely rare.
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ranking = 2
keywords = dermis
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5/15. Hyalinosis cutis et mucosae: a 30 year follow up of a female patient.

    Hyalinosis cutis et mucosae is an inherited disease, characterized by deposition of a hyaline-like substance in the dermis and internal organs. In the present study, we report a 30 year follow up in a female patient with hyalinosis cutis et mucosae treated using dermabrasion and oral retinoids. In our opinion, the lesion on uncovered body areas should be corrected as soon as possible to enable a normal psychophysical development. The lesions on skin and oral mucosa may be successfully removed without remission with dermabrasion in early childhood. In addition, oral retinoids in adulthood may produce some improvement.
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keywords = dermis
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6/15. A case of symptomatic primary HIV infection.

    A 30-year-old homosexual Japanese man had fourteen days of fever, malaise, appetite loss, sore throat, and four days of diarrhea and slightly congested eyes before he developed a skin eruption. He presented with measles-like exanthems on his face, trunk, and extremities. Deep red enanthems were seen on his left buccal mucosa opposite the premolar teeth, and whitish enanthems were seen on the buccal and gingival mucosa. HIV rna was detected at the high concentration of 5.8 x 10(6) copies /ml in his serum. cerebrospinal fluid examination revealed aseptic meningitis with 5,488 copies /ml of HIV rna. Anti-HIV 1 antibodies against Gp160 and p24 tested by Western blot assay showed seroconversion on day 5 of his admission, seven days after he developed the skin eruptions. The fever lasted for three weeks from the initial onset, and the skin eruptions lasted for twelve days. Histopathologically, a mononuclear cell infiltration was seen mainly in the upper dermis surrounding small vessels and sweat ducts, with CD8 cytotoxic T lymphocytes predominant. Additionally, CD1a putative interdigitating dendritic cells had also infiltrated perivascularly, and were surrounded by CD8 and CD4 T cells. in situ hybridization study failed to detect HIV products in skin biopsy specimens. Our findings suggested that CD8 T cells and their interaction with CD1a dendritic cells in the skin may be important in inducing skin manifestations in acute hiv infections.
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keywords = dermis
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7/15. Recurrent mucocutaneous lymphocytic infiltration possibly associated with Epstein-Barr virus infection.

    A 32-year-old man had a history of recurrent but self-healing necrotizing nodules on the face and oral mucosa. Histologically the lesion was composed of necrotizing foci surrounded with dense CD8 T lymphocytic infiltration in the dermis. Laboratory examination showed marked blood CD8 T lymphocytosis with a reversed ratio of CD4 /CD8 of 0.11. Except for a very high titer of antibodies against Epstein-Barr-virus-associated antigens, no signs of infection were observed.
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ranking = 1
keywords = dermis
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8/15. Laugier-Hunziker syndrome: a clinical, histopathologic, and ultrastructural study of four cases and review of the literature.

    Four cases of Laugier-Hunziker syndrome are described. In all patients (two men and two women between 39 and 57 years of age) pigmentation of the lower lip and hard palate was found. in addition, two patients had involvement of the buccal mucosa; another patient also had pigmentation of the upper lip, the gums, the soft palate, and the fingers of both hands. Histopathologic examination demonstrated an accumulation of melanin in the basal layer keratinocytes and an increase in the number of melanophages in the papillary dermis. Ultrastructural study showed the presence of numerous mature melanosomes in the cytoplasm of the keratinocytes of the basal layer and of the melanophages in the papillary dermis. Alterations of the melanocytes were not observed.
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ranking = 2
keywords = dermis
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9/15. Peutz-Jeghers-like melanotic macules associated with esophageal adenocarcinoma.

    A 67-year-old white man with esophageal adenocarcinoma presented with Peutz-Jeghers-like macules on the lips, face, nipples, and rectal mucosa. Pathological examination of the macules showed atypical melanocytes in the epidermis. The pleomorphic large melanocytes resembled the neoplastic cells seen in melanoma in situ. This is the first report of proliferation of atypical melanocytes in Peutz-Jeghers-like melanosis associated with a primary esophageal adenocarcinoma.
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ranking = 1
keywords = dermis
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10/15. Focal mucinosis of the upper aerodigestive tract in children.

    Cutaneous mucinosis is an uncommon lesion characterized by mucin accumulation in the dermis. Rarely, mucinosis may arise in the oral cavity; reported cases have been observed in patients older than 16 years. We identified two children younger than 5 years of age with focal mucinosis of the upper aerodigestive tract. A 3-year-old boy had been intubated at age 2 months, suffered from recurrent respiratory infections, and developed mucinosis of the larynx, a previously unreported site for this unusual lesion. The second patient, a 4-year-old girl with a cleft palate, developed palatine mucinosis.
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ranking = 1
keywords = dermis
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