Cases reported "Skin Diseases"

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1/69. 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 = hyperplasia
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2/69. oral manifestations of Schimmelpenning syndrome: case report and review of literature.

    Schimmelpenning syndrome (SS) is characterised by specific skin manifestations, skeletal defects, and central nervous system abnormalities. Here, the SS is briefly reviewed, and the oral and dental manifestations are described in a patient whose medical findings were previously published and included severe hypophosphatemic rickets. Significant oral and dental features included papillomatous lesions of the gingiva, hemihyperplasia (hemihypertrophy) of the tongue, bone cysts, aplasia of teeth, enlarged pulp chambers, hypoplastic or absent enamel, and an odontodysplasia-like permanent tooth.
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keywords = hyperplasia
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3/69. Troglitazone improves psoriasis and normalizes models of proliferative skin disease: ligands for peroxisome proliferator-activated receptor-gamma inhibit keratinocyte proliferation.

    BACKGROUND: psoriasis is often treated with agents that activate nuclear hormone receptors for glucocorticoids, retinoids, and vitamin d. The peroxisome proliferator-activated receptor-gamma (PPARgamma) is a related nuclear hormone receptor that can be activated by its ligands, including the thiazolidinediones. OBJECTIVE: To assess whether treatment with troglitazone, a currently available thiazolidinedione used to treat diabetes mellitus, has an effect on psoriasis in normoglycemic patients and whether ligands for PPARgamma have an effect on models of psoriasis. DESIGN: Open-label administration of troglitazone in patients with psoriasis and evaluation of drug actions in cellular, organ, and transplant models of psoriasis. SETTING: University and community hospital outpatient departments and university laboratories. patients: patients with chronic, stable plaque psoriasis and control subjects. Five patients with psoriasis received troglitazone (none withdrew); 10 different untreated patients and 10 controls provided tissue samples. INTERVENTIONS: Oral troglitazone therapy at various dosages in patients with psoriasis; also, use of troglitazone, ciglitazone, and 15-deoxy-delta-12,14-prostaglandinJ2 in psoriasis models. MAIN OUTCOME MEASURES: Investigator-determined clinical results in patients and cell counts and histological evidence in models. RESULTS: All patients' psoriasis improved substantially during troglitazone therapy. Peroxisome proliferator-activated receptor-gamma was expressed in human keratinocytes; ligands for PPARgamma inhibited the proliferation of normal and psoriatic human keratinocytes in culture. Troglitazone treatment normalized the histological features of psoriatic skin in organ culture and reduced the epidermal hyperplasia of psoriasis in the severe combined immunodeficient mouse and human skin transplant model of psoriasis (P<.05 compared with untreated controls). CONCLUSIONS: Peroxisome proliferator-activated receptor-gamma might be a useful intracellular target for the treatment of psoriasis; further study is needed to assess the clinical value of ligands for PPARgamma, including troglitazone.
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ranking = 1
keywords = hyperplasia
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4/69. Virginal breast hypertrophy.

    Macromastia is the massive enlargement of the breast, unilateral or bilateral, disproportional to growth in the remainder of the body. Most patients seen with macromastia have breasts that develop normally at puberty but simply reach excessive size. Virginal breast hypertrophy is a rare and distinct disorder with the rapid onset of macromastia at the onset of puberty. Although the disorder has been reported in the surgical, pediatric, and gynecologic literature, virginal breast hypertrophy has not been reported in dermatologic journals. We report two cases of virginal breast hypertrophy. Patient 1 is a 14-year-old girl who developed macromastia 6 months after a liver transplant for alpha 1-antitrypsin deficiency. She had intense erythema as well as verrucous hyperplasia overlying the breasts. Patient 2 was a 12-year-old girl who experienced severe bilateral breast enlargement 4 months after beginning treatment for thyrotoxicosis. She had extreme tenderness, erythema, and edema of the breasts and was treated with tamoxifen citrate, with improvement of her symptoms. Although virginal breast hypertrophy is a rare disorder, the dermatologist may be asked to consult on the associated skin changes in these cases and should be aware of its existence. The definitive therapy is surgical, but until breast growth is stabilized, cutaneous manifestations need to be managed.
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ranking = 1
keywords = hyperplasia
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5/69. Premature calvarial synostosis and epidermal hyperplasia (Beare-Stevenson syndrome-like anomalies) resulting from a P250R missense mutation in the gene encoding fibroblast growth factor receptor 3.

    We report on a patient with a severe premature calvarial synostosis and epidermal hyperplasia. The phenotype was consistent with that of a mild presentation of Beare-Stevenson syndrome but molecular analysis of the IgIII-transmembrane linker region and the transmembrane domain of the gene encoding the FGFR2 receptor, revealed wild-type sequence only. Subsequently, molecular analysis of the FGFR3 receptor gene identified a heterozygous P250R missense mutation in both the proposita and her mildly affected father. This communication extends the clinical spectrum of the FGFR3 P250R mutation to encompass epidermal hyperplasia and documents the phenomenon of activated FGFR receptors stimulating common downstream developmental pathways, resulting in overlapping clinical outcomes.
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ranking = 6
keywords = hyperplasia
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6/69. Recurrent intravascular papillary endothelial hyperplasia developing from a pyogenic granuloma.

    Intravascular papillary endothelial hyperplasia (IPEH) is a rare benign vascular tumour. Many histological features are similar to those of low-grade angiosarcoma, a common, but more serious condition. Clinical and histological differentiation is important to avoid overtreatment of this benign condition. We report on a 34-year-old woman who developed recurrent IPEH at the site of a previously excised pyogenic granuloma.
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ranking = 5
keywords = hyperplasia
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7/69. Persistent atypical lymphocytic hyperplasia following tick bite in a child: report of a case and review of the literature.

    We report a 6-year-old girl who developed a red papule on the posterior neck at the site of a previous tick bite. Initial biopsy was performed a year after the bite and the specimen showed a dense lymphoid infiltrate with admixed CD30 cells. The patient was referred to our center because of concern about the development of a CD30 lymphoproliferative disorder. The lesion was completely excised. histology showed no evidence of a clonal lymphoproliferative disorder or borrelia infection, but persistence of CD30 cells. This case demonstrates that a tick bite reaction can persist for more than 1 year and show immunophenotypic and morphologic overlap with a CD30 lymphoproliferative disorder. Complete history with thorough clinical and histopathologic evaluation is necessary to arrive at the correct diagnosis.
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ranking = 4
keywords = hyperplasia
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8/69. Subcutaneous angiolymphoid hyperplasia (Kimura disease). Report of a case.

    A subcutaneous mass removed from the cheek showed histologic features of subcutaneous angiolymphoid hyperplasia (Kimura disease) at its early stage. The condition shows a wide spectrum of pathologic changes. At its early stage, the main findings consist of active vascular proliferation with plump endothelial cells and varying degrees of lymphocytic, histiocytic, and eosinophilic infiltration. The lesion at its later stage features hyperplastic blood vessels with inconspicuous endothelial cells, well-formed lymphoid follicles, and varying degrees of lymphocytic and eosinophilic infilitration. Blood eosinophilia is frequently seen. review of the literature and study of our own case strongly suggest that this disease is a distinct clinical and pathologic entity.
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ranking = 5
keywords = hyperplasia
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9/69. Papulonodular lichenoid and pseudolymphomatous reaction at the injection site of hepatitis b virus vaccination.

    immunization with the hepatitis b virus (HBV) is effective and safe with an estimated incidence of adverse reactions, either local or systemic, of less than 0.1%. Cutaneous side effects are rare and include lichen planus (LP) and lichenoid reactions. We report the case of a 21-year-old female, in whom a persistent, papulonodular lesion developed at the site of the injection, 6 weeks after the second dose of the HBV. Histological examination revealed lichenoid and pseudolymphomatous features. In addition, sensitization to thiomersal, a vaccine constituent, was documented by patch testing. The association of LP with chronic liver disease is well established. Furthermore, less than 20 cases of lichen or lichenoid reactions, following HBV vaccination, have been reported. Although several arguments have been presented, it is still debated whether there is a causal association or the occurrence of LP following HBV vaccination is a simple coincidence. It has been speculated that a T-cell-mediated, graft-versus-host-like reaction, triggered by a sensitizing protein, is directed against keratinocytes expressing an epitope of hepatitis B surface antigen or a similar epitope. Our case may represent a localized lichenoid reaction to HBV vaccination, a local reactive hyperplasia or a persisting delayed hypersensitivity reaction to a vaccine constituent. This is the first case of a local lichenoid reaction at the injection site of the HBV vaccine, providing further documentation for a causal association linking the HBV vaccine with LP.
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ranking = 1
keywords = hyperplasia
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10/69. Histologic features of cutaneous sinus histiocytosis (Rosai-Dorfman disease): study of cases both with and without systemic involvement.

    Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a systemic proliferation of cells that resemble the sinus histiocytes of lymph nodes. Whereas initial reports highlighted the often striking cervical adenopathy, more than 40% of patients have extranodal involvement. Cutaneous lesions are the most common form of extranodal disease, but cases that present as cutaneous disease without lymph node involvement are rare. We examined biopsies from three patients with cutaneous lesions of sinus histiocytosis, none of whom had detectable systemic involvement, and compared them to those of two patients whose lymph nodes were involved by the disease. The histology of skin lesions in all five cases showed dense nodular or diffuse infiltrates of "histiocytes," emperipolesis of lymphocytes, neutrophils, and plasma cells. "histiocytes" were present in lumens of dilated lymphatics. At the peripheries of the lesions were lymphoid aggregates and thick-walled vessels surrounded by plasma cells. Staining with anti-S-100 protein antibody showed marked cytoplasmic positivity in the "histiocytes" in each case. The only features that differed among the two groups were the presence of pseudoepitheliomatous hyperplasia and infiltrates of eosinophils in one case in which the disease was limited to the skin. We believe that cutaneous sinus histiocytosis can be specifically diagnosed by skin biopsy. Because cutaneous sinus histiocytosis may be unfamiliar to general pathologists, dermatopathologists, and dermatologists, cases limited to the skin may be under-recognized.
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ranking = 1
keywords = hyperplasia
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