Cases reported "Leg Dermatoses"

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1/17. Evidence for thyrotropin receptor immunoreactivity in pretibial connective tissue from patients with thyroid-associated dermopathy.

    Pretibial myxedema (PTM), mainly characterized by the accumulation of glycosaminoglycans in the dermis and subcutaneous tissue, is an extrathyroidal manifestation of autoimmune Graves' disease (GD), almost always associated with Graves' ophthalmopathy (GO). The thyrotropin receptor (TSH-R) has been proposed as the common target antigen in GD, GO and PTM, with evidence for receptor transcripts and/or protein in these locations. The aim of this study has been to investigate whether receptor protein is present in the pretibial tissues. skin biopsies were obtained from two patients with PTM and two normal subjects without thyroid disease. A portion of each sample was fixed to produce semi-thin sections for Toluidine Blue or periodic acid Schiff (PAS) staining. The remainder was snap frozen to generate cryostat sections for immunohistochemical analysis using three monoclonal antibodies against TSH-R. In the skin from the two patients suffering from PTM, the dermis was infiltrated by inflammatory cells (lymphocytes, B cells, macrophages, mast cells) and adipocytes. The collagen fibers were dissociated by edema and by the accumulation of a PAS-positive material. Immunodetection of TSH-R produced positive staining on cells localized in the dermis, beneath the epidermis or close to the hypodermis. These cells were elongated and resembled fibroblasts. No immunoreactivity was observed in the dermis from control patients without thyroid disease. In conclusion, we have evidence for TSH-R immunoreactivity in the pretibium of patients with GD, GO and PTM. Further studies are needed to unambiguously identify the positive cells and determine whether the reactivity is due to the receptor itself or to a cross-reacting protein.
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2/17. Protean manifestations of human papillomavirus type 60 infection on the extremities.

    BACKGROUND: Human papillomavirus type 60 (HPV-60) induces a ridged wart or an epidermal cyst on the sole of the foot, exhibiting identical pathological changes, with a single refractile eosinophilic intracytoplasmic inclusion body in infected cells. However, there is no information on the role of HPV-60 in the development of cutaneous lesions on other anatomical sites. OBJECTIVES: To perform the clinicopathological analysis of various cutaneous lesions of a patient in relation to HPV genotype. PATIENT: A 50-year-old male patient developed multiple papules, plaques and nodules on his hand, arm and legs. RESULTS: Clinicopathologically, the lesions were classified into three categories. A common wart on the finger showed papillomatosis and acanthosis characterized by numerous keratohyalin granules. Plane warts on the arm showed perinuclear vacuolization of the cells in the upper Malpighian layer. On the other hand, a pigmented papillomatous nodule on the finger, and the other lesions on the hands and legs exhibited similar histological features with a unique cytoplasmic eosinophilic inclusion body. All the three categorized lesions were equally positive for HPV capsid antigen by immunohistochemistry. By blot hybridization analysis for HPV sequences, it was revealed that a common wart on the finger and plane warts on the arm harboured HPV-27 and HPV-3, respectively, while all the other lesions harboured HPV-60. The histological localization of each viral dna was confirmed in the corresponding lesions by in situ hybridization. CONCLUSIONS: HPV-60 is able to induce papular and nodular lesions on the extremities.
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3/17. Widespread morphoea following radiotherapy for carcinoma of the breast.

    We report a case of a 60-year-old lady who was treated with radiotherapy for breast cancer of both breasts 8 years apart. Thirteen years after the first dose of radiotherapy she developed localized morphoea in all the irradiated skin of the chest wall and also the gaiter regions of both lower legs. radiation-induced localized morphoea has been previously reported; however, there is no previous publication of an occurrence at a distant site as in this case. This case demonstrates that morphoea can occur distant to the original breast carcinoma and site of radiotherapy. We postulate that radiotherapy can induce neoantigen formation, which initiates a T cell response and subsequent tissue growth factor alpha release. Tissue growth factor alpha induces fibroblast activation and collagen production may persist due to a positive feedback mechanism within the fibroblast. The reason why the disease did not generalize remains unclear.
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4/17. Localized pretibial pemphigoid and pemphigoid nodularis.

    We describe a 75-year-old woman with a chronic, blistering eruption on the left leg whose clinical and immunopathologic features were consistent with a diagnosis of localized pretibial pemphigoid. After a disease-free interval of 5 years she developed a generalized prurigo nodularis-like eruption. Immunofluorescence studies revealed deposition of IgG and C3 along the dermoepidermal junction, and circulating autoantibodies against the dermoepidermal junction were demonstrated. Indirect immunoelectron microscopic examination of saponin-treated skin samples showed deposits of immunoreactants over the intracellular part of the hemidesmosomes. By Western immunoblotting the 230 kd bullous pemphigoid antigen was recognized by circulating autoantibodies. Thus our patient had two unusual clinical variants of bullous pemphigoid: localized pretibial pemphigoid and pemphigoid nodularis.
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5/17. Superficial thrombophlebitic tuberculide.

    BACKGROUND: Tuberculides are the result of immunologic reactions to hematogenously spread antigenic components of mycobacterium tuberculosis. There are three recognized tuberculides--papulonecrotic tuberculide, erythema induratum of Bazin, and lichen scrofulosorum. In 1997, in japan, Hara and coworkers reported five patients with what they called "nodular granulomatous phlebitis," which they proposed was a fourth type of tuberculide. We describe a patient who presented with features identical to those reported by Hara et al. in order to draw attention to the previous report and to support the concept of a fourth tuberculide which clinically resembles superficial thrombophlebitis. methods: A black South African man presented with cord-like thickening of superficial veins on the antero-medial aspects of the lower legs. Nodular swellings were palpable along the course of these veins. There was no evidence of tuberculosis elsewhere in the body, but the patient had a strongly positive tuberculin reaction. skin biopsies were performed for histologic examination, culture, and polymerase chain reaction (PCR). RESULTS: Histologic examination showed a granulomatous infiltrate localized to the veins in the subcutaneous fat. Stains for acid-fast bacilli and culture were negative, but PCR was positive for M. tuberculosis dna. The lesions responded promptly to antituberculous therapy. CONCLUSIONS: Our patient showed features identical to those of cases described by Hara and coworkers and assigned as a fourth type of tuberculide. As the lesions clinically resemble superficial thrombophlebitis, we propose the term "superficial thrombophlebitic tuberculide" rather than "nodular granulomatous phlebitis."
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6/17. Hyperkeratosis lenticularis perstans (Flegel's disease). In situ characterization of T cell subsets and Langerhans' cells.

    We report a patient with hyperkeratosis lenticularis perstans (HLP) manifesting as multiple reddish-brown hyperkeratotic papules on the lower extremities. Typical histologic features of HLP include hyperkeratosis, thinning or absence of the granular layer and a band-like infiltrate in the upper dermis underlying an atrophic epidermis. In order to determine the cellular composition of the infiltrate, skin biopsy specimens were studied immunohistochemically using a series of commercially available monoclonal antibodies. The dermal infiltrate consists predominantly of helper/inducer T cells (Leu-4 , Leu-3a ). Suppressor/cytotoxic T cells (Leu-2a ) were fewer at the periphery of the infiltrate. The majority of T cells were activated as they expressed HLA-DR-antigen. Large numbers of Leu-6 Langerhans' cells were observed at the dermo-epidermal interface. Few natural killer cells (Leu-11b ) were noted within the dermal infiltrate. These findings support the hypothesis than an active cellular immune reaction involving the epidermis is of pathogenic importance for HLP.
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7/17. Chronic pruritic eruption in patients with acquired immunodeficiency syndrome associated with increased antibody titers to mosquito salivary gland antigens.

    Five of seven patients with acquired immunodeficiency syndrome (AIDS) who had pruritus and a chronic, nonspecific-appearing skin eruption had increased antibody titers to antigens in the salivary glands of aedes taeniorhynchus, a salt marsh mosquito common to South florida. We hypothesize that the pruritus and skin lesions in patients with AIDS represent a form of chronic "recall" reaction. Increased antibody titers to mosquito salivary gland antigens may be a consequence of nonspecific B cell activation, a feature of AIDS.
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8/17. lichen planus presenting with erythema-multiforme-like bullous lesions in a patient with systemic scleroderma.

    A patient with systemic scleroderma is described who developed a cutaneous eruption of papules and vesicobullae. Over time, the latter turned into papules. The histopathological and immunofluorescence features of the papular lesions were unequivocally those of lichen planus, while those of the bullous lesions reminded of erythema multiforme. Histologically, erythema multiforme shares common features with lichenoid reactions, such as necrotic keratinocytes. Our case suggests that erythema multiforme and lichen planus may coexist or succeed each other as different stereotype immune reactions against the same antigen(s) within the epidermis.
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9/17. IgA anticardiolipin antibodies associated with Henoch-Schonlein purpura.

    Henoch-Schonlein purpura is associated with the deposition of immune complexes containing IgA. The nature of the antigen in these immune complexes is uncertain but in some reported cases has included autoantigens such as IgA rheumatoid factor and IgA antineutrophil cytoplasmic antibody. We report the finding of an IgA class anticardiolipin antibody in a 51-year-old patient with Henoch-Schonlein purpura. A potential role for IgA autoantibodies in Henoch-Schonlein purpura needs to be further explored.
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10/17. Localized bullous pemphigoid: report of a case with an immunofluorescence and electron microscopical studies on the lesional distribution of 180-KD bullous pemphigoid antigen, beta 4 integrin, and type VII collagen.

    A 67-year-old woman with a left-sided hemiplegia had localized bullous pemphigoid demonstrating typical clinical lesions on the left pretibial skin and the radial-side skin of the right forearm. The histology showed a subepidermal blister with extensive hyperkeratosis, hypergranulosis, and acanthosis. Direct immunofluorescence revealed distinct linear deposits of IgG and C3 at the dermo-epidermal junction in the perilesional skin and in the roof of the blisters, but few deposits in nonlesional skin. Electron microscopy revealed separation in the lamina lucida. Indirect immunofluorescence of type VII collagen showed its localization in the blister floor. The distribution of the 180-KD bullous pemphigoid antigen (BPA) and beta 4 integrin, hemidesmosomal transmembrane proteins, were studied in the lesional skin by indirect immunofluorescence. Both 180-KD BPA and beta 4 integrin were localized in the blister roof. By immunoelectron microscopy, beta 4 integrin was detected in small groups on the cell surface facing the blister cavity. Since the epitope of the monoclonal antibody to 180-KD BPA used here is known to be localized at a distance of 20 to 50 nm from the membrane surface and this epitope retained in the blister roof, it appears that the blister was produced in the deep lamina lucida. The lesions were cleared with topical 0.05% clobetasole propionate ointment.
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