Cases reported "Skin Diseases"

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1/43. Unusual giant comedo naevus.

    Comedo naevi are usually well circumscribed, and although extensive cases have been reported individual lesions crossing the midline are rare. Associated neurological, skeletal and ophthalmological abnormalities are also recognized. thus, the patient now reported is unusual in that she had an extensive systematized comedo naevus with crossing of the midline but no associated abnormalities. Topical tretinoin was helpful in improving the texture and appearance of the comedones, and various larger lesions responded to curettage.
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ranking = 1
keywords = naevus
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2/43. Inflammatory myofibroblastic tumor of the skin.

    We report a case of inflammatory myofibroblastic tumor (IMF) of the skin in a female with a history of Wegeners granulomatosis. The patient had a painless, erythematous, and indurated lesion of the left elbow. The resected specimen revealed a 4 cm x 3 cm nodule involving the entire dermis and superficial portions of subcutis with a stellate profile at scanning magnification. There were spindle cells in fascicles and whorls and a mixed inflammatory cell infiltrate of plasma cells, lymphocytes, neutrophils, and eosinophils. The spindle cells were immunoreactive for vimentin, muscle specific actin, and smooth muscle actin. The polyclonal and polymorphous nature of the inflammatory cells was confirmed by immunohistochemical studies. This is the first case of IMF of the skin documented by immunostaining.
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ranking = 0.017965172172927
keywords = spindle cell, spindle
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3/43. Glial heterotopia in the subcutaneous tissue overlying T-12.

    Heterotopic glial nodules occur most commonly in the head and neck area, and are theorized to arise following abnormalities in the development of the facial and skull bone plates. However, in spite of the fact that some of these lesions are associated with communication with the central nervous system (CNS), the lack of a meningeal component, argues against simple herniation and separation of brain tissue through a defect in the skull. We present an infant with a nodule directly over the spine present in the T-12 region with no skin abnormalities. magnetic resonance imaging (MRI) and computerized axial tomography (CT) showed no spinal abnormalities with an overlying fibrotic soft tissue mass. The patient had no other associated clinical findings. Histologic findings showed a cellular component arising within the reticular dermis with a deep circumscribed margin. The nodule contained irregularly shaped cells containing abundant cytoplasm and indistinct cellular margins with bland nuclei. These cells were clustered around and between a fibro-mucinous stroma. Immunohistochemical stains showed positive staining for S-100 protein, vimentin, GFAP, NSE, and CD57, and negative staining for Ki-67, CD34, Neurofilament protein, cytokeratin, and EMA. The spindle cells showed positive staining for CD34 and vimentin. The clinical and histologic features and immunohistochemical profiles are used to separate this lesion from the closely related, ependymal rests, ependymomas, and primary cutaneous chorodomas.
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ranking = 0.0089825860864636
keywords = spindle cell, spindle
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4/43. A sporadic case of progressive mucinous histiocytosis.

    Hereditary progressive mucinous histiocytosis is a rare autosomal dominant non-Langerhans cell histiocytosis. We describe a sporadic case of this syndrome in a 64-year-old woman who had multiple dark-red dome-shaped papulonodules located mainly on the back of her hands, forearms and thighs. light microscopy revealed a circumscribed upper dermal aggregate of ovoid or spindle-shaped histiocytes with abundant mucin deposition. iron deposits and numerous mast cells were scattered throughout the tumour but giant cells were rare. Electron microscopy revealed a high number of zebra bodies and myeloid bodies in the cytoplasm of the histiocytes. immunohistochemistry showed positive labelling with alpha-1 antitrypsin, factor xiiia and CD68, while CD1a, CD34 and S100 protein were negative. The differential diagnosis of histiocytic syndromes is discussed.
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ranking = 0.0027924400163257
keywords = spindle
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5/43. Combined cutaneous hamartoma encompassing benign melanocytic naevus, vellus hair cyst and epidermoid cyst.

    We describe a combined cutaneous hamartoma in a 57-year-old man, which comprised a vellus hair cyst, an epidermoid cyst and an intradermal melanocytic naevus in a single facial tumour. The vellus hair cyst was filled with keratinous material with unusual slit-like lacunae. While numerous cases of epidermoid cyst have been reported in association with an intradermal melanocytic naevus, our case is a rare example of a lesion combining several hamartomatous elements.
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ranking = 1.2
keywords = naevus
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6/43. A unique case of a benign disseminated angioproliferation combining features of Kaposi's sarcoma and diffuse dermal angioendotheliomatosis.

    A female patient undergoing chronic hemodialysis had disseminated, violaceous, and partly ulcerated plaques develop on the trunk. Lesions had erupted simultaneously over a period of 4 weeks and resolved within 5 months after steroid treatment. By histopathology, the papillary dermis was densely filled with blood vessels lined by a single layer of differentiated endothelial cells, a growth pattern resembling diffuse dermal angioendotheliomatosis. In some areas, endothelial cells were spindle shaped and formed discontinuous lumina. Red blood cells were interspersed within these slits, giving the lesions a kaposiform appearance. By immunohistochemistry, endothelial cells reacted with the antibodies anti-von willebrand factor, anti-CD31, and anti-CD34 and with the lectin ulex europaeus-1. The course of the disease combined with the unusual histopathology makes this case a unique form of a benign disseminated kaposiform angioproliferation.
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ranking = 0.0027924400163257
keywords = spindle
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7/43. Fibroblastic rheumatism: a case without rheumatological symptoms.

    Fibroblastic rheumatism is a rare syndrome characterized by the association of multiple cutaneous nodules with symmetric polyarthritis. We report on a patient who presented a 4-year history of pink to skin-coloured nodular lesions symmetrically localized at para-articular sites without evident rheumatological symptoms. Histopathology of a skin nodule led to the diagnosis of fibroblastic rheumatism showing a poorly circumscribed dermal proliferation of spindle and stellate fibroblast-like cells embedded in thickened collagen bundles with a marked reduction of elastic fibres. x-rays of both hands and feet showed metacarpophalangeal, metatarsalphalangeal and interphalangeal erosions, unexpected by patient history. This case of fibroblastic rheumatism appears unique in view of the absence of any clinical manifestation of polyarthritis at 7 years from appearance of skin lesions.
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ranking = 0.0027924400163257
keywords = spindle
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8/43. nephrogenic fibrosing dermopathy: an unusual skin condition associated with kidney disease.

    We report the case of a patient who, while on long-term hemodialysis (HD), developed nephrogenic fibrosing dermopathy, a newly described sclerosing skin disorder. This disorder is characterized by thickened, hardened skin with brawny hyperpigmentation and raised plaques. The most common patient complaints are pruritus and dysesthesia. The extremities are predominantly involved with sparing of the torso and face. Dysfunction of internal organs has not been described, distinguishing it from other fibrosing conditions such as scleroderma. The skin biopsy is characterized by haphazardly arranged dermal collagen spindle cells in the reticular dermis. Extensive mucin deposits are interposed between collagen bundles and there are an increased number of fibroblast-type cells. In contrast to scleroderma, inflammatory cells are generally absent. Corticosteroid therapy can be tried, but in our patient was of no benefit.
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ranking = 0.0089825860864636
keywords = spindle cell, spindle
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9/43. nephrogenic fibrosing dermopathy with systemic involvement.

    BACKGROUND: There is a growing literature regarding sclerotic and panniculitic cutaneous conditions seen in patients with end-stage renal disease (eg, calciphylaxis and soft tissue calcification). nephrogenic fibrosing dermopathy (NFD) is a recent designation to describe cutaneous findings in patients with end-stage renal disease who developed sclerotic plaques with scleromyxedema-like histologic features. Soft tissue calcification is rare in patients with NFD and systemic involvement has not been reported. OBSERVATIONS: We describe a patient with end-stage renal disease who developed diffuse indurated woody plaques consistent with NFD in association with soft tissue calcification with catastrophic sequelae. A deep excisional biopsy specimen from the patient revealed thickened collagen bundles in the reticular dermis, plump bipolar spindle cells, and increased mucin. Focally, there were zones of calcium deposition in dermal collagen without vessel calcification. autopsy of the patient revealed extensive fibrosis and calcification of the diaphragm, psoas muscle, renal tubules, and rete testes. The patient died 11 months after developing NFD. CONCLUSION: A subset of patients with NFD may have significant systemic involvement.
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ranking = 0.0089825860864636
keywords = spindle cell, spindle
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10/43. Mycobacterial spindle cell pseudotumor of skin.

    Spindle cell pseudotumors may occur due to mycobacterial infection in immunocompromised hosts, particularly those with acquired immunodeficiency syndrome (AIDS). Most of the reported mycobacterial spindle cell pseudotumors were found in the lymph nodes. We report a case of spindle cell pseudotumor in a 37-year-old man with AIDS who presented with a firm nodule over his right arm. Histologically, the tumor was composed of proliferative spindle cells admixed with histiocytes and inflammatory cells. Ziehl-Neelsen stain revealed many acid-fast bacilli in the spindle cells and histiocytes. The acid-fast bacilli were shown to be mycobacterium avium intracellulare by culture and sequencing of the polymerase chain reaction product of mycobacterial 65-kDa heat shock protein gene. Immunohistochemically, the spindle cells were reactive to CD68, suggesting macrophage differentiation of these cells. It is important for pathologists to recognize this unusual manifestation of mycobacterial infection in immunocompromised patients and avoid mistaking the lesion for a mesenchymal neoplasm.
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ranking = 0.080843274778172
keywords = spindle cell, spindle
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