Cases reported "Alopecia"

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1/6. common variable immunodeficiency treated with a recombinant human IgG, tumour necrosis factor-alpha receptor fusion protein.

    common variable immunodeficiency (CVI) is characterized by a failure in B-cell differentiation and impaired immunoglobulin secretion, but with a variable clinical presentation, including the development of sarcoidal granulomas and autoimmune diseases, as well as an increased incidence of malignancies. We present a 21-year-old white man who carried a diagnosis of juvenile rheumatoid arthritis and presented 6 years later with scarring alopecia showing sarcoidal granulomas. Further work confirmed the diagnosis of CVI, and with increasing systemic symptoms, it was elected to treat the patient with a tumour necrosis factor (TNF)-alpha antagonist, a TNF-alpha receptor IgG1 fusion protein. The patient showed improvement in his systemic symptoms and some hair regrowth after 3 months of therapy, and continued improvement in his systemic disease with only mild scalp hair thinning in the areas of prior involvement after almost 1 year of therapy. CVI and sarcoid may have overlapping clinical and immunological findings. Previous therapies for CVI, including intravenous immunoglobulin, have not altered the mortality of the disease. TNF-alpha is a primary cytokine and is elevated in CVI, and specific inhibition of TNF-alpha in this patient was effective in moderating his disease, including his skin disease.
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2/6. Great cases from the University of pennsylvania.

    New skin diseases are reported yearly. Some well-known conditions may develop unusual manifestations. Other cutaneous findings are simply rare. While patients with such diseases represent only a small fraction of our practice they are an important subset of our experience. Many of these diseases are treatable, pinpoint the discovery of internal disease, or simply allow for a diagnosis and prognosis to be given to a concerned patient who had previously escaped classification. Such cases expand our knowledge and provide excitement to our day. Several patients from whom I have learned the most in the last few years are detailed for your enjoyment.
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3/6. Treatment of lichen planopilaris with mycophenolate mofetil.

    Mycophenolate mofetil (MMF) is an immunosuppressive drug that has recently been used to treat autoimmune and inflammatory skin diseases. We report the first case of lichen planopilaris (LPP) successfully treated with MMF. The treatment of our patient demonstrates a novel therapeutic option for patients with LPP; MMF treatment may be preferable to azathioprine treatment because MMF has a safer adverse-effect profile. Larger studies must be performed to establish the risk-benefit ratio of various therapeutic dosages of MMF for these patients.
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4/6. alopecia neoplastica due to metastatic breast carcinoma vs. extramammary Paget's disease: mimicry in epidermotropic carcinoma.

    Clinically similar skin diseases can exhibit phenotypic mimicry as demonstrated here for alopecia neoplastica. An 89-year-old female patient had been treated for scalp eczema for several years until biopsy revealed pagetoid carcinoma. Her clinical history showed a mastectomy 20 years ago. For that reason the differential diagnosis was extramammary Paget's disease or epidermotropic metastasis of the breast carcinoma. immunohistochemistry revealed positivity of MUC1, indicating that the latter is the case.
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5/6. Syringolymphoid hyperplasia with alopecia.

    A 26-year-old man presented with an unusual skin lesion consisting of a hairless area on the left leg studded with reddish-brown papules, together with follicular hyperkeratosis and circumscribed anhidrosis. Skin biopsies showed dense lymphocytic infiltrates in the dermis and around hyperplastic sweat ducts and glands. Similar clinical and histological features have been described in two previous case reports. We propose the term syringolymphoid hyperplasia with alopecia for this unusual skin disease.
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6/6. Follicular mucinosis associated with scarring alopecia, oligoclonal T-cell receptor V beta expansion, and staphylococcus aureus: when does follicular mucinosis become mycosis fungoides?

    A diagnosis of alopecia mucinosa, occurring as a single scalp lesion, was made in a 40-year-old white woman who had a history of trauma. Follicular mucinosis, staphylococcus aureus, and oligoclonal expansion of the T-cell receptor V beta chain genes 6 and 7 were present in the skin. Epidermotropic T-cell skin diseases with oligoclonal T-cell proliferations may be the result of HLA- and cytokine-determined reaction patterns to persistent antigens.
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