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1/7. Topical nitrogen mustard ointment with occlusion for Langerhans' cell histiocytosis of the scalp.

    BACKGROUND: Topical nitrogen mustard solution has been used as an effective alternative to corticosteroids for the treatment of cutaneous eruptions of Langerhans' cell histiocytosis (LCH). When used as an ointment under occlusion, nitrogen mustard may still be effective and possess less risk of unwanted side effects. methods: A patient with scalp LCH was treated topically with nitrogen mustard ointment 0.01% under occlusion. RESULTS: The lesions cleared in 3 weeks without irritation. CONCLUSION: Topical nitrogen mustard ointment 0.01% under occlusion is a well-tolerated, non-irritating treatment for scalp LCH.
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2/7. Langerhans cell histiocytosis of the female genital tract.

    Langerhans cell histiocytosis (LCH) of the female genital tract is rare. Four new cases are reported, and there is a review of the 38 cases in the literature. This disease may involve the vulva, vagina, cervix, endometrium, and ovary. Four distinct patient groups, segregated on the basis of initial presentation and subsequent anatomic extent of disease, were categorized as follows: (1) "pure" genital LCH, (2) genital LCH with subsequent multi-organ involvement, (3) oral or cutaneous LCH with subsequent genital and multi-organ involvement, and (4) diabetes insipidus with subsequent genital and multi-organ disease. Although involvement of the genital tract can occur at any age, it is most common in young adulthood. Clinically, LCH may mimic either primary neoplasia or various inflammatory lesions; the major pathologic differential diagnosis is venereal and other inflammatory diseases. The pure genital form may have a distinct nosologic position in the spectrum of LCH similar to the "pure," self-limited cutaneous histiocytosis seen in infants. There is no correlation between histologic findings and the outcome of the genital lesions. There is also no correlation between clinical presentation and/or the extent of involvement and outcome of genital lesions; complete regression, partial improvement, persistent lesions, and recurrences were seen in all four groups of patients. The treatment of genital LCH is not well defined and is highly individualized. Therapy has included surgery, radiation, topical corticosteroids, topical nitrogen mustard, systemic chemotherapy, and combination therapy; mixed results were obtained with all treatment modalities. Although no modality has been shown to yield a superior outcome, complete surgical excision is advocated as initial therapy.
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ranking = 0.125
keywords = mustard
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3/7. Letterer-Siwe disease in adults.

    histiocytosis X (HX) is a rare disorder of histiocytic proliferation characterized by a broad spectrum of clinicopathologic disease. An unusual case of Letterer-Siwe disease (LSD) or subacute disseminated HX in a 71-year-old woman is presented. The patient had a 3-year history of splenomegaly before skin lesions developed. She presented to our clinic at 1.5 years later and the diagnosis of HX was made by skin biopsy. Topical nitrogen mustard (NM) therapy resulted in complete clearing of cutaneous lesions. Her condition was stable over the next 10 months. However, she subsequently suffered a rapid and fatal dissemination of her disease. Systemic treatment with prednisone, vinblastine sulphate, and suppressin A (SA) (a calf thymus derived hormone preparation that specifically induces suppressor T-cells) was ineffective. Characteristic histopathologic, immunohistochemical, and electron microscopic findings of HX are illustrated. A review of the adult cases of LSD and treatment options for HX are presented and discussed.
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ranking = 0.125
keywords = mustard
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4/7. histiocytosis X in two infants--treated with topical nitrogen mustard.

    Two infants suffering from histiocytosis X were treated successfully with a topically applied nitrogen mustard. All cutaneous symptoms disappeared completely within 2 weeks in one case while the skin improved significantly in the other. No irritation or dermatitis was experienced from the treatment. The only side-effect noted was hyperpigmentation.
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ranking = 0.625
keywords = mustard
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5/7. hand-Schuller-Christian disease in a septuagenarian.

    hand-Schuller-Christian disease is a disease primarily affecting children, occasionally young adults, and rarely older adults. Very few cases have been reported in individuals past their fifth decade. A review of the English language literature indicates that the onset of hand-Schuller-Christian disease occurs after the fifth decade in less than 4% of reported cases. A 75-year-old woman's symptoms of hand-Schuller-Christian disease began in her 76th year, and her cutaneous symptoms were controlled with a topically applied nitrogen mustard.
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ranking = 0.125
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6/7. histiocytosis X: treatment with topical nitrogen mustard.

    The case histories of two elderly patients with cutaneous histiocytosis X treated topically with nitrogen mustard are presented. The cutaneous lesions cleared within 2 to 3 weeks, and remission was maintained with daily topical administration of nitrogen mustard. The clinical impression of improvement was substantiated by light and electron microscopic studies prior to and after therapy.
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ranking = 0.75
keywords = mustard
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7/7. Langerhans cell histiocytosis in an adult.

    We present the case of a 27-year-old Caucasian woman who suffered from Langerhans cell histiocytosis with axillary and scalp involvement. She also developed diabetes insipidus after 2 years of skin lesions. Topical nitrogen mustard therapy was performed for the skin lesions, but we had to stop this therapy because of severe local irritation and systemic urticaria. Afterwards, we administered etoposide systemically, but no improvement appeared in 6 weeks. Finally we used methotrexate for 3 months and the result was very good at the end of the first month.
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ranking = 0.125
keywords = mustard
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