Histiocytosis X in Two Adults

Nethercott, James R.

doi:10.1001/archderm.1983.01650260065021

Cited by 17 publications

(7 citation statements)

References 21 publications

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“…Staging results showed abnormalities in 12 of 74 cases (Table 2). In four of 74 patients staging revealed extracutaneous LCH in bone and/or lungs 4–7 . Three patients without a history of lymphoma or leukaemia showed abnormalities in blood and/or bone marrow, and two of them developed a (myelo)monocytic leukaemia 8–10 .…”

Section: Resultsmentioning

confidence: 96%

Langerhans cell histiocytosis first presenting in the skin in adults: frequent association with a second haematological malignancy

Edelbroek¹,

Vermeer²,

Jansen

et al. 2012

British Journal of Dermatology

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Summary Background Langerhans cell histiocytosis (LCH) in adults first presenting in the skin is rare. Guidelines for staging, treatment and follow‐up are lacking. Objectives To better define staging procedures, treatment results and clinical course in adult patients with LCH first presenting in the skin. Methods Eighteen adult patients with LCH first presenting in the skin were collected from five centres collaborating in the Dutch Cutaneous Lymphoma Group. Clinical records and (skin) biopsy specimens were reviewed and follow‐up data were obtained. A literature search on adult patients with LCH presenting in the skin was performed. Results Staging procedures showed extracutaneous disease in three of 16 patients who were adequately staged. One patient had a histologically confirmed lytic LCH bone lesion, while two patients had a myelodysplastic syndrome. During follow‐up two of 18 patients developed extracutaneous localizations of LCH. Five patients developed a second haematological malignancy, including (myelo)monocytic leukaemia (two cases), histiocytic sarcoma (one case), diffuse large B‐cell lymphoma (one case) and peripheral T‐cell lymphoma (one case). Review of the literature revealed six other adult patients with a second haematological malignancy preceding or following a diagnosis of LCH. Conclusions The results of the present study suggest an increased risk of a second haematological malignancy in adult patients with LCH presenting in the skin. Extensive staging at presentation and long‐term follow‐up are therefore warranted in such patients.

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Section: Resultsmentioning

confidence: 96%

Langerhans cell histiocytosis first presenting in the skin in adults: frequent association with a second haematological malignancy

Edelbroek¹,

Vermeer²,

Jansen

et al. 2012

British Journal of Dermatology

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“…Most patients with classical ALSD have a poor prognosis because of systemic involvement; in contrast, there seems to be a subset of cases, in whieh only or mainly the skin is affected [3,8,14,15], Involvement of the skin as the only manifesta- tion of Langerhans cell histiocytosis is uncommon; only 22 adult cases of pure cutaneous Langerhans cell histiocytosis have been reported in the literature [3,11,14,[16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31]. The clinical features are similar to infantile ALSD [5,32].…”

Section: Discussionmentioning

confidence: 99%

Langerhans‐cell histiocytosis confined to the skin of an elderly patient

Pauluzzi

Trevisan

Sartori

1994

Acad Dermatol Venereol

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“…1,2 Past treatments for cutaneous LCH have included surgical excision, topical 1,3,4 or systemic steroids, localized radiation therapy, 5 topical nitrogen mustard, [6][7][8][9] phototherapy, 10,11 methotrexate, 12 total skin electron beam therapy, vinblastine, 13 and systemic interferon. 14, 15 We report the first case of adult cutaneous LCH responsive to topical imiquimod.…”

Section: Adult Cutaneous Langerhans Cell Histiocytosis Responsive To mentioning

confidence: 99%

“…1,3,4,7-9 Whereas mild skin involvement responded to topical steroids for 2 out of 3 patients, 1,3,4 more severe and widespread cutaneous forms were responsive to topical mechlorethamine in 3 out of 3 patients, at times resulting in up to two and a half years of remission. [7][8][9] Systemic interferon (IFN) has been effective in the treatment of widespread skin-limited LCH refractory to multiple therapies for 2 out of 2 adult patients, 14,15 resulting in complete remission for up to 29 months in one adult. 14 Imiquimod is a topical immunomodulator believed to function as a potent stimulator of T helper-1 cytokines causing local release of interferon-alfa, 16 IFN gamma, and interleukin-2.…”

Section: Adult Cutaneous Langerhans Cell Histiocytosis Responsive To mentioning

confidence: 99%