Nodular `Pure' Mucocutaneous Histiocytosis X in an Adult

Caputo, Ruggero; Gianotti, Raffaele; Monti, M.

doi:10.1001/archderm.1987.01660340034012

Cited by 7 publications

(3 citation statements)

References 5 publications

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“…However, patients presenting with generalized papular lesions and/or ulcerating lesions in the skin folds appear much more resistant to treatment. Many agents including topical and systemic corticosteroids, topical nitrogen mustard, imiquimod, narrowband UVB, PUVA, radiotherapy, thalidomide, methotrexate, retinoids, vinblastine, interferon and cladribine have been tried and have been reported successful in some patients 4–67 . However, no single treatment was effective in all cases.…”

Section: Discussionmentioning

confidence: 99%

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: Discussionmentioning

confidence: 99%

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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“…Langerhans cell histiocytosis affects mainly infants and children (2,3), however, it can occur at any age (16,17). It can be classified into localized and disseminated forms (3) ( Table 1).…”

Section: Discussionmentioning

confidence: 99%