Rapid resolution of primary vulval adult Langerhans cell histiocytosis with very potent topical corticosteroids

Foley, Susan M.; Panting, Katherine; Bell, Hazel Κ.; Leonard, Niamh; Franks, Andrea

doi:10.1111/j.1440-0960.2009.00585.x

Cited by 11 publications

(10 citation statements)

References 15 publications

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“…The differential diagnoses include inflammatory ulcers, lichen sclerosus, plaque psoriasis, and other sexually transmitted diseases, such as common herpes simplex, chancroid, granuloma inguinale, lymphogranuloma venereum, and syphilitic chancres. The disease may be mistaken for squamous cell carcinoma, melanoma, extramammary Paget's disease, cutaneous tuberculosis, and Behçet's disease …”

Section: Discussionmentioning

confidence: 99%

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Vulvar Langerhans cell histiocytosis and thalidomide: an effective treatment option

et al. 2016

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Section: Discussionmentioning

confidence: 99%

“…The prognosis is highly variable and depends on the extent of disease and which organs are affected. Overall survival at 5 years is 90% and is higher in patients with localized disease …”

Section: Discussionmentioning

confidence: 99%

Vulvar Langerhans cell histiocytosis and thalidomide: an effective treatment option

et al. 2016

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“…LCH isolated to the vulva is a rare phenomenon, with only 23 cases described in the current literature (Table ). The age at presentation ranges from 1 to 85, and present as erosions, ulcers, umbilicated papules and verrucous lesions . Because of the rarity of LCH occurring in this particular location, no single treatment protocol has been established .…”

Section: Discussionmentioning

confidence: 99%

“…Some patients were treated by complete surgical excision with or without adjuvant radiation or chemotherapy, while others were treated by local radiation or systemic chemotherapy. Three other cases were treated with topical steroids alone as a first‐line therapy, and complete remission was observed in two of the cases . A recent review article by El‐Safadi et al found that local recurrence occurred in 62% of the reported cases, often after prolonged periods of clinical remission.…”

Section: Discussionmentioning

confidence: 99%

Langerhans cell histiocytosis associated with lichen sclerosus of the vulva: case report and review of the literature

et al. 2012

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Langerhans cell histiocytosis (LCH) is characterized by a clonal proliferation of bone marrow-derived Langerhans cells. While cutaneous involvement is relatively common, LCH restricted to the vulvar area is a rare phenomenon and can occur in different clinical settings. Occasionally, vulvar LCH heralds subsequent multi-organ involvement with an aggressive clinical course. Even cases of LCH isolated to the vulvar area can present with local recurrences despite excision and radiation. We present a case of a 68-year-old female with a 1-month history of pruritic lesions on her vulva. Physical examination showed whitish plaques with scattered nodular areas on the labia majora. A vulvar biopsy showed a background of lichen sclerosus (LS) with foci of oval to polygonal cells with moderately abundant eosinophilic cytoplasm and folded nuclei showing frequent nuclear grooves. Immunohistochemical staining showed that the cells were positive for CD1a and S-100, confirming the diagnosis of LCH. On further workup, there was no evidence of disseminated disease involving other organs. While vulvar LCH is uncommonly seen, and with only one previous case report in the literature associated in the setting of lichen sclerosus, this case illustrates the importance of recognizing this condition and ensuring proper clinical follow-up to rule out a systemic involvement.

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“…During followup only two of these patients (patients 6 and 9) developed new skin lesions. Patients with generalized reddish-brown papules (patients [10][11][12][13][14] and particularly patients with ulcerating lesions in the skin folds or anogenital region (patients [15][16][17][18] were more resistant to treatment (see Table 2). Initial treatment resulted in a complete remission in only two of nine patients.…”

Section: Treatment and Follow-upmentioning

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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Rapid resolution of primary vulval adult Langerhans cell histiocytosis with very potent topical corticosteroids

Cited by 11 publications

References 15 publications

Vulvar Langerhans cell histiocytosis and thalidomide: an effective treatment option

Vulvar Langerhans cell histiocytosis and thalidomide: an effective treatment option

Langerhans cell histiocytosis associated with lichen sclerosus of the vulva: case report and review of the literature

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

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