Vulvar Langerhans’ cell histiocytosis: a case report

Mlyncek, M; Uharcek, P; Durcanský, D

doi:10.1080/00016340600613709

Cited by 2 publications

(1 citation statement)

References 6 publications

(13 reference statements)

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“…Later, these were found to be different manifestations of a single disease named histiocytosis X [4]. Recently this classification has changed to LCH, in recognition of the primary cell involved.…”

Section: Discussionmentioning

confidence: 99%

Isolated Langerhans cell histiocytosis of the vulva: a case report and review of the literature

et al. 2007

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Isolated Langerhans cell histiocytosis (LCH) of the female genital tract is very rare. A review of the literature revealed that only 15 cases of primary vulvar LCH have previously been published in the English literature. We describe an additional case of confined vulvar LCH. A 49-year-old woman presented with an ulcerous lesion that turned out to be LCH confined to the vulva only. After surgical excision, four recurrences followed, which were treated again by surgery. After the fourth recurrence, adjuvant radiotherapy was applied. When the fifth recurrence occurred, only surgical excision was performed, and the patient has now been disease-free for 51 months. There are no standard treatment options for this rare disease. The most effective treatment options still remain elusive. In our case in the end surgery proved to be effective.

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Section: Discussionmentioning

confidence: 99%

Isolated Langerhans cell histiocytosis of the vulva: a case report and review of the literature

et al. 2007

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Langerhans Cell Histiocytosis in an Adult Female with Multisystem Involvement

Gupta¹,

Singh²,

Chugh³

et al. 2023

The Indian Journal of Chest Diseases and Allied Sciences

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Langerhans cell histiocytosis (LCH) also known as histiocytosis X, is a rare systemic disorder arising from the clonal proliferation of myeloid dendritic cells (histiocytes) with a tendency to involve single or multiple organ systems with variable clinical course and prognosis. Clinical presentation usually depends on the site of involvement. The organs commonly affected in adults by order of decreasing frequency include lungs, bone, skin, pituitary glands, lymph nodes, and the liver. Vulval and perianal involvement is extremely rare in adults. We describe the case of a 31-year-old non-smoker adult female with multisystemic LCH involving the vulva, perianal region, and lung. Probable involvement of other sites with LCH included mandibular bone, pituitary gland, skin, lymph nodes, liver, thyroid, and colon. She is undergoing systemic chemotherapy and has completed two cycles of cytarabine and steroids without any complications. Treatment is not standardized due to the very less incidence of the disease and inadequate knowledge regarding its pathophysiology. Langerhans cell histiocytosis remains a major concern for treating physicians because of its rarity with many faces and requires careful consideration for management.

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Vulvar Langerhans’ cell histiocytosis: a case report

Cited by 2 publications

References 6 publications

Isolated Langerhans cell histiocytosis of the vulva: a case report and review of the literature

Isolated Langerhans cell histiocytosis of the vulva: a case report and review of the literature

Langerhans Cell Histiocytosis in an Adult Female with Multisystem Involvement

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