Langerhans cell histiocytosis of vulva and perineum

Sirka, Chandra Sekhar; Sethy, Madhusmita; Rout, Arpita Nibedita; Sahu, Kananbala

doi:10.25259/ijdvl_399_20

Cited by 3 publications

(1 citation statement)

References 5 publications

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“…Genital involvement with LCH has been previously reported in literature with various patterns such as pure genital LCH, genital tract LCH with subsequent multiorgan involvement, oral or cutaneous LCH with subsequent genital and multiorgan involvement, and diabetes insipidus with organ involvement. [48][49][50] Vulva is the most commonly involved site followed by the vagina, cervix, endometrium, and ovary. Genital involvement can occur at any age but is most commonly seen in young adults.…”

Section: Discussionmentioning

confidence: 99%

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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Section: Discussionmentioning

confidence: 99%

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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What is a vulva?

Zdilla

2022

Anat Sci Int

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Recurrent multisystem Langerhans cell histiocytosis involving the female genitalia: A case report

Yuan,

Zhang,

Guo

et al. 2024

World J Clin Cases

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BACKGROUND Langerhans cell histiocytosis (LCH) is a histiocytic proliferative disease caused by clonal proliferation of Langerhans cells, which is currently defined as an inflammatory myeloid tumor. It is rare in adults, with an incidence of 1–2 per million, and is highly heterogeneous in clinical presentation, with unpredictable disease progression and outcome. CASE SUMMARY A 52-year-old postmenopausal female patient presented to the gynecology department in July 2023 with bilateral vulvar masses. She was diagnosed with recurrent multisystem LCH. The patient had previously been diagnosed with a single-system and single-focal LCH in October 2021 due to a right maxillofacial mass, which resolved after surgical treatment. A chemotherapy regimen was developed after multidisciplinary consultation. Six cycles of chemotherapy resulted in partial remission, and maintenance chemotherapy is currently being administered. CONCLUSION Recurrent LCH involving the bilateral vulva has been poorly reported. Comprehensive imaging and pathological evaluation is important for diagnosis. The model of joint multidisciplinary specialist diagnosis and treatment is worthy of clinical application.

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Langerhans cell histiocytosis of vulva and perineum

Abstract: Dermoscopy compared with naked eye examination for the diagnosis of primary melanoma: A meta-analysis of studies performed in a clinical setting.

Cited by 3 publications

References 5 publications

Langerhans Cell Histiocytosis in an Adult Female with Multisystem Involvement

Langerhans Cell Histiocytosis in an Adult Female with Multisystem Involvement

What is a vulva?

Recurrent multisystem Langerhans cell histiocytosis involving the female genitalia: A case report

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