Two case report studies of Langerhans cell histiocytosis with an analysis of 918 patients of Langerhans cell histiocytosis in literatures published in China<sup>†</sup>

Zhu, Li; Li, Yanqiu; Yan, Wu; Qu, Xiaolei; Hamze, Firas; Chen, Siyuan; Chen, Hong-Xiang; Li, Jiawen; Wang, Chunsen; Tu, Yating; Chang-zheng, Huang

doi:10.1111/j.1365-4632.2009.04360.x

Cited by 21 publications

(27 citation statements)

References 7 publications

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“…Previous to the unifying concept advanced by Jaffe and Lichtenstein in 1944, some clinical syndromes had been independently described and later named eponymously [4]. Letterer-Siwe disease usually occurs in children under one year of age, has a poor prognosis and manifests itself as fever and skin rash, hepatomegaly, splenomegaly, pulmonary symptoms and anemia [5]. Hand-Schuller-Christian disease usually affects children older than three years old and is defined by the triad of exophthalmos, diabetes insipidus and skull defect.…”

Section: Resultsmentioning

confidence: 99%

“…Hand-Schuller-Christian disease usually affects children older than three years old and is defined by the triad of exophthalmos, diabetes insipidus and skull defect. Eosinophilic granuloma refers to a single bone lesion in older children, teenagers or adults with symptoms of pain, swelling and, eventually, pathologic fracture, but with a very good prognosis [1,5,6]. However, many cases do not fit exactly in any of these late descriptions, which have more historical importance than clinical usage and, currently, cases have been classified into two major categories:…”

Section: Resultsmentioning

confidence: 99%

“…Vesicles and pustules may also come into being. Nodular lesions are particularly pres- and anti-S100 antibodies, with negativity for anti-CD68 [5,8]. Langerin is another useful highly specific Langerhans cell marker.…”

Section: Resultsmentioning

confidence: 99%

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Langerhans cell histiocytosis—a case report

Jeunon

Sousa

Santos-Rodrigues

et al. 2012

Dermatol Pract Concept

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A 17-year-old male presented for dermatologic consultation with slightly elevated reddish papules covered by yellowish scales in the scalp for the last two years and reddish and indurated ulcers in the perineum lasting six months. Additional complaints included polyuria, polydipsia, delay in the development of secondary sexual characteristics and hearing loss of the right ear secondary to a medium otitis. Lesions from scalp and perineum were sampled for histopathologic examination and revealed a dense cellular infiltrate made up of mononuclear cells with conspicuous eosinophilic cytoplasm and large cleaved vesicular nucleus, some of them with shapes resembling the format of a kidney and others reminiscent of coffee beans. Numerous intermingling eosinophils were present. The diagnosis of Langerhans cell histiocytosis was then rendered and confirmed by positive immunostaining of neoplastic cells for anti-CD1a and anti-S100 protein antibodies. The work-up revealed diabetes insipidus, hypogonadotropic hypogonadism, hiperprolactenemia, growing-hormone deficiency and thickness of the pituitary stalk. The patient was treated with prednisone and vinblastin based chemotherapy regimen for six months with complete remission, but presented recurrence of some lesions in the scalp, which were handled with topical mustard and corticosteroids. After chemotherapy, the endocrinologic disturbances were corrected with hormonal replacement therapy. The patient is currently in good health with a follow-up of five years. abstRaCt

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Langerhans cell histiocytosis—a case report

Jeunon

Sousa

Santos-Rodrigues

et al. 2012

Dermatol Pract Concept

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“…Cutaneous involvement is frequently present in LCH. Skin manifestation is more common in children, but numerous adult patients are also presented with skin lesions . As up to 30–80% of LCH patients develop skin symptoms, the diagnosis is frequently provided by histopathological examination of the skin biopsy …”

Section: Introductionmentioning

confidence: 99%

“…Based on the presented data it can be estimated that so far approximately not more than 30 skin samples were examined . As skin is a frequently involved organ and is an easily accessible site for biopsy to establish the histopathological diagnosis of LCH patients it is very important to obtain data on the availability of BRAF mutation status in such samples too …”

Section: Introductionmentioning

confidence: 99%