Satisfactory Remission Achieved by PUVA Therapy in Langerhans Cell Hisiocytosis in an Elderly Patient

Sakai, Hiroyuki; Ibe, Masaki; Takahashi, Hidetoshi; Matsuo, Shinobu; Okamoto, Kensaku; Makino, Isao; Oomori, Yukio; Iizuka, Hajime

doi:10.1111/j.1346-8138.1996.tb03966.x

Cited by 31 publications

(17 citation statements)

References 14 publications

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“…Phototherapy: Psoralen plus ultraviolet A (PUVA) [31] and narrow band ultraviolet (UV) B [32] are effective in treating cutaneous LCH in individual case reports. It is difficult to treat patients with intertriginous or scalp involvement and would be contraindicated in penile disease (Grade C1).…”

Section: Background Process Of Development and Restrictionsmentioning

confidence: 99%

Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net

Girschikofsky

Aricò

Castillo

et al. 2013

Orphanet J Rare Dis

313

446

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Langerhans Cell Histiocytosis (LCH) is an orphan disease of clonal dendritic cells which may affect any organ of the body. Most of the knowledge about the diagnosis and therapy is based on pedriatic studies. Adult LCH patients are often evaluated by physicians who focus on only the most obviously affected organ without sufficient evaluation of other systems, resulting in patients being underdiagnosed and/or incompletely staged. Furthermore they may be treated with pediatric-based therapies which are less effective and sometimes more toxic for adults. The published literature on adult LCH cases lacks a comprehensive discussion on the differences between pediatric and adult patients and there are no recommendations for evaluation and comparative therapies. In order to fill this void, a number of experts in this field cooperated to develop the first recommendations for management of adult patients with LCH. Key questions were selected according to the clinical relevance focusing on diagnostic work up, therapy, and follow up. Based on the available literature up to December 2012, recommendations were established, drafts were commented by the entire group, and redrafted by the executive editor. The quality of evidence of the recommendations is predominantly attributed to the level of expert opinion. Final agreement was by consensus.

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Section: Background Process Of Development and Restrictionsmentioning

confidence: 99%

Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net

Girschikofsky

Aricò

Castillo

et al. 2013

Orphanet J Rare Dis

313

446

View full text Add to dashboard Cite

show abstract

“…In skin‐only cases, weekly oral methotrexate (MTX) with or without 6‐mercaptopurine (or its prodrug azathioprine) is recommended . Light therapies in skin‐only patients have been reported to be effective . In contrast to pediatric patients, radiotherapy could be an option for SS‐LCH with bone lesions, with acceptable side‐effects .…”

Section: Treatments For Ss‐lchmentioning

confidence: 99%

Langerhans cell histiocytosis in adults: Advances in pathophysiology and treatment

2018

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Langerhans cell histiocytosis (LCH) is a rare systemic disorder characterized by the accumulation of CD1a+/Langerin+ LCH cells and wide‐ranging organ involvement. Langerhans cell histiocytosis was formerly referred to as histiocytosis X, until it was renamed in 1987. Langerhans cell histiocytosis β was named for its morphological similarity to skin Langerhans cells. Studies have shown that LCH cells originate from myeloid dendritic cells rather than skin Langerhans cells. There has been significant debate regarding whether LCH should be defined as an immune disorder or a neoplasm. A breakthrough in understanding the pathogenesis of LCH occurred in 2010 when a gain‐of‐function mutation in BRAF (V600E) was identified in more than half of LCH patient samples. Studies have since reported that 100% of LCH cases show ERK phosphorylation, indicating that LCH is likely to be a clonally expanding myeloid neoplasm. Langerhans cell histiocytosis is now defined as an inflammatory myeloid neoplasm in the revised 2016 Histiocyte Society classification. Randomized trials and novel approaches have led to improved outcomes for pediatric patients, but no well‐defined treatments for adult patients have been developed to date. Although LCH is not fatal in all cases, delayed diagnosis or treatment can result in serious impairment of organ function and decreased quality of life. This study summarizes recent advances in the pathophysiology and treatment of adult LCH, to raise awareness of this “orphan disease”.

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“…Moreover, multiple organs, including the bones, eyes, lymph nodes, marrow, liver and spleen, might be affected. Psoralen plus ultraviolet A (PUVA) has been reported to be effective for the treatment of cutaneous LCH lesions 4,5 . In addition, topical nitrogen mustard and thalidomide therapy and p.o.…”

Section: Introductionmentioning

confidence: 99%

“…Psoralen plus ultraviolet A (PUVA) has been reported to be effective for the treatment of cutaneous LCH lesions. 4,5 In addition, topical nitrogen mustard and thalidomide therapy and p.o. administration of isotretinoin are thought to be effective in adult patients with cutaneous LCH.…”

Section: Introductionmentioning

confidence: 99%

Langerhans cell histiocytosis treated with narrow-band ultraviolet B

Aihara

Hasegawa

Okuyama

et al. 2010

The Journal of Dermatology

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A 58-year-old man had widespread multiple erythemas that had been untreated for 20 years. The cutaneous lesions increased in size for a year. Histopathological examination revealed increased number of large, dysplastic, histiocytic cells in the dermis; most of these cells tested positive for Langelin, CD1a and S-100 staining. The patient was diagnosed with Langerhans cell histiocytosis (LCH) and treated with narrow-band ultraviolet B (NB-UVB) radiation. The erythema improved after treatment, but the tumor remained. Therefore, the tumor was surgically excised. NB-UVB therapy might be effective for the treatment of superficial lesions, but not tumoral lesions of LCH.

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Satisfactory Remission Achieved by PUVA Therapy in Langerhans Cell Hisiocytosis in an Elderly Patient

Cited by 31 publications

References 14 publications

Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net

Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net

Langerhans cell histiocytosis in adults: Advances in pathophysiology and treatment

Langerhans cell histiocytosis treated with narrow-band ultraviolet B

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