Diverse Cutaneous Presentations of Langerhans Cell Histiocytosis in Children: A Retrospective Cohort Study

Morren, Marie‐Anne; Broecke, Katrien Vanden; Vangeebergen, Leen; Sillevis-Smitt, J.H.; Berghe, P Van Den; Hauben, Esther; Jacobs, Sandra; Gool, Stefaan W. Van

doi:10.1002/pbc.25834

Cited by 77 publications

(76 citation statements)

References 26 publications

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“…The manifestation of LHCH often resembles eczematous, psoriasiform or acneiform skin lesions with coalescing papules or plaques which may show scale or crust . Eczematous lesions can resemble seborrhoeic dermatitis . An infiltrated nodular type is also being seen, and there can be ulceration, especially in skin folds .…”

Section: Skin Manifestations Of Histiocytosesmentioning

confidence: 99%

Histiocytosis – cutaneous manifestations of hematopoietic neoplasm and non‐neoplastic histiocytic proliferations

Luder

Nordmann

Ramelyte

et al. 2018

Acad Dermatol Venereol

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Histiocytoses are rare disorders characterized by the accumulation of cells derived from macrophages, dendritic cells or monocytes in various tissues. There is a broad spectrum of disease manifestations with some subtypes commonly showing skin lesions, while in others, the skin is rarely involved. Here, we describe cutaneous manifestations of histiocytoses belonging to the Langerhans group (L group), the group of cutaneous and mucocutaneous histiocytoses (C group) and the group of Rosai-Dorfman disease (RDD) and related histiocytoses (R group) according to the current classification. Characteristic clinical presentations noted were a rust-brown colour or xanthomatous aspect in many cases of histiocytoses. Histological criteria for differentiation are described. Immunohistochemistry shows positivity for S100 and CD1a in Langerhans-cell histiocytoses (LHCH) of the L group, while CD68 positivity with S100 and CD1a negativity are typical in histiocytoses of the C group of cutaneous and mucocutaneous histiocytoses. RDD in the R group shows positivity for S100 and CD68, while CD1a is negative. We further review the pathogenesis of LHCH based on insights on the central role of the mitogen-activated protein (MAPK) kinase pathway. Common mutations in various histiocytic populations of diverse ontogeny and at different stages of differentiation may be responsible for the diverse clinical picture of this neoplastic entity. For histiocytoses of the C group and R group, a reactive origin is discussed with the exception of the disseminated form of juvenile xanthogranuloma. We suggest exploring the role of an origin from skin residing histiocytes for the isolated cutaneous manifestation in some types. With regard to therapeutic options, skin-directed therapies are the first choice in limited disease, while systemic chemotherapy has traditionally been used in extensive disease. In Langerhans-cell histiocytoses and related entities, therapy by BRAF inhibition has led to a breakthrough especially in patients with an activation of the MAPK pathway.

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Section: Skin Manifestations Of Histiocytosesmentioning

confidence: 99%

Histiocytosis – cutaneous manifestations of hematopoietic neoplasm and non‐neoplastic histiocytic proliferations

Luder

Nordmann

Ramelyte

et al. 2018

Acad Dermatol Venereol

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“…Prema nekim autorima 53% oboljelih sa multisistemskim oblikom bolesti ima i kožne lezije (1,2,8). Pojava kožnih lezija nakon 3 mjeseca života koje progrediraju tokom vremena, razvoj eksternog otitisa ili lezije na sluzokoži nedvojbeno se javljaju kod pacijenata koji će razviti multisistemski oblik bolesti (13).…”

Section: Diskusijaunclassified

“…Oboljeli sa ekstenzivnim kožnim lezijama češće zahtijevaju hemoterapiju u odnosu na oboljele sa diskretnim promjenama koje pokazuju spontanu rezoluciju ili mogu biti tretirani samo topikalnim kortokosteroidima (16). Kod kortikosteroid rezistentnih slučajeva kožne lezije se mogu tretirati i topikalnim nitrogen mustradom ili psoralenom uz ultravioletnu A svjetlost (PUVA) (1,13). Solitarne koštane lezije mogu biti liječene samo kiretažom uz lokalnu intralezionu primjenu kortikosterida.…”

Section: Diskusijaunclassified

“…Oboljeli sa multisistemskim oblikom bolesti sa/bez zahvatanja riziko organa zahtijevaju sistemsku polihemioterapiju prema LCH protokolu. BRAF i MEK inhibitori bi mogli predstavljati terapiju budućnosti (13).…”

Section: Diskusijaunclassified

“…Pored toga, činjenica da se i kod djece sa jednosistemskim oblikom bolesti i spontanom rezolucijom promjena mogu javiti kasni recidivi ili progresija bolesti u multisistemski oblik zahtijeva brižljivo praćenje ovih pacijenata tokom prvih 5 godina po završenom liječenju (13).…”

Section: Diskusijaunclassified

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Histiocitoza Langerhansovih Ćelija - Od Jednosistemskog Do Multisistemskog Oblika

Malčić-Zanić¹,

Predojević-Samardžić²,

Đurđević-Banjac³

et al. 2018

SCEPED

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SažetakHistiocitoza Langerhansovih ćelija je rijetko oboljenje nastalo usljed proliferacije antigen prezentujućih Langerhansovih dendriditičnih ćelija iz koštane srži koje infiltrišu različite organe. Bolest je nepredvidivog toka, od moguće spontane remisije pa do teških diseminovanih oblika sa oštećenjem vitalnih organa i letalnim ishodom uprkos primjenjenoj terapiji.U radu je prikazan slučaj 22 mjeseca stare djevojčice kod koje su se od drugog mjeca života javile promjene po koži sa tendencijom pogoršanja tokom vremena. U dobi od 22 mjeseca djevojčici je verifikovana tumorska masa iza lijevog uha sa znacima diabetes insipidusa (žedjanje, poliurija, polidipsija). Dijagnoza LCH je postvaljena patohistološki na osnovu bioptata kože i tumorske mase lijeve temporalne kosti. Uprkos terapiji bolest se reaktivirala nakon godinu dana terapije zbog čega je primjenjena i sekundarna hemoterapija. Usljed razvijenog diabetes inspidusa uvedena je doživotna supstituciona terapija antiduretskim hormonom.Izolovane kožne lezije koje ne reaguju na lokalnu terapiju i koje imaju tendenciju pogoršanja bi trebale pobuditi sumnju na LCH. U ovim slučajevima dodatna ispitivanja i bipsija kože su mandatorni radi blagovremene potvrde dijagnoze i adekvatnog liječenja. Uprkos terapiji djeca sa razvijenim multisistemskim oblikom bolesti mogu imati neadekvatan odgovor na terapiju.UDK 616.289-07

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Nonhealing Crusted Scalp Lesions in a 4-Year-Old Boy

2018

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Diverse Cutaneous Presentations of Langerhans Cell Histiocytosis in Children: A Retrospective Cohort Study

Abstract: Late onset and a protracted course of skin lesions are associated with MS-LCH, whereas WT BRAF is found in rapidly resolving skin lesions.

Cited by 77 publications

References 26 publications

Histiocytosis – cutaneous manifestations of hematopoietic neoplasm and non‐neoplastic histiocytic proliferations

Histiocytosis – cutaneous manifestations of hematopoietic neoplasm and non‐neoplastic histiocytic proliferations

Histiocitoza Langerhansovih Ćelija - Od Jednosistemskog Do Multisistemskog Oblika

Nonhealing Crusted Scalp Lesions in a 4-Year-Old Boy

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