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*[[Breast pathology]].
*[[Papillary hidradenoma]].
*[[Intraductal papilloma]].
==References==
{{Reflist|2}}
[[Category:Breast pathology]]
[[Category:Diagnosis]]
487e03b95c7d65a56341a9f7abbda89060770116bb274aac97560ff410b082bd librepath-intraductal-papilloma-breast.txt
[[Image:Langerhans cell histiocytosis - very high mag.jpg|thumb|right|Langerhans cell histiocytosis. [[H&E stain]]. (WC/Nephron)]]
'''Langerhans cell histiocytosis''', abbreviated '''LCH''', is a rare disorder of tissue macrophages. It broadly fits into the category of ''[[histiocytoses]]''. It used to known as ''eosinophilic granuloma''.
It has been referred to by several eponyms - '''Hand-Schüller-Christian disease''', '''Abt-Letterer-Siwe disease''' or '''Letterer-Siwe disease''', and '''histiocytosis X'''.
This article deals with LCH in general. A separate article exists for ''[[pulmonary Langerhans cell histiocytosis]]''.
==General==
===Overview===
LCH is really four (or three) diseases (depending on how one classifies it) - that happen to share the same histology:<ref name=Ref_PCPBoD8_338-9>{{Ref PCPBoD8|338-9}}</ref><ref name=pmid16295427>{{Cite journal | last1 = Chhabra | first1 = UD. | last2 = Desai | first2 = SS. | last3 = Jambhekar | first3 = NA. | title =...
{| class="wikitable sortable"
! Disease
! Other name(s)
! Prognosis
! Demographic
! Location
! Risks/cause
|-
| [[Pulmonary Langerhans cell histiocytosis]]
| Eosinophilic granuloma
| good with smoking cessation
| adults - smokers
| lung only; typically upper lung field
| due to smoking
|-
| Multifocal multisystem Langerhans cell histiocytosis
| multisystem LCH, Letterer-Siwe disease
| outcome dependent on organ involved,<ref name=pmid21351807>{{Cite journal | last1 = Minkov | first1 = M. | title = Multisystem Langerhans cell histiocytosis in children: current treatment and future directions. | journal = Paediatr Drugs | volume = 13 | issue = 2 | pages = 75-86 | month = Apr | year = 2011 | doi = 1...
| usu. children < 2 years old, rarely adults<ref name=pmid22470214>{{Cite journal | last1 = Garg | first1 = A. | last2 = Kumar | first2 = P. | title = Multisystem Langerhans cell histiocytosis in adult. | journal = Indian J Dermatol | volume = 57 | issue = 1 | pages = 58-60 | month = Jan | year = 2012 | doi = 10.4103/...
| multiple systems (skin, spleen, liver, lung, bone marrow)
| possibly genetic ‡
|-
| Unifocal Langerhans cell histiocytosis †
| Eosinophilic granuloma
| may spontaneously regress, may cure with surgery
| children (?)
| bone only
| possibly genetic ‡
|-
| Multifocal unisystem Langerhans cell histiocytosis †
| multifocal LCH, eosinophilic granuloma, Hand-Schuller-Christian syndrome = bone defect, diabetes insipidus & exopthalmos
| may spontaneously regress, may cure with surgery (?)
| children (?)
| usu. bone; may be in: skin, lungs, stomach
| possibly genetic ‡
|}
Note:
* † Robbins lumps these groups together.
* ‡ Incompletely understood. Somatic BRAF mutations identified in approximately half of the individuals.<ref name=pmid20519626>{{Cite journal | last1 = Badalian-Very | first1 = G. | last2 = Vergilio | first2 = JA. | last3 = Degar | first3 = BA. | last4 = MacConaill | first4 = LE. | last5 = Brandner | first5 = B. | las...
===Clinical presentation===
Features - dependent on subtype:<ref name=Ref_PCPBoD8_338-9>{{Ref PCPBoD8|338-9}}</ref>
*May present with fever, anemia, bone pain, bone fracture, diabetes insipidus, exophthalmos.
*Can be an incidental finding.
==Microscopic==
Features:<ref name=pmid16295427/>
*Langerhans cells histiocytes - '''key feature'''.
**Clusters of cells (histiocytes) with a reniform (kidney-shaped) nucleus and abundant foamy cytoplasm.
***Nucleus may look like a "[[coffee bean nucleus|coffee bean]]", i.e. have nuclear grooves (similar to those in [[papillary thyroid carcinoma]]) -- appearance dependent on the rotation of the nucleus.<ref>BN. 15 March 2011.</ref> May be called "buttock cells".
***Chromatin pattern: fine granular, light gray.
*+/-Eosinophils - often prominent.
*+/-Fibrosis - common.
*+/-Other inflammatory cells - neutrophils, plasma cells (uncommon).
*+/-Multinucleated giant cells - uncommon.
DDx:
*[[Kimura disease]] - eosinophilia.
*See ''[[lymph node pathology]]''.
*See ''[[Long_power_list#Many_eosinophils|lesions with many eosinophils]]''.
===Images===
<gallery>
Image:Langerhans cell histiocytosis - very low mag.jpg| LCH - very low mag. (WC/Nephron)
Image:Langerhans cell histiocytosis - low mag.jpg| LCH - low mag. (WC/Nephron)
Image:Langerhans cell histiocytosis - intermed mag.jpg| LCH - intermed. mag. (WC/Nephron)
Image:Langerhans cell histiocytosis - very high mag.jpg| LCH - very high mag. (WC/Nephron)
</gallery>
====www====
*[http://path.upmc.edu/cases/case533.html LCH - several images (upmc.edu)].
==IHC==
*CD1a +ve.
*S100 +ve.
*CD207 ([[AKA]] Langerin) +ve.<ref name=omim604862>{{OMIM|604862}}</ref>
==Molecular==
*Commonly have BRAF mutations ~ 40-70% of cases.<ref name=pmid26980021>{{Cite journal | last1 = Alayed | first1 = K. | last2 = Medeiros | first2 = LJ. | last3 = Patel | first3 = KP. | last4 = Zuo | first4 = Z. | last5 = Li | first5 = S. | last6 = Verma | first6 = S. | last7 = Galbincea | first7 = J. | last8 = Cason | ...
**The ''[[BRAF V600E mutation|V600E mutation]]'' is the most common BRAF mutation.<ref name=pmid27094161>{{Cite journal | last1 = Tatsuno | first1 = M. | last2 = Shioda | first2 = Y. | last3 = Iwafuchi | first3 = H. | last4 = Yamazaki | first4 = S. | last5 = Iijima | first5 = K. | last6 = Takahashi | first6 = C. | las...