Progressive nodular histiocytosis

Progressive nodular histiocytosis most frequently affects young to middle-aged adults who show up with widely distributed, randomly distributed, reddish-brown, cutaneous papules and nodules that are not painful or pruritic.{{cite journal | last1=Williams | first1=Abhilasha | last2=Thomas | first2=AbrahamG | last3=Kwatra | first3=KanwardeepSingh | last4=Jain | first4=Kunal | title=Progressive nodular histiocytosis associated with eale′s disease | journal=Indian Journal of Dermatology | publisher=Medknow | volume=60 | issue=4 | year=2015 | issn=0019-5154 | doi=10.4103/0019-5154.160492 | doi-access=free | page=388| pmc=4533540 }} Progressive nodular histiocytosis's clinical course is characterized by an unwavering lack of spontaneous remission.{{cite journal | last1=Numbere | first1=Numbereye | last2=Pukhalskaya | first2=Tatsiana | last3=Bowman | first3=Blythe | last4=Campbell | first4=Katelynn | last5=Smoller | first5=Bruce | title=Progressive Nodular Histiocytosis: Report of a Case and Review of the Literature | journal=Case Reports in Pathology | publisher=Hindawi Limited | volume=2021 | date=September 17, 2021 | issn=2090-679X | doi=10.1155/2021/5531820 | doi-access=free | pages=1–6| pmid=34567817 | pmc=8463211 }} Over time, lesions grow larger and more numerous, and they can cause noticeable disfigurement.{{cite journal | last1=Kiyohara | first1=T | last2=Makimura | first2=K | last3=Miyamoto | first3=M | last4=Shijimaya | first4=T | last5=Nagano | first5=N | last6=Nakamaru | first6=S | last7=Tanimura | first7=H | title=Progressive Nodular Histiocytosis with Large Nodules and a Bulky Mass | journal=Acta Dermato Venereologica | publisher=Medical Journals Sweden AB | volume=99 | issue=6 | year=2019 | issn=0001-5555 | doi=10.2340/00015555-3045 | pages=610–611| pmid=30226532 | doi-access=free }} Although mucous involvement is possible,{{cite journal | last1=Glavin | first1=F. L. | last2=Chhatwall | first2=H. | last3=Karimi | first3=K. | title=Progressive nodular histiocytosis: a case report with literature review, and discussion of differential diagnosis and classification | journal=Journal of Cutaneous Pathology | publisher=Wiley | volume=36 | issue=12 | date=October 26, 2009 | issn=0303-6987 | doi=10.1111/j.1600-0560.2009.01454.x | pages=1286–1292| pmid=19878386 | s2cid=22414587 }} internal organs are typically unaffected.{{cite journal | last1=Hilker | first1=Olaf | last2=Kovneristy | first2=Alexander | last3=Varga | first3=Rita | last4=Neubert | first4=Thorsten | last5=Wesselmann | first5=Ulrich | last6=Flaig | first6=Michael J. | last7=Ruzicka | first7=Thomas | last8=Burgdorf | first8=Walter | last9=Lehmann | first9=Percy | title=Progressive nodular histiocytosis | journal=JDDG: Journal der Deutschen Dermatologischen Gesellschaft | publisher=Wiley | volume=11 | issue=4 | date=December 11, 2012 | issn=1610-0379 | doi=10.1111/j.1610-0387.2012.08069.x | pages=301–307| pmid=23231636 }} Mechanical interference caused by lesions in critical locations, such as the eyelids or the soles of the feet, can result in functional impairment.{{cite journal | last1=Salunke | first1=Aarti | last2=Belgaumkar | first2=Vasudha | last3=Chavan | first3=Ravindranath | last4=Dobariya | first4=Rinkesh | title=Laryngeal involvement with fatal outcome in progressive nodular histiocytosis: A rare case report | journal=Indian Dermatology Online Journal | publisher=Medknow | volume=7 | issue=6 | year=2016 | issn=2229-5178 | doi=10.4103/2229-5178.193913 | doi-access=free | page=516| pmid=27990389 | pmc=5134168 }} There is a rare possibility that the cutaneous lesions could cause systemic effects directly; microcytic anemia due to significant intralesional iron sequestration has been documented. Obstructive lesions in the upper airway have been associated with death, despite the fact that they are usually not life-threatening.

Histologically, it is typified by a diffuse infiltrate of Touton giant cells and xanthomatized histiocytes mixed in with spindle-shaped histiocytes with a whorl-like growth pattern.

The primary method of treating progressive nodular histiocytosis is surgical excision. However, a few cases have been reported to have improved following methotrexate administration.{{cite journal | last1=Huet | first1=Flavien | last2=Brenaut | first2=Emilie | last3=Costa | first3=Sebastian | last4=Lemasson | first4=Gilles | last5=Sonbol | first5=Haitham | last6=Misery | first6=Laurent | title=Progressive nodular histiocytosis improved by methotrexate | journal=European Journal of Dermatology | publisher=John Libbey Eurotext | volume=27 | issue=6 | year=2017 | issn=1167-1122 | doi=10.1684/ejd.2017.3115 | pages=661–663| pmid=29165296 | s2cid=21473573 }} Other treatments such as carbon dioxide laser, intralesional as well as systemic steroids, and antineoplastic agents such as imatinib have mostly shown no effect on progressive nodular histiocytosis. Unfortunately, for those who are affected, recurrence is possible even after treatment.

• Non-X histiocytosis

{{reflist}}

• {{cite journal | last=Burgdorf | first=Walter H. C. | title=Progressive Nodular Histiocytoma | journal=Archives of Dermatology | volume=117 | issue=10 | date=1981-10-01 | issn=0003-987X | doi=10.1001/archderm.1981.01650100046027 | page=644 | pmid=6269500 | ref=none}}
• {{cite journal | last1=ZELGER | first1=B.W.H. | last2=STAUDACHER | first2=CH. | last3=ORCHARD | first3=G. | last4=WILSON-JONES | first4=E. | last5=BURGDORF | first5=W.H.C. | title=Solitary and generalized variants of spindle cell xanthogranuloma (progressive nodular histiocytosis) | journal=Histopathology | publisher=Wiley | volume=27 | issue=1 | year=1995 | issn=0309-0167 | doi=10.1111/j.1365-2559.1995.tb00285.x | pages=11–19 | pmid=7557901 | s2cid=25453049 | ref=none}}

• [https://www.visualdx.com/visualdx/diagnosis/progressive+nodular+histiocytoma?diagnosisId=52724&moduleId=101 VisualDx]

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{{Histiocytosis}}

Category:Monocyte- and macrophage-related cutaneous conditions

Category:Histiocytosis