Progressive nodular histiocytosis

Medical condition

Progressive nodular histiocytosis
Specialty	Dermatology

Progressive nodular histiocytosis is a cutaneous condition clinically characterized by the development of two types of skin lesions: superficial papules and deeper larger subcutaneous nodules. Progressive nodular histiocytosis was first reported in 1978 by Taunton et al. It is a subclass of non-Langerhans cell histiocytosis and a subgroup of xanthogranuloma.

Signs and symptoms

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. Progressive nodular histiocytosis's clinical course is characterized by an unwavering lack of spontaneous remission. Over time, lesions grow larger and more numerous, and they can cause noticeable disfigurement. Although mucous involvement is possible, internal organs are typically unaffected. Mechanical interference caused by lesions in critical locations, such as the eyelids or the soles of the feet, can result in functional impairment. 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.

Diagnosis

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.

Treatment

The primary method of treating progressive nodular histiocytosis is surgical excision. However, a few cases have been reported to have improved following methotrexate administration. 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.

References

James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 978-0-7216-2921-6.
Taunton, O. David (1978-10-01). "Progressive Nodular Histiocytoma". Archives of Dermatology. 114 (10): 1505. doi:10.1001/archderm.1978.01640220054014. ISSN 0003-987X. PMID 214042.
^ Chuang, Fu-Chen; Chern, Erick; Wu, Wei-Ming (2011). "Progressive nodular histiocytosis: a rare type of xanthogranuloma". Dermatologica Sinica. 29 (3). Medknow: 98–100. doi:10.1016/j.dsi.2011.07.005. ISSN 1027-8117.
^ Williams, Abhilasha; Thomas, AbrahamG; Kwatra, KanwardeepSingh; Jain, Kunal (2015). "Progressive nodular histiocytosis associated with eale′s disease". Indian Journal of Dermatology. 60 (4). Medknow: 388. doi:10.4103/0019-5154.160492. ISSN 0019-5154. PMC 4533540.
Numbere, Numbereye; Pukhalskaya, Tatsiana; Bowman, Blythe; Campbell, Katelynn; Smoller, Bruce (September 17, 2021). "Progressive Nodular Histiocytosis: Report of a Case and Review of the Literature". Case Reports in Pathology. 2021. Hindawi Limited: 1–6. doi:10.1155/2021/5531820. ISSN 2090-679X. PMC 8463211. PMID 34567817.
Kiyohara, T; Makimura, K; Miyamoto, M; Shijimaya, T; Nagano, N; Nakamaru, S; Tanimura, H (2019). "Progressive Nodular Histiocytosis with Large Nodules and a Bulky Mass". Acta Dermato Venereologica. 99 (6). Medical Journals Sweden AB: 610–611. doi:10.2340/00015555-3045. ISSN 0001-5555. PMID 30226532.
^ Glavin, F. L.; Chhatwall, H.; Karimi, K. (October 26, 2009). "Progressive nodular histiocytosis: a case report with literature review, and discussion of differential diagnosis and classification". Journal of Cutaneous Pathology. 36 (12). Wiley: 1286–1292. doi:10.1111/j.1600-0560.2009.01454.x. ISSN 0303-6987. PMID 19878386. S2CID 22414587.
^ Hilker, Olaf; Kovneristy, Alexander; Varga, Rita; Neubert, Thorsten; Wesselmann, Ulrich; Flaig, Michael J.; Ruzicka, Thomas; Burgdorf, Walter; Lehmann, Percy (December 11, 2012). "Progressive nodular histiocytosis". JDDG: Journal der Deutschen Dermatologischen Gesellschaft. 11 (4). Wiley: 301–307. doi:10.1111/j.1610-0387.2012.08069.x. ISSN 1610-0379. PMID 23231636.
^ Salunke, Aarti; Belgaumkar, Vasudha; Chavan, Ravindranath; Dobariya, Rinkesh (2016). "Laryngeal involvement with fatal outcome in progressive nodular histiocytosis: A rare case report". Indian Dermatology Online Journal. 7 (6). Medknow: 516. doi:10.4103/2229-5178.193913. ISSN 2229-5178. PMC 5134168. PMID 27990389.
Huet, Flavien; Brenaut, Emilie; Costa, Sebastian; Lemasson, Gilles; Sonbol, Haitham; Misery, Laurent (2017). "Progressive nodular histiocytosis improved by methotrexate". European Journal of Dermatology. 27 (6). John Libbey Eurotext: 661–663. doi:10.1684/ejd.2017.3115. ISSN 1167-1122. PMID 29165296. S2CID 21473573.

External links

VisualDx

Classification	D ICD-11: EE81 SNOMED CT: 765141005
External resources	Orphanet: 158022 Scholia: Q7248852

v t e Histiocytosis
WHO-I/Langerhans cell histiocytosis/ X-type histiocytosis	Letterer–Siwe disease Hand–Schüller–Christian disease Eosinophilic granuloma Congenital self-healing reticulohistiocytosis
WHO-II/non-Langerhans cell histiocytosis/ Non-X histiocytosis	Juvenile xanthogranuloma Hemophagocytic lymphohistiocytosis Erdheim-Chester disease Niemann–Pick disease Sea-blue histiocytosis Benign cephalic histiocytosis Generalized eruptive histiocytoma Xanthoma disseminatum Progressive nodular histiocytosis Papular xanthoma Hereditary progressive mucinous histiocytosis Reticulohistiocytosis (Multicentric reticulohistiocytosis, Reticulohistiocytoma) Indeterminate cell histiocytosis
WHO-III/malignant histiocytosis	Histiocytic sarcoma Langerhans cell sarcoma Interdigitating dendritic cell sarcoma Follicular dendritic cell sarcoma
Ungrouped	Rosai–Dorfman disease

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