Indeterminate cell histiocytosis

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Indeterminate cell histiocytosis
Other namesIndeterminate dendritic cell tumor
Specialty Dermatology

Indeterminate cell histiocytosis(LCH) is an uncommon proliferative illness where the predominant cells have characteristics from both non-Langerhans cell histiocytosis (NLCH) and Langerhans cell histiocytosis (LCH) in terms of morphology and immunophenotypic characteristics. [1] Wood et al. originally described ICH in 1985 as a neoplastic disease arising from dermal indeterminate cells that lack Birbeck granules but are characteristically positive for S-100 and CD1a. [2]

Contents

Signs and symptoms

Clinically, ICH is defined by pink to reddish, varying-sized, painless, non-itching papules or nodules that develop on otherwise healthy skin (sparing mucosae). These lesions can appear as a single, distinct group of lesions, or several generalized papules dispersed over the trunk, face, and limbs. They can also show signs of stable disease, remission and recurrence, or spontaneous remission. [3]

Causes

Possible causes include scabies, [4] mosquito bites, [5] and a clonal drive. [6]

Treatment

Treatment include 5% 5-fluorouracil cream, topical pure coal tar, electron beam therapy, phototherapy, and total excision. [7] [8]

See also

Related Research Articles

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A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary system—the organ system that encloses the body and includes skin, nails, and related muscle and glands. The major function of this system is as a barrier against the external environment.

<span class="mw-page-title-main">Dermatofibroma</span> Medical condition

A dermatofibroma, or benign fibrous histiocytomas, is a benign nodule in the skin, typically on the legs, elbows or chest of an adult. It is usually painless.

<span class="mw-page-title-main">Langerhans cell</span> Cell type

A Langerhans cell (LC) is a tissue-resident macrophage of the skin once thought to be a resident dendritic cell. These cells contain organelles called Birbeck granules. They are present in all layers of the epidermis and are most prominent in the stratum spinosum. They also occur in the papillary dermis, particularly around blood vessels, as well as in the mucosa of the mouth, foreskin, and vaginal epithelium. They can be found in other tissues, such as lymph nodes, particularly in association with the condition Langerhans cell histiocytosis (LCH).

<span class="mw-page-title-main">Langerhans cell histiocytosis</span> Medical condition

Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes.

<span class="mw-page-title-main">Letterer–Siwe disease</span> Medical condition

Letterer–Siwe disease, (LSD) or Abt-Letterer-Siwe disease, is one of the four recognized clinical syndromes of Langerhans cell histiocytosis (LCH) and is the most severe form, involving multiple organ systems such as the skin, bone marrow, spleen, liver, and lung. Oral cavity and gastrointestinal involvement may also be seen. LCH and all its subtypes are characterized by monoclonal migration and proliferation of specific dendritic cells.

<span class="mw-page-title-main">Granuloma annulare</span> Medical condition

Granuloma annulare (GA) is a common, sometimes chronic skin condition which presents as reddish bumps on the skin arranged in a circle or ring. It can initially occur at any age, though two-thirds of patients are under 30 years old, and it is seen most often in children and young adults. Females are two times as likely to have it as males.

<span class="mw-page-title-main">Chronic multifocal Langerhans cell histiocytosis</span> Medical condition

Chronic multifocal Langerhans cell histiocytosis, previously known as Hand–Schüller–Christian disease, is a type of Langerhans cell histiocytosis (LCH), which can affect multiple organs. The condition is traditionally associated with a combination of three features; bulging eyes, breakdown of bone, and diabetes insipidus, although around 75% of cases do not have all three features. Other features may include a fever and weight loss, and depending on the organs involved there may be rashes, asymmetry of the face, ear infections, signs in the mouth and the appearance of advanced gum disease. Features relating to lung and liver disease may occur.

<span class="mw-page-title-main">Angiofibroma</span> Medical condition

Angiofibroma (AGF) is a descriptive term for a wide range of benign skin or mucous membrane lesions in which individuals have:

  1. benign papules, i.e. pinhead-sized elevations that lack visible evidence of containing fluid;
  2. nodules, i.e. small firm lumps usually >0.1 cm in diameter; and/or
  3. tumors, i.e. masses often regarded as ~0.8 cm or larger.

Actinic prurigo is a rare sunlight-induced, pruritic, papular, or nodular skin eruption. Some medical experts use the term actinic prurigo to denote a rare photodermatosis that develops in childhood and is chronic and persistent; this rare photodermatosis, associated with the human leukocyte antigen HLA-DR4, is often called "Familial polymorphous light eruption of American Indians" or "Hereditary polymorphous light eruption of American Indians" but some experts consider it to be a variant of the syndrome known as polymorphous light eruption (PMLE). Some experts use the term actinic prurigo for Hutchinson's summer prurigo and several other photodermatoses that might, or might not, be distinct clinical entities.

<span class="mw-page-title-main">Acrokeratoelastoidosis of Costa</span> Medical condition

Acrokeratoelastoidosis of Costa or Acrokeratoelastoidosis is a hereditary form of marginal keratoderma, and can be defined as a palmoplantar keratoderma. It is distinguished by tiny, firm pearly or warty papules on the sides of the hands and, occasionally, the feet. It is less common than the hereditary type of marginal keratoderma, keratoelastoidosis marginalis.

<i>Leishmania tropica</i> Species of protozoan parasite

Leishmania tropica is a flagellate parasite and the cause of anthroponotic cutaneous leishmaniasis in humans. This parasite is restricted to Afro-Eurasia and is a common cause of infection in Afghanistan, Iran, Syria, Yemen, Algeria, Morocco, and northern India.

<span class="mw-page-title-main">Blueberry muffin baby</span> Medical condition

Blueberry muffin baby, also known as extramedullary hematopoiesis, describes a newborn baby with multiple purpura, associated with several non-cancerous and cancerous conditions in which extra blood is produced in the skin. The bumps range from one to seven mm, do not blanch and have a tendency to occur on the head, neck and trunk. They often fade by three to six weeks after birth, leaving brownish marks. When due to a cancer, the bumps tend to be fewer, firmer and larger.

<span class="mw-page-title-main">Langerhans cell sarcoma</span> Medical condition

Langerhans cell sarcoma (LCS) is a rare form of malignant histiocytosis. It should not be confused with Langerhans cell histiocytosis, which is cytologically benign. It can present most commonly in the skin and lymphatic tissue, but may also present in the lung, liver, and bone marrow. Treatment is most commonly with surgery or chemotherapy.

Acral persistent papular mucinosis (APPM) is a rare form of lichen myxedematosus. It is characterized by small papules on the backs of the hands, wrists, and extensor aspects of the distal forearms, with no further clinical or laboratory indications. Lesions tend to persist and may grow in number gradually. Because there are no symptoms, treatment is rarely required.

Benign cephalic histiocytosis(BCH) is a non-Langerhan's histiocytosis that is uncommon and self-limiting, usually beginning towards the end of the first year of life. Gianotti et al. originally described it in 1971. Initially affecting the head and neck, this condition is characterized by several small eruptions of yellow to reddish-brow papules that heal on their own. Histological investigations have demonstrated that this disorder is associated with dermal proliferation of histiocytes, characterized by intracytoplasmic comma-shaped bodies, covered vesicles, and desmosome-like structure.

Generalized eruptive histiocytoma is a rare cutaneous condition characterized by widespread, erythematous, essentially symmetrical papules, particularly involving the trunk and proximal extremities.

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Histiocytic diseases in dogs are a group of diseases in dogs which may involve the skin, and which can be difficult to differentiate from granulomatous, reactive inflammatory or lymphoproliferative diseases. The clinical presentation and behaviour as well as response to therapy vary greatly among the syndromes.

References

  1. Vener, C.; Soligo, D.; Berti, E.; Gianelli, U.; Servida, F.; Ceretti, E.; Caputo, R.; Passoni, E.; Lambertenghi Deliliers, G. (2007). "Indeterminate cell histiocytosis in association with later occurrence of acute myeloblastic leukaemia". British Journal of Dermatology. Oxford University Press (OUP). 156 (6): 1357–1361. doi:10.1111/j.1365-2133.2007.07880.x. ISSN   0007-0963. PMID   17459045. S2CID   41005413.
  2. WOOD, GARY S.; HU, CHUNG-HONG; BECKSTEAD, JAY H.; TURNER, RODERICK R.; WINKELMANN, R. K. (1985). "The Indeterminate Cell Proliferative Disorder: Report of a Case Manifesting as an Unusual Cutaneous Histiocytosis". The Journal of Dermatologic Surgery and Oncology. Wiley. 11 (11): 1111–1119. doi:10.1111/j.1524-4725.1985.tb01399.x. ISSN   0148-0812. PMID   3902927.
  3. Zerbini, Maria Claudia Nogueira; Sotto, Mirian Nacagami; Campos, Fernando Peixoto Ferraz de; Abdo, Andre Neder Ramires; Pereira, Juliana; Sanches, José Antônio; Martins, Jade Cury (2016). "Indeterminate cell histiocytosis successfully treated with phototherapy". Autopsy and Case Reports. Editora Cubo. 6 (2): 33–38. doi:10.4322/acr.2016.038. ISSN   2236-1960. PMC   4982782 . PMID   27547741.
  4. Hashimoto, Ken; Fujiwara, Keiko; Punwaney, Juanita; DiGregorio, Fiorino; Bostrom, Paul; El-Hoshy, Khaled; Aronson, Peter J.; Schoenfeld, Robert J. (2000). "Post-Scabetic Nodules: A Lymphohistiocytic Reaction Rich in Indeterminate Cells". The Journal of Dermatology. Wiley. 27 (3): 181–194. doi:10.1111/j.1346-8138.2000.tb02148.x. ISSN   0385-2407. PMID   10774146. S2CID   23463988.
  5. Mo, Xianglan; Guo, Wenwen; Ye, Hongtao (2015). "Primary Indeterminate Dendritic Cell Tumor of Skin Correlated to Mosquito Bite". Medicine. Ovid Technologies (Wolters Kluwer Health). 94 (34): e1443. doi:10.1097/md.0000000000001443. ISSN   0025-7974. PMC   4602924 . PMID   26313805.
  6. Brown, Ryanne A.; Kwong, Bernice Y.; McCalmont, Timothy H.; Ragsdale, Bruce; Ma, Lisa; Cheung, Christine; Rieger, Kerri E.; Arber, Daniel A.; Kim, Jinah (November 12, 2015). "ETV3-NCOA2 in indeterminate cell histiocytosis: clonal translocation supports sui generis". Blood. American Society of Hematology. 126 (20): 2344–2345. doi:10.1182/blood-2015-07-655530. ISSN   0006-4971. PMID   26438513.
  7. Miracco, Clelia; Raffaelli, Miranda; de Santi, Maria; Margherita, M.S; Fimiani, Michele; Tosi, Piero (February 1988). "Solitary cutaneous reticulum cell tumor. Enzyme-immunohistochemical and electron-microscopic analogies with IDRC sarcoma". The American Journal of Dermatopathology. 10 (1): 47–53. doi:10.1097/00000372-198802000-00006. PMID   2845833 . Retrieved 24 January 2024.
  8. Berti, Emilio (August 1, 1988). "Unusual Cutaneous Histiocytosis Expressing an Intermediate Immunophenotype Between Langerhans' Cells and Dermal Macrophages". Archives of Dermatology. American Medical Association (AMA). 124 (8): 1250. doi:10.1001/archderm.1988.01670080062020. ISSN   0003-987X. PMID   3401031.

Further reading