acantholysis

{{distinguish|Acanthosis}}

{{Infobox medical condition

| name = Acantholysis

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| image = Diseases of Swine 25-5.png

| caption = Foot-and-mouth disease - acantholysis in a sample of a skin vesicle: Necrosis of the stratum spinosum can be observed, and keratinocytes floating in the vesicular fluid (spongiosa).

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| field = Dermatology

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Acantholysis is the loss of intercellular connections, such as desmosomes, resulting in loss of cohesion between keratinocytes,Kumar, Vinay; Fausto, Nelso; Abbas, Abul (2004) Robbins & Cotran Pathologic Basis of Disease (7th ed.). Saunders. Page 1230. {{ISBN|0-7216-0187-1}}. seen in diseases such as pemphigus vulgaris, Grover’s disease, and Hailey-Hailey Disease.{{cite journal |last1=Kumaran |first1=MSendhil |last2=Kanwar |first2=AmrinderJ |last3=Seshadri |first3=Divya |title=Acantholysis revisited: Back to basics |journal=Indian Journal of Dermatology, Venereology and Leprology |date=2013 |volume=79 |issue=1 |pages=120–126 |doi=10.4103/0378-6323.104688|doi-access=free |pmid=23254748 }}{{cite web|url=http://www.medilexicon.com/medicaldictionary.php?t=375|publisher=medilexicon.com|title=Acantholysis - Medical Definition from MediLexicon|accessdate=2017-04-22|archive-url=https://web.archive.org/web/20160731060135/http://www.medilexicon.com/medicaldictionary.php?t=375|archive-date=2016-07-31|url-status=dead}} It is absent in bullous pemphigoid, making it useful for differential diagnosis. This disruption between cells causes intra-epidermal clefts, vesicles and bullae due to cells becoming rounded and no longer attached to one another.{{cite journal |last1=Kumaran |first1=MSendhil |last2=Kanwar |first2=AmrinderJ |last3=Seshadri |first3=Divya |title=Acantholysis revisited: Back to basics |journal=Indian Journal of Dermatology, Venereology and Leprology |date=2013 |volume=79 |issue=1 |pages=120–126 |doi=10.4103/0378-6323.104688|doi-access=free |pmid=23254748 }}

Focusing on Pemphigus vulgaris, a blistering auto-immune disease, during acantholysis, circulating autoantibodies cause disruption of cell-cell and cell-matrix adhesion.{{cite journal |last1=Mohammad Beigi |first1=Pooya Khan |title=Overview of Pemphigus Vulgaris |journal=A Clinician's Guide to Pemphigus Vulgaris |date=2018 |pages=11–12 |doi=10.1007/978-3-319-67759-0_2|isbn=978-3-319-67758-3 }} The antibodies circulate against intercellular adhesion structures and demosomal protein desmoglein (DSG), which causes the disruption.{{cite journal |last1=Mohammad Beigi |first1=Pooya Khan |title=Overview of Pemphigus Vulgaris |journal=A Clinician's Guide to Pemphigus Vulgaris |date=2018 |page=11 |doi=10.1007/978-3-319-67759-0_2|isbn=978-3-319-67758-3 }} Acantholytic cells also known as Tzanck cells are a distinguishing feature when diagnosing Pemphigus vulgaris.{{cite journal |last1=Singhi |first1=Mk |last2=Gupta |first2=LalitKumar |title=Tzanck smear: A useful diagnostic tool |journal=Indian Journal of Dermatology, Venereology and Leprology |date=2005 |volume=71 |issue=4 |pages=295 |doi=10.4103/0378-6323.16632|doi-access=free |pmid=16394449 }} The Tzanck test can be used to diagnosis Pemphigus vulgaris for patients who are uncomfortable with a biopsy.{{cite journal |last1=Singhi |first1=Mk |last2=Gupta |first2=LalitKumar |title=Tzanck smear: A useful diagnostic tool |journal=Indian Journal of Dermatology, Venereology and Leprology |date=2005 |volume=71 |issue=4 |pages=295 |doi=10.4103/0378-6323.16632|doi-access=free |pmid=16394449 }} he test can be used to identify acantholytic cells which are classified as large round keratinocytes characterized by an enlarged nucleus, indistinct or missing nucleoli and plentiful basophilic cytoplasm.{{cite journal |last1=Singhi |first1=Mk |last2=Gupta |first2=LalitKumar |title=Tzanck smear: A useful diagnostic tool |journal=Indian Journal of Dermatology, Venereology and Leprology |date=2005 |volume=71 |issue=4 |pages=295 |doi=10.4103/0378-6323.16632|doi-access=free |pmid=16394449 }} This histological feature is also seen in herpes simplex infections (HSV 1 and 2) and varicella zoster infections (chicken pox and shingles).

== Epidemiology ==

The incidence of acantholysis varies according to the condition it is associated with.

In Pemphigus vulgaris, the incidence ranges from 0.076 to 5 per 100,000 person-years.{{cite journal |last1=Gupta |first1=Vibha K. |last2=Kelbel |first2=Theodore E. |last3=Nguyen |first3=Daniela |last4=Melonakos |first4=Katherine C. |last5=Murrell |first5=Dédée F. |last6=Xie |first6=Yan |last7=Mullard |first7=Andrew |last8=Reed |first8=Philip L. |last9=Seiffert-Sinha |first9=Kristina |last10=Sinha |first10=Animesh A. |title=A Globally Available Internet-Based Patient Survey of Pemphigus Vulgaris: Epidemiology and Disease Characteristics |journal=Dermatologic Clinics |date=1 July 2011 |volume=29 |issue=3 |pages=393–404 |doi=10.1016/j.det.2011.03.016|pmid=21605804 }} The occurrence of Pemphigus vulgaris is most common in adults between ages of 50 and 60 years old.{{cite journal |last1=Mohammad Beigi |first1=Pooya Khan |title=Epidemiology and Etiology |journal=A Clinician's Guide to Pemphigus Vulgaris |date=2018 |pages=15–16 |doi=10.1007/978-3-319-67759-0_4|isbn=978-3-319-67758-3 }} Approximately 70% of cases of this condition come from India, China, Malaysia and the Middle East. While Pemphigus vulgaris affects all, the highest incidence occurs in Ashkenazi Jews.{{cite journal |last1=Mohammad Beigi |first1=Pooya Khan |title=Epidemiology and Etiology |journal=A Clinician's Guide to Pemphigus Vulgaris |date=2018 |pages=15–16 |doi=10.1007/978-3-319-67759-0_4|isbn=978-3-319-67758-3 }} Some common factors that cause this condition are stress, medication, surgical and dental procedures, physical trauma or other illnesses.{{cite journal |last1=Kumaran |first1=MSendhil |last2=Kanwar |first2=AmrinderJ |last3=Seshadri |first3=Divya |title=Acantholysis revisited: Back to basics |journal=Indian Journal of Dermatology, Venereology and Leprology |date=2013 |volume=79 |issue=1 |pages=120–126 |doi=10.4103/0378-6323.104688|doi-access=free |pmid=23254748 }} Genetics is also a strong factor in inheriting Pemphigus vulgaris. This has been showed in Ashkenazi Jews with Pemphigus vulgaris.{{cite journal |last1=Mohammad Beigi |first1=Pooya Khan |title=Epidemiology and Etiology |journal=A Clinician's Guide to Pemphigus Vulgaris |date=2018 |pages=15–16 |doi=10.1007/978-3-319-67759-0_4|isbn=978-3-319-67758-3 }}

Grover’s disease, which is also caused by acantholysis, is most common in older adults with older adults likely to have more extensive and longer duration of the disease.{{cite journal |last1=Parsons |first1=Jerome Michael |title=Transient acantholytic dermatosis (Grover's disease): A global perspective |journal=Journal of the American Academy of Dermatology |date=1 November 1996 |volume=35 |issue=5 |pages=653–666 |doi=10.1016/S0190-9622(96)90715-X|pmid=8912557 }} The disease is around 1.6 to 2.1 times more common in males than females.{{cite journal |last1=Parsons |first1=Jerome Michael |title=Transient acantholytic dermatosis (Grover's disease): A global perspective |journal=Journal of the American Academy of Dermatology |date=1 November 1996 |volume=35 |issue=5 |pages=653–666 |doi=10.1016/S0190-9622(96)90715-X|pmid=8912557 }} Some factors that are associated with causing Grover’s disease are environmental factors like extensive sunlight exposure or heat and preexisting illnesses such as cancer and infections.{{cite journal |last1=Parsons |first1=Jerome Michael |title=Transient acantholytic dermatosis (Grover's disease): A global perspective |journal=Journal of the American Academy of Dermatology |date=1 November 1996 |volume=35 |issue=5 |pages=653–666 |doi=10.1016/S0190-9622(96)90715-X|pmid=8912557 }}