Acantholysis
| Acantholysis | |
|---|---|
| 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). | |
| Specialty | Dermatology |
Acantholysis is the loss of intercellular connections, such as desmosomes, resulting in loss of cohesion between keratinocytes,[1] seen in diseases such as pemphigus vulgaris, Grover’s disease, and Hailey-Hailey Disease.[2][3] 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.[4]
Focusing on Pemphigus vulgaris, a blistering auto-immune disease, during acantholysis, circulating autoantibodies cause disruption of cell-cell and cell-matrix adhesion.[5] The antibodies circulate against intercellular adhesion structures and demosomal protein desmoglein (DSG), which causes the disruption.[6] Acantholytic cells also known as Tzanck cells are a distinguishing feature when diagnosing Pemphigus vulgaris.[7] The Tzanck test can be used to diagnosis Pemphigus vulgaris for patients who are uncomfortable with a biopsy.[8] 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.[9] This histological feature is also seen in herpes simplex infections (HSV 1 and 2) and varicella zoster infections (chicken pox and shingles).
Epidemiology
[edit | edit source]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.[10] The occurrence of Pemphigus vulgaris is most common in adults between ages of 50 and 60 years old.[11] 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.[12] Some common factors that cause this condition are stress, medication, surgical and dental procedures, physical trauma or other illnesses.[13] Genetics is also a strong factor in inheriting Pemphigus vulgaris. This has been showed in Ashkenazi Jews with Pemphigus vulgaris.[14]
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.[15] The disease is around 1.6 to 2.1 times more common in males than females.[16] 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.[17]
See also
[edit | edit source]References
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