**6. Enzyme-Linked Immunosorbent Assay (ELISA)**

Indirect immunofluorescence is not reactive with all pemphigus sera and does not differentiate between desmoglein-3 and desmoglein-1[41]. In response, ELISAs have been developed using the recombinant ectodomains of desmoglein-1 and -3 [42]. In commercially available assays,

review [50] assessed interventions for PV and PF and concluded that there is inadequate information available to ascertain optimal therapy for pemphigus. They ascertained that the quality of most studies was not high and the majority examine patients with newly diagnosed or active disease. Another consideration in the evaluation of data is lack of generally accepted definitions and measurements for the clinical evaluation of patients with pemphigus and the definitions of disease control and remission. A recent consensus statement has been released

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As high-dose systemic corticosteroids, followed by alternate immune-suppressive agents, serves as the mainstay of initial therapy for PV, there is the need to exclude underlying latent infectious diseases that can be reactivated by the corticosteroids (e.g.: HIV, Hepatitis B and C, and tuberculosis). In addition, screening for the diseases initiated or exacerbated by high-dose

Systemic corticosteroids are currently the mainstay of treatment as they have a rapid onset of action and are effective in controlling disease and improving prognosis [52]. However significant side effects such as diabetes, osteoporosis, adrenal suppression, peptic ulceration, weight gain, increased susceptibility to infection, mood changes, proximal myopathy, Cushing's syndrome, and cataracts limit their usefulness. Adjuvant treatments have therefore been introduced as steroid sparing agents. Various corticosteroid regimens are used to treat pemphigus, the most common of which is a gradual reduction of an oral formulation [47]. In newly diagnosed patients, an initial daily dose of 0.5 mg/kg of prednisone/prednisolone, (or equivalent) appears preferable to 1 mg/kg. A randomized trial compared these two regimens in 19 patients with PV and 3 with PF followed for 5 years, with remission defined as less than 15 mg of corticosteroids per day to maintain disease control [53]. No difference was observed in remission or the incidence of complications between the high and low dose regimens. In a randomized trial that included only patients with newly diagnosed PV, pulsed oral dexame‐ thasone provided no additional benefit to the combination of oral prednisone and azathioprine with remission defined as cessation of systemic treatment [54]. Furthermore, there were an increased number of adverse events in those participants receiving pulsed dexamethasone. However, the possible benefit of high dose pulsed intravenous corticosteroids in achieving disease control and maintaining remission was suggested in a small case-controlled retro‐ spective study of patients with PV initially unresponsive to low dose of prednisone (less than 40 mg daily) [55] and an open study of new diagnosed PV patients [56]. There have no studies

to date examining the effects of high dose pulsed intravenous corticosteroids in PF.

trant disease but this remains to be substantiated in randomized studies.

Currently no optimal regimen for corticosteroid therapy has been defined for the treatment of pemphigus despite its proven benefits. Hence in routine practice, a tailored regime is recom‐ mended. A starting dose of prednisolone 0.5 mg/kg daily is prudent that may need to be increased, until no new blister formation is observed. Such higher doses of corticosteroids including pulsed therapy may be warranted in newly diagnosed severe disease and recalci‐

to assist with future trials enabling improved comparisons to be made [51].

'steroids, such as hypertension, diabetes mellitus and osteoporosis is prudent.

**8. Systemic corticosteroids**

**Figure 10.** Indirect immunofluorescence with the serum of a patient with pemphigus foliaceus and monkey oesopha‐ gus substrate reveals deposition of specific IgG in the intercellular cement space substance of the epidermis resulting in a chicken wire appearance. There is an increased deposition in the upper layers of the epidermis.

these desmoglein ectodomains have been expressed in insect cells (MBL, Nagoya, Japan), or in human HEK293 cells (Euroimmun, Lubeck, Germany) [43]. Meta-analyses suggest that available ELISAs are highly sensitive and specific, and has a higher diagnostic accuracy than indirect immunofluorescence [44]. The quantification of these autoantibodies is useful in monitoring disease activity because the titre of circulating autoantibodies has been observed to correlate with disease activity. Specifically, the level of desmoglein-1 antibodies matches the severity of skin disease, and the level of desmoglein-3 reflects the severity of mucosal disease [45],[46].
