(1)
Misdiagnosis Association & Society, Seattle, WA, USA
Keywords
Pemphigus vulgarisCorticosteroidsUrea reductionMycophenolate mofetilCyclophosphamidesRituximabIntravenous immunoglobulin (IVIG)PlasmapheresisIntroduction
Pemphigus is the name of a group of life-threatening blistering diseases of the skin and mucous membranes. The base of treatment for this disease is corticosteroids ; however, recently, new drugs, such as rituximab , have been verified for more severe forms of it.
In the author’s previously unpublished study, the effect of rituximab on variation in the laboratory indices of pemphigus vulgaris patients is addressed. After investigation of the files of pemphigus patients who received rituximab in Razi Hospital, Tehran, Iran, from 2008 to 2013, 39 patients were entered into the study. All patients had lab sheets containing CR (creatinine), urea, ALT (alanine aminotransferase), AST (aspartate aminotransferase), Plt (platelet), Hgb (hemoglobin), and WBC (white blood cell) before and after receiving rituximab. The patients received rituximab four times at a dosage of 500 mg in 4 successive weeks. The lab results before receiving the first dose of rituximab were compared to the results after receiving treatment. The effect of rituximab on the variation in lab indices with the adjustment effect of age, gender, disease duration, sites of involvement, received adjoins, and the background disease was also investigated.
In the initial analysis, rituximab only had a significant effect on urea reduction . In the CellCept® (mycophenolate mofetil ) receiving subgroup, the mixed consumption of rituximab led to a significant reduction in WBC. In the subgroup having background disease, rituximab had a statistically significant impact on platelet reduction. In the subgroup having no background disease, rituximab had a statistically significant effect on urea reduction .
The lab indices were shown to have no significant relationship with age and disease duration. Thus, it can be predicted that disease duration and age would have no effect in the relationship between rituximab and lab indices’ variations.
Although in stratified single-variable analysis for adjusting the effect of other variables (involvement sites and received adjoins) on the relation of rituximab and lab indices, some of these variables showed interacting effects with rituximab on the variations of lab indices. However, due to the low volume of sample and non-normal distribution of most of these variables, it was impossible to do multivariable analysis for investigation of their independent and interactive effects on variations of lab indices in an integrated manner; therefore, we cannot make certain comments about their relationships.
Types of Pemphigus
Pemphigus is the name of a group of life-threatening blistering diseases that have characteristic acantholysis leading to formation of intraepithelial blisters in mucus and skin [1]. The acantholysis process is induced via attachments of flowing autoantibodies to adhesion molecules in the cells [2]. Patients with pemphigus have mucosal erosions, blisters, papules, and cutaneous erosions.
The different types of pemphigus are pemphigus vulgaris, pemphigus foliaceus, immunoglobulin A (IgA) pemphigus, and paraneoplastic pemphigus. Different types of pemphigus are differentiated by clinical symptoms, related autoantigens, and histological methods. Pemphigus vulgaris has mucosal and mucocutaneous involvement. The blisters are acantholytic and suprabasal. The autoantibodies responsible for the disease are against desmoglein (DSG) 1 or both desmoglein 3 and 1.
Pemphigus foliaceus only involves the skin. The blisters are acantholytic and subcorneal. The responsible autoantibodies are against desmoglein 1.
IgA pemphigus has the form of grouped erythematous crusts, papules, and vesicle plucks. Blisters can be subcorneal or intraepithelial and acantholytic. The responsible autoantibodies are against desmocollin (DSC) 1 [3].
Paraneoplastic pemphigus involves vast and resistant stomatite along with different cutaneous findings. The responsible autoantibodies are against desmoplakin (DSP) or other desmosomal antigens.
Pemphigus vulgaris is the most common type of pemphigus, but is still very rare. The chance of its occurrence is between 0.1 and 0.5 per 100,000 people [4].
Pemphigus often occurs among adults and the average age of onset is 40–60 years old. It is very rare among children [5, 6]. Its prevalence is almost the same in the two sexes [7]. Almost all the pemphigus vulgaris patients have mucosal involvement. The mouth is the most common site of involvement and is often the first site of involvement. Other mucosal membranes such as conjunctivae, nose, esophagus, vulva, vagina, cervix, and anus are rarely involved [8]. As mucosal blisters are fragile and burst easily, in clinical examination it is difficult to find intact blisters, and instead the examiner tends to find mucosal erosions. Buccal and palatal mucosa are the most common sites of blister involvement in the mouth cavity [9].