Is transition between subtypes of pemphigus possible? A series of pemphigus vulgaris patients showing the transition to pemphigus foliaceus.

BACKGROUND: Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are subtypes of pemphigus with distinct clinical and laboratory features. The transition between these two subtypes has rarely been reported previously. METHODS: The data of PV patients who exhibited clinical and immunoserological transition to PF during the follow-up period were retrospectively evaluated regarding their demographical, clinical, and laboratory characteristics. RESULTS: Among 453 patients diagnosed with PV, 13 (2.9%) patients exhibited clinical and immunoserological transition from PV to PF. The mean age of PV patients at the time of diagnosis was 39.8 ± 14.7 (19‒62) years and 7 (53.8%) of them were female. These patients showed clinical and immunoserological transition from PV to PF after a period ranging from 4 months to 13 years (mean 36.2 ± 41 months). In addition to typical clinical features of PF, all patients had positive anti-desmoglein-1 and negative anti-desmoglein-3 antibody levels after the clinical transition had occurred without any mucosal involvement. During a mean 7.8 ± 5.8 (2‒21) years of follow-up period after the transition from PV to PF, only one female patient had experienced a re-transition to PV characterized by a relapse of disease involving mucosal surfaces with positive anti-desmoglein-3 antibody levels following a 5-year period of remission period without treatment. STUDY LIMITATIONS: Single-center study with a retrospective study design. CONCLUSION: Our series is the largest group of patients reported to show the transition from PV to PF to date with a long follow-up period. The reason behind the disappearance of anti-desmoglein-3 antibodies and the pathogenesis of this phenomenon is not yet elucidated.

Is transition between subtypes of pemphigus possible? A series of pemphigus vulgaris patients showing the transition to pemphigus foliaceus

Abstract Background Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are subtypes of pemphigus with distinct clinical and laboratory features. The transition between these two subtypes has rarely been reported previously. Methods The data of PV patients who exhibited clinical and immunoserological transition to PF during the follow-up period were retrospectively evaluated regarding their demographical, clinical, and laboratory characteristics. Results Among 453 patients diagnosed with PV, 13 (2.9%) patients exhibited clinical and immunoserological transition from PV to PF. The mean age of PV patients at the time of diagnosis was 39.8 ± 14.7 (19‒62) years and 7 (53.8%) of them were female. These patients showed clinical and immunoserological transition from PV to PF after a period ranging from 4 months to 13 years (mean 36.2 ± 41 months). In addition to typical clinical features of PF, all patients had positive anti-desmoglein-1 and negative anti-desmoglein-3 antibody levels after the clinical transition had occurred without any mucosal involvement. During a mean 7.8 ± 5.8 (2‒21) years of follow-up period after the transition from PV to PF, only one female patient had experienced a re-transition to PV characterized by a relapse of disease involving mucosal surfaces with positive anti-desmoglein-3 antibody levels following a 5-year period of remission period without treatment. Study limitations Single-center study with a retrospective study design. Conclusion Our series is the largest group of patients reported to show the transition from PV to PF to date with a long follow-up period. The reason behind the disappearance of anti-desmoglein-3 antibodies and the pathogenesis of this phenomenon is not yet elucidated.

Comorbidities of bullous pemphigoid: A single-center retrospective case-control study from Turkey.

Although significant associations between bullous pemphigoid (BP) and certain comorbidities, primarily subtypes of neurological disorders, have been reported in several populations, it has yet to be demonstrated whether a correlation exists between pre-existing comorbidities and serum titers of anti-BP180 and 230 immunoglobulin G (IgG) antibodies among BP patients. The aim of the current study is to investigate the demographic and clinical features of BP patients in a large series from Turkey, determine the prevalence of pre-existing neurological and systemic disorders, and assess the correlation between the existence of certain comorbidities and basal serum titers of anti-BP180 and 230 IgG autoantibodies. Thus, data from 145 BP patients diagnosed in the study's center between 1987 and 2017 were retrospectively analyzed and compared with 310 age- and sex-matched control subjects. The serum titers of anti-BP 180 and 230 IgG autoantibodies were compared between the patients with and without comorbidities and its subtypes among 55 patients with available serum basal anti-BP levels. Twenty-eight of the BP patients (19.3%) had already been diagnosed with at least one neurological disorder at the onset of BP. According to regression analysis, preexisting neurological disorders (p = 0.017), stroke (p = 0.017), and malignancies (p = 0.005) were found to be higher among the study's BP patients than the controls. The serum titers of anti-BP180 and 230 that were measured at the time of diagnosis were significantly higher in patients with neurological disorders than in patients without neurological disorders (p = 0.042; p = 0.018). Among the pre-existing comorbidities, neurological disorders, particularly stroke, and malignancies were found to be significantly connected to the occurrence of BP in the selected Turkish population. The high titers of serum anti-BP180 and 230 IgG antibodies at the time of BP diagnoses may highlight undiagnosed pre-existing neurological disorders by provoking suspicion.

Evaluation of non-endemic pemphigus foliaceus in a large series of patients: a single-center retrospective study from Turkey focuses on the relapses.

BACKGROUND: Pemphigus foliaceus is exceedingly rare around the world, except within the few regions where it occurs as an endemic variant. Various factors can trigger immune mechanisms that induce pemphigus foliaceus or worsen its course. OBJECTIVE: To determine the demographic and clinical characteristics of the patients with pemphigus foliaceus in a large series from a non-endemic country, investigate the triggering factors, and seasonal patterns. METHODS: The data of the patients diagnosed with pemphigus foliaceus in the study's center between 1989-2018 were retrospectively analyzed. RESULTS: Sixty-eight patients (mean age, 45.7 ± 14.5 years) were included in the study. The number of onsets reached its peak in spring-summer (p = 0.008). A total of 117 relapses occurred in 42 patients and were most common in spring-summer (not significant). Specific trigger factors were detected in 45 relapses. In the other 72 relapses, the peak was observed in spring-summer (p = 0.005). There were no significant differences in the demographic and clinical variables investigated between relapsed and non-relapsed patients. STUDY LIMITATIONS: Retrospective design. CONCLUSIONS: Triggering factors could not be identified in more than half of the relapses in the study's series. The subgroup of relapses (without identified causes), as well as the onsets of the disease, showed a significant seasonal variation with a peak in spring-summer; however, the seasonal variable did not justify the total group of relapses. Although the seasonal variation may be caused by a combination of factors, UV radiation should be considered a trigger factor for the peaks in spring-summer, particularly in Turkey.

Comparison of three different dose regimens of methylprednisolone as initial therapy for pemphigus vulgaris of moderate severity: A single-center retrospective study from Turkey.

BACKGROUND: Currently, no consensus exists on the dosing regimen of corticosteroids in relation to disease severity in pemphigus vulgaris. OBJECTIVE: To evaluate the efficacy of three initial dose regimens of methylprednisolone in the treatment of moderate pemphigus. METHODS: We retrospectively analysed the data of 46 patients with moderate pemphigus vulgaris, who initially received either low (0.60-0.80 mg/kg/day), moderate (0.81-1.20 mg/kg/day) or high (1.21-2.0 mg/kg/day) doses of methylprednisolone. RESULTS: The median time to disease control and hospital stay was shortest in the high-dose group (8.5 days and 27.5 days, respectively). The low-dose group had the highest rate of adjuvant therapy (88.8%, P = 0.035). Cumulative methylprednisolone doses and corticosteroid-related adverse events were similar in the moderate- and high-dose groups. The initial methylprednisolone dose showed a significant inverse correlation with time to disease control (r = -0.319, P = 0.031) and a weak inverse correlation with length of hospital stay (r = -0.282, P = 0.058). LIMITATIONS: Retrospective design and small patient size are major limitations. CONCLUSION: In the treatment of moderate pemphigus vulgaris, high initial doses of corticosteroid seem to provide early disease control and shortened hospital stay without notable increases in cumulative corticosteroid doses. This treatment strategy may lower the risk for nosocomial infections and reduce the economic burden of pemphigus.

Infective endocarditis in two patients with pemphigus vulgaris under multiagent immunosuppressive drug therapy: A rare entity to remember in the differential diagnosis of fever of unknown origin.

Pemphigus vulgaris (PV) is an autoimmune blistering skin disease that may require multiagent immunosuppressive drug therapies in severe cases. In addition to the well-established corticosteroid sparing agents azathioprine, mycophenolate mofetil, and methotrexate, rituximab is being increasingly used alone or in combination in the management of PV. Due to the chronic course of the disease, the cumulative effects of these therapy agents over long follow-up periods may result in various adverse reactions, including bacterial and viral infections. Infective endocarditis (IE) is one of the rarest complications of PV treatment with only a few reported cases. In the present study, the present authors discuss two PV patients without prior histories of cardiovascular disease, complicated with native aortic valve IE, which led to death in one of the patients. Because determining the origin of fever is difficult in patients under immunosuppressive therapy, it is particularly challenging to diagnose IE coupled with a fever of unknown origin. Therefore, dermatologists must be vigilant for the dermatological signs of IE and use a multidisciplinary approach to the differential diagnosis of fever of unknown origin.

Polycyclic annular presentation of pemphigus vulgaris with an eosinophil predominance in two pregnant patients.

Pemphigus during pregnancy has a more complicated course owing to the limitations in treatment options and alterations in the severity and presentation of the clinical features. We would like to present two pemphigus vulgaris (PV) cases associated with pregnancy with an unusual clinical appearance exhibiting polycyclic, annular, vesiculobullous plaques with marked eosinophil infiltration in histopathology. To the best of our knowledge pregnancy-associated pemphigus cases with this particular clinical presentation have not been reported in the literature. Changes in the immunologic and hormonal state during pregnancy may play a role in altering the classic clinical presentation and treatment response of PV.

Childhood pemphigus vulgaris: five cases in 16 years.

Pemphigus vulgaris (PV) usually occurs in adults. There are only a few reports of large PV series concerning childhood cases. We report here five cases of PV in patients younger than 16 years. They were analyzed among 169 PV cases out of a total of 192 pemphigus patients diagnosed between 1988-2004. The ratio of childhood cases was 2.9% in our large PV series. This relatively high ratio of childhood patients suggests that PV should not be neglected in the differential diagnosis of bullous lesions in childhood. Four of the five cases were followed up between 2-4 years and all of these four cases showed at least one relapse. PV also seems to show a relapsing course in the pediatric age group like in adults.

Vascular involvement in Behçet's disease: a retrospective analysis of 2319 cases.

BACKGROUND: Vascular lesions can be the presenting sign of Behçet's disease (BD) preceding classical symptoms. The aim of this study was to evaluate the prevalence and types of vascular involvement in BD. METHODS: Among 2319 patients diagnosed with BD according to the criteria of the International Study Group for BD, 332 patients (279 male, 53 female) with vascular involvement were included in this study. RESULTS: Prevalence of vascular involvement was 14.3%. Vascular involvement was found to be more common in males (P<0.001, male:female ratio 5.26:1). Mean age at onset of vascular involvement was 30.58+/-7.88. Vascular lesion was the presenting sign of BD in 50 patients (2.1%), whereas in 109 patients (4.7%) it started together with other clinical symptoms at the age of diagnosis. Superficial vein thrombophlebitis (SVT) was the most common vascular symptom (53.3%) followed by deep vein thrombosis (DVT) (29.8%). Arterial lesions were rare (3.6%). Of the patients 13.3% had more than one type of vascular involvement. Lower extremities were the most frequent localization in patients with SVT, whereas the femoral vein was mainly involved in patients with DVT and the pulmonary artery was mainly involved in the patients with arterial lesions. CONCLUSION: In countries like Turkey where BD is more frequent, young male patients presenting with vascular involvement should be evaluated for BD.