Oral Pemphigus Vulgaris After Chilean Earthquake.

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Oesophagitis dissecans superficialis: an acute, benign phenomenon associated with pemphigus vulgaris

Pemphigus vulgaris (PV) is an autoimmune dermatosis that may evolve to severely compromise the skin and/or mucosa. Autoantibodies directed against epithelial cadherins, such as desmogleins 1 and 3, lead to acantholysis and culminate in blister formation. Involvement of the oral mucosa is common, but other squamous stratified epithelia may also be the target of the autoimmune aggression. We report a woman with PV that was in partial remission, who developed an unusual acute phenomenon, known as oesophagitis dissecans superficialis.

Treatment of pemphigus vulgaris and pemphigus foliaceus: experience with 71 patients over a 20 year period

Forty one cases of pemphigus vulgaris and thirty cases of pemphigus foliaceus were investigated at Hospital Universitário Clementino Fraga Filho from 1978 to 1999. They were divided into two treatment groups: one group received up to 100 mg of oral prednisone daily and the other group received >120 mg daily. The dose up to 100 mg provided good initial control of pemphigus vulgaris and pemphigus foliaceus and did not increase the mortality rate associated to disease. The dose >120 mg induced higher morbidity. These data allowed us to establish a regimen of oral prednisone (1-2 mg/kg/daily) with maximum of 120 mg daily in the treatment of pemphigus vulgaris and pemphigus foliaceus.

Refractory pemphigus vulgaris associated with herpes infection: case report and review

BACKGROUND: Pemphigus vulgaris (PV) is an autoimmune disease characterized by blistering of the skin and mucosa, which develops due to the interaction between predisposing genetic and environmental factors. Infections caused by members of the Herpesviridae family have been suggested as a possible triggering factor for PV. OBJECTIVE AND METHODS: In this report, we investigate the presence of herpesviruses in refractory lesions on the right upper eyelid. The lesion has persisted despite the treatment with corticosteroids. Polymerase chain reaction (PCR) and DNA sequence analysis have been used to detect the DNA of HSV 1/2, VZV, EBV, CMV, HHV-6, HHV-7, and HHV-8. RESULTS: The sample collected from the right upper eyelid has tested positive for HSV 1/2. Sequence analysis has confirmed the PCR results and allowed the identification of the HSV strain as belonging to type 1. After treatment with acyclovir, the lesion of the right upper eyelid has cleared and not relapsed. CONCLUSION: When patients present PV lesions which are refractory to corticosteroid therapy, herpetic infection should be considered.

Nail manifestations in pemphigus vulgaris

Nail involvement in pemphigus vulgaris is rare. We describe 5 patients with pemphigus vulgaris presenting nail involvement. In this disease, nail manifestations present, by order of frequency, as chronic paronychia, onychomadesis, onycholysis, Beau's lines and trachyonychia. All our 5 cases presented with paronychia, and 1 of them also had Beau's lines. Treatment with prednisone and/or cyclophosphamide controlled mucocutaneous and nail manifestations in all cases.

Endemic pemphigus foliaceus (fogo selvagem) and pemphigus vulgaris: immunoglobulin G heterogeneity detected by indirect immunofluorescence

Pemphigus are autoimmune intraepidermal blistering diseases in which immunoglobulin G (IgG) autoantibodies are directed against desmosomal glycoproteins. The aim of this study was to determine the IgG subclass profile of endemic pemphigus foliaceus (fogo selvagem) and pemphigus vulgaris utilizing indirect immunofluorescence. PATIENTS AND METHODS: Twenty-five patients with pemphigus vulgaris, 25 with endemic pemphigus foliaceus (fogo selvagem), and 25 healthy controls were analyzed by indirect immunofluorescence for circulating autoantibodies (total IgG and its subclasses). RESULTS: Our data revealed a significant correlation (P <.05) of disease activity and autoantibody levels in both forms of pemphigus, i.e., negative titers related to clinical remission, whereas positive results related to active disease. Immunoglobulin G subclass analysis in fogo selvagem demonstrated that in patients in remission, 56% showed positive immunoglobulin G4; in active disease, immunoglobulin G4 was the predominant subclass (100% positive in all cases). The IgG subclass profile in pemphigus vulgaris showed that in patients in remission, only 10% were positive for immunoglobulin G4; in active disease, positivity for immunoglobulin G4 was present in 78% to 88% of the cases. CONCLUSION: Subclass characterization of immunoglobulin G autoantibodies is a useful tool for pemphigus follow-up, since immunoglobulin G4 (IgG4) is the subclass that is closely related to recognition of pathogenic epitopes, and consequently with disease activity. Careful monitoring should be performed for fogo selvagem in clinical remission with a homogeneous IgG4 response, since this may indicate more frequent relapses.

Orodigital pemphigus vulgaris : a pathogenic role of anti-desmoglein-3 autoantibodies in pemphigus paronychia?

Paronychia is a rare complication of pemphigus vulgaris (PV), and the immunological profile of patients with digital disease has not been assessed so far. We report 2 cases of PV with oral mucosa and periungual involvement, who had high titers of anti- desmoglein (Dsg)-3 circulating antibodies. These observations raise the possibility that expression of Dsg-1 and Dsg-3 in distinct areas of periungual skin is disease specific and that anti-Dsg-3 antibodies alone may have an as yet unrecognized importance for the development of paronychia in PV.

Low-level laser therapy on the treatment of oral and cutaneous pemphigus vulgaris: case report

Pemphigus vulgaris is a chronic autoimmune mucocutaneous disease that initially is manifested by painful intraoral erosions and ulcers which spread to other mucosa and the skin, generally more than 5 months after oral lesion manifestation. The treatment consists of prednisone alone or in combination with an immunosuppressive agent, and the clinical response is perceived within 2 to 4 weeks. Low-level laser therapy has been effective in accelerating the healing of injured tissue, thus inducing cell proliferation and increasing ATP, nucleic acid, and collagen synthesis. We reported two cases of pemphigus vulgaris that received systemic treatment associated with low-level laser therapy for oral and cutaneous lesions. We observed prompt analgesic effect in oral lesions and accelerated healing of oral and cutaneous wounds. Therefore, the present report suggests LLLT as a noninvasive technique that should be considered as an adjuvant therapy in oral and skin disorders in patients with PV.

Gingival lesions diagnosed as pemphigus vulgaris in an adolescent. Case report

Desquamative gingivitis (DG) is a fairly common disorder in which the gingivae show chronic desquamation. Originally considered to be related to hormonal changes at menopause, since many of the patients are middle-aged women, DG is now recognized to be mainly a manifestation of a number of disorders ranging from vesiculobullous diseases to adverse reactions to a variety of chemicals or allergens. Desquamative gingivitis can be an important early clinical manifestation of serious systemic diseases such as pemphigus vulgaris. The authors present a case that illustrates the importance of a specific diagnosis in patients with desquamative gingival lesions previously treated for 6 months as classical gingivitis. Gingival biopsy showed histologic patterns typical of pemphigus vulgaris. The patient was treated with systemic and topical corticosteroids in association with miconazole the patient is now under control with low-close systemic corticosteroids. Proper recognition of lesions in the oral mucosa leads, in several situations, to an early diagnosis of a systemic disease.

Evaluation of cases of pemphigus vulgaris and pemphigus foliaceus from a reference service in Pará state, Brazil

BACKGROUND: Pemphigusis a bullous, rare and chronic autoimmune disease. There are two major forms of pemphigus: vulgaris and foliaceus. Epidemiological data and clinical outcome in patients diagnosed in the Brazilian Amazon states are still rare. OBJECTIVES: To study the occurrence of the disease during the study period and analyze the epidemiological profile of patients, the most common subtype of pemphigus, and the clinical evolution of patients. METHODS: Retrospective analysis of medical records of hospitalized patients with pemphigus foliaceus and pemphigus vulgaris in the period from 2003 to 2010 in Dermatology Service of Hospital Fundação Santa Casa de Misericórdia do Pará, Belém, Northern Brazil. RESULTS: We found a total of 20 cases of pemphigus during the study period, 8 of which were of foliaceus pemphigus and 12 of vulgaris pemphigus. Pemphigus foliaceus had the predominance of male patients (75%), showed satisfactory clinical evolution, and was characterized by absence of pediatric cases. Pemphigus vulgaris affected more women (66.7%), showed mean hospital stay of 1 to 3 months (50%), and there were three cases of death (25%). The prescribed immunosuppressive drugs included prednisone with or without combination of azathioprine and/or dapsone. Sepsis was associated with 100% of the deaths. CONCLUSIONS: The occurrence of the disease is rare, there are no familiar/endemic outbreaks in the sample. Evolution is usually favorable, but secondary infection is associated with worse prognosis. The choice of best drugs to treat pemphigus remains controversial.

Pemphigus vulgaris in adolescence: a case report

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Vulvo-cervico-vaginal manifestations and evaluation of Papanicolaou smears in pemphigus vulgaris and pemphigus foliaceus

Background: Vulvo-cervico-vaginal involvement has rarely been reported in pemphigus vulgaris (PV) and has not been reported in pemphigus foliaceus (PF). Objectives: We sought to evaluate genital lesions and Papanicolaou (Pap) smears in female patients with PV and PF. Methods: This prospective study includes all consecutive cases of female patients with PV and PF seen from May 2009 to February 2010. Gynecologic examination was performed and Pap smears were collected for cytologic analysis from each patient. Results: A total of 56 patients were given a diagnosis of pemphigus (41 PV and 15 PF). Genital involvement was observed in 9 patients with PV (22%) and the vulva was the most common genital site of involvement. Of these 9 patients, 8 presented with active skin/mucous lesions. Four of 15 patients with PF had genital lesions and vulva was the exclusive site of involvement. Three of 4 patients with PF and genital involvement also showed active cutaneous lesions. Six of 56 patients (5 PV and 1 PF) presented with atypical squamous cells of undetermined significance in Pap smear analysis. Upon further pathologic review, acantholytic cells were seen, confirming the diagnosis of pemphigus. Limitations: A small number of PF cases were studied. Conclusions: Vulvar lesions were the second most frequent site of mucous membrane PV. Herein we report the first case to our knowledge of symptomatic genital lesions in a patient with PF. Moreover, acantholytic cells in Pap smears were found in a patient with PF who was in complete remission off therapy with no clinical genital lesions and no circulating anti-desmoglein-1 and anti-desmoglein-3 autoantibodies. Gynecologic evaluation in patients with pemphigus, including a careful evaluation of Pap smears, should be recommended. (J Am Acad Dermatol 2012;67:409-16.)

Laryngeal involvement in pemphigus vulgaris: a proposed classification

Objective: (1) To investigate the incidence of laryngeal involvement in a large series of patients with pemphigus vulgaris, using endoscopic examination, (2) to describe the lesions, and (3) to establish a classification of laryngeal involvement in pemphigus vulgaris based on the location of the lesions. Study design: Prospective study. Methods: A total of 40 sequentially treated pemphigus vulgaris patients, diagnosed using clinical, histological and immunofluorescence criteria, were evaluated for laryngeal manifestations using endoscopic examination. The results were used to establish a graded classification of laryngeal involvement according to the location of the lesions. Results: Active laryngeal lesions (ulcers or blisters) were found in 16 patients (40 per cent). Of these, 37.5 per cent were classified as grade I, 20 per cent as grade II, 20 per cent as grade III and 17.5 per cent as grade IV. Conclusion: Laryngeal involvement is common in pemphigus vulgaris and must be considered at the point of diagnosis. Grade I lesions are the most frequent.

Clinical activity of pemphigus vulgaris relates to IgE autoantibodies against desmoglein 3

Pemphigus vulgaris (PV) is a severe autoimmune bullous skin disease and is primarily associated with IgG against desmoglein 3 (dsg3), a desmosomal adhesion protein. In light of the recent association of autoreactive T helper (Th) 2 cells with active PV, the present study sought to relate the occurrence of Th2-regulated dsg3-specific autoantibody subtypes, i.e. IgE and IgG4, in 93 well-characterized PV patients. Patients with acute onset PV (n=37) showed the highest concentrations of serum IgE and IgG4 autoantibodies, which were significantly lower in PV patients in remission (n=14). Furthermore, there was a strong correlation between dsg3-reactive IgE and IgG4 in acute onset, but not in chronic active (n=42) or remittent patients. Additionally, intercellular IgE deposits were detected in the epidermis of acute onset PV. Thus, dsg3-specific IgE and IgG4 autoantibodies are related to acute onset disease which provides additional support to the concept that PV is a Th2-driven autoimmune disorder.

Pemphigus vulgaris identifies plakoglobin as key suppressor of c-Myc in the skin

The autoimmune disease pemphigus vulgaris (PV) manifests as loss of keratinocyte cohesion triggered by autoantibody binding to desmoglein (Dsg)3, an intercellular adhesion molecule of mucous membranes, epidermis, and epidermal stem cells. Here we describe a so far unknown signaling cascade activated by PV antibodies. It extends from a transient enhanced turn over of cell surface-exposed, nonkeratin-anchored Dsg3 and associated plakoglobin (PG), through to depletion of nuclear PG, and as one of the consequences, abrogation of PG-mediated c-Myc suppression. In PV patients (6/6), this results in pathogenic c-Myc overexpression in all targeted tissues, including the stem cell compartments. In summary, these results show that PV antibodies act via PG to abolish the c-Myc suppression required for both maintenance of epidermal stem cells in their niche and controlled differentiation along the epidermal lineage. Besides a completely novel insight into PV pathogenesis, these data identify PG as a potent modulator of epithelial homeostasis via its role as a key suppressor of c-Myc.