Surgical management of gingival recessions in patients with refractory gingival pemphigus vulgaris: A multidisciplinary challenge.

BACKGROUND: Mucogingival surgery for root coverage of gingival recessions (GRs) is usually performed in patients with unremarkable periodontal and systemic health. However, the predictable results of surgical procedures and increasingly high aesthetic expectations of patients necessitate optimal management of GR also in patients with systemic conditions that affect the oral cavity. In patients with pemphigus vulgaris (PV), mucosal fragility and complicated surgical management of inflamed soft tissues are major challenges. METHODS AND RESULTS: A 36-year-old female patient with PV and deep GR on the mandibular incisors is presented. After initial unresponsiveness to steroids and immunosuppressants, complete clinical remission was achieved through repeated rituximab infusions and topical platelet-rich plasma. After > 1 year of stable clinical remission off therapy the patient successfully underwent surgical procedures for vertically coronally advanced flap with connective tissue graft. CONCLUSIONS: To the best of our knowledge, no studies have described the surgical management of GR in PV patients. Although controlled studies are required to confirm present results, complete and stable clinical remission is necessary to avoid complications. Collaboration among dermatologists, oral medicine specialists, and periodontologists is essential to determine whether mucogingival surgery for root surface exposure is indicated for PV patients. KEY POINTS: Why are these cases new information? This is the first report of root coverage in a patient with oral PV What are the keys to the successful management of these cases? The achievement of complete and stable clinical remission from oral PV Multidisciplinary collaboration among dermatologists, oral medicine specialists, and periodontologists What are the primary limitations to success in these cases? The refractoriness of gingival lesions induced by PV Poor mucogingival conditions of inflamed gingival tissues exacerbated by PV.

Esophagogastroduodenoscopy and Esophageal Involvement in Patients with Pemphigus Vulgaris.

Pemphigus vulgaris (PV) is a rare autoimmune blistering disease involving the skin and mucous membranes. The prevalence of esophageal involvement remains uncertain. The aim of our study was to determine the frequency of esophageal involvement in patients with PV. This is a single-center electronic database retrospective review of patients with a diagnosis of PV. Data abstracted included demographics, disease characteristics (biopsy results, symptoms, areas affected, treatments), and esophagogastroduodenoscopy (EGD) reports. Of the 111 patients that met eligibility criteria, only 22 (19.8%) underwent EGD. Demographic data were similar except those who underwent EGD were more likely to be female (77.3% vs. 51.7%, p = 0.05) and have hypertension (50.0% vs. 24.7%, p = 0.04). Esophageal symptoms were common in both groups; however, those experiencing dysphagia were more likely to undergo EGD (50.0% vs. 20.2%, p = 0.007). Those who underwent EGD had more refractory disease (≥ 3 treatment modalities: 100% vs. 58.4%, p < 0.001), but did not differ in areas affected. Of those who underwent EGD, only 4 (18.2%) had esophageal abnormalities either prior to PV diagnosis (1) or during a disease flare (3). Those having a flare were more likely to experience odynophagia (69.2%) or weight loss (61.5%), p = 0.02 and p = 0.05, respectively. While esophageal symptoms were common in our cohort of PV patients, a minority of patients underwent EGD, and the vast majority of those were unremarkable. This suggests that while esophageal symptoms are common in PV, permanent esophageal injury is more rare.

Nasal, oral, and pharyngolaryngeal manifestations of pemphigus vulgaris: Endoscopic ororhinolaryngologic examination.

Pemphigus vulgaris is an autoimmune blistering disorder that involves the skin and mucous membranes. Few reports have described nasal and oropharyngolaryngeal lesions in pemphigus vulgaris using an endoscopic ororhinolaryngologic examination. We retrospectively reviewed the clinical records of 11 patients with pemphigus vulgaris between 2001 and 2013 with respect to their symptoms, lesion sites, lesion features, and treatments received. All patients had undergone an endoscopic ororhinolaryngologic examination. Their mucosa-related symptoms were sore throat, oral pain, odynophagia, gingival bleeding, hoarseness, and epistaxis. The most frequent sites were the oral cavity (gingiva and buccal mucosa), larynx (epiglottis and vocal fold), oropharynx (soft palate), and nasal cavity (nasal septum). Lesions were typically characterized by erosion, erosion with a whitish exudate, and erythematous patches. Thus, our study findings reveal that pemphigus vulgaris involves both the nasal and oropharyngolaryngeal regions. Patients with pemphigus vulgaris should undergo an endoscopic ororhinolaryngologic examination to determine the range of their lesions.

Pemphigus vulgaris of the cervix: diagnostic difficulties associated with the Pap test.

Pemphigus vulgaris (PV) is a rare mucocutaneous disease caused by the abnormal production of antibodies against epithelial cell surface glycoproteins, resulting in loss of cell adhesion and intraepithelial blister formation. Cervical involvement in PV has been poorly reported, and there is little information regarding the criteria about consequential cytological changes identified in a Papanicolaou-stained cervicovaginal smear (Pap smear). Here, we report a case of PV manifesting in the cervix as well as the difficulty associated with the cytomorphological identification and interpretation of acantholytic cells. This case involved a 40-year-old patient with no history of Pap test abnormalities and no prior diagnosis of PV. In the cytological assessment, cells were identified both in isolation and in clusters that exhibited round nuclei of increased volume, inconspicuous nucleoli, and perinuclear halos. The patient underwent a cervical biopsy that revealed vesiculobullous lesions and morphological pattern consistent with PV. A skin biopsy confirmed this diagnosis. We concluded that knowledge of PV cytomorphology is important because difficulties associated with the identification and interpretation of acantholytic cells might be responsible for false positive diagnoses of cervical neoplasia. However, a suspected diagnosis of PV is possible if the cytological findings are carefully correlated with the clinical data.

Mediastinal follicular dendritic cell sarcoma with paraneoplastic pemphigus.

Follicular dendritic cell sarcoma is a rare neoplasm of immune accessory cells. It occurs primarily in lymph nodes. Occurrences in the mediastinum are rarely reported. Diagnosis and management of follicular dendritic cell sarcoma remain unclear, and it is an under-recognized clinical entity. Only a few cases of paraneoplastic pemphigus as the first presentation of follicular dendritic cell sarcoma have been reported. We report an unusual case of follicular dendritic cell sarcoma of the anterior mediastinum, presenting as paraneoplastic pemphigus in a 62-year-old man. Typical histological features confirmed the diagnosis of follicular dendritic cell sarcoma, and surgical resection was successfully performed.

Pemphigus vulgaris: bilateral plica semilunaris involvement.

PURPOSE: To present a case of pemphigus vulgaris, involving the conjunctiva, specifically the plica semilunaris, in both eyes of a 33-year-old woman. METHODS: Ocular evaluation showed bilateral plica semilunaris vegetations extending for 3 mm linearly. Sequential excisional biopsies of both affected plica were performed and sent for pathological examination and immunofluorescent staining. RESULTS: After each excisional biopsy, the patient's ocular symptoms resolved. Excisional biopsies showed suprabasilar clefting within the epithelium; lymphoplasmacytic infiltration and immunopathology showed intraepithelial intercellular and basement membrane zone staining with immunoglobulin G consistent with pemphigus vulgaris. At 1-year follow-up, the patient continues to be asymptomatic with no signs of recurrence. CONCLUSIONS: Excisional biopsy, in a very symptomatic patient with pemphigus vulgaris with conjunctival vegetations, may hasten his or her recovery.

Pemphigus vulgaris treated with allogeneic hematopoietic stem cell transplantation following non-myeloablative conditioning.

OBJECTIVES: Successful treatment of severe, refractory Pemphigus vulgaris (PV) is reported. METHODS: Reduced intensity, non-myeloablative conditioning was employed, followed by allogeneic hematopoietic stem cell transplantation (HSCT) from a fully matched sibling. RESULTS: Treatment of refractory PV with myeloablation and subsequent allogenic HSCT has been previously reported, and sustained remission with this approach has been achieved. Toxicity, however, related to condition regimens remains high. CONCLUSION: Since no cytotoxic chemotherapy was used, it is hereby hypothesized that clinical improvement may result from regulatory action from the donor's marrow: a "graft modifying the disease" effect, which may prove useful in the management of autoimmune diseases.

Pemphigus vulgaris: a therapeutic option for disease control.

Pemphigus vulgaris (PV) is an autoimmune intra-epithelial dermatologic disease that is characterized by epithelial blistering and affected cutaneous and/or mucosal surfaces. The disease is caused by circulating immunoglobulin G (IgG) antibodies directed against desmosomes. These antibodies interfere with keratinocyte adhesion, causing acantholysis, which results in blister formation. At present, death from pemphigus infection is unusual; however, morbidity and mortality from chronic corticosteroid use remain considerable, indicating a need for additional therapies. This article presents a case of PV in a female patient that was treated with conventional corticosteroid therapy and discusses recent adjuvant therapy.

[Pemphigus vulgaris and intraepithelial neoplasia localized to the vagina]. / Pemphigus vulgaire et néoplasie intra-épithéliale de localisation vaginale.

BACKGROUND: Involvement of the vaginal mucosa in pemphigus vulgaris is a rare occurrence. Here we report an original case that resulted in discovery of intra-epithelial neoplasia at the same site. PATIENTS AND METHODS: A 63 year-old woman was followed for 18 years for pemphigus vulgaris treated with prednisone, initially in combination with azathioprine. An erosive lesion was discovered in the pouch of Douglas during routine gynecological examination and demonstrated the histological features of pemphigus, despite remission of the disease at other sites. In spite of resumption of azathioprine and prednisone, the vaginal lesion continued to spread. A further biopsy revealed intra-epithelial vaginal neoplasia together with images of suprabasal cleavage and acantholysis. Surgical removal was carried out. DISCUSSION: Intra-epithelial carcinoma associated with pemphigus vulgaris has been described in rare cases in the cervix but never in the vagina.

Pénfigo vulgar en la infancia / Pemphigus vulgaris in childhood

El pénfigo vulgar en la infancia es muy infrecuente, con poco más de 50 casos descritos en la literatura. Presentamos un nuevo caso de pénfigo vulgar en un niño de 11 años, comentando su evolución y respuesta al tratamiento y revisamos los casos comunicados en este grupo de edad, con especial atención al manejo terapéutico y al pronóstico de esta enfermedad en la infancia

A new case of pemphigus vulgaris in an 11 years-old boy is reported. Pemphigus vulgaris is infrequent in childhood, with over 50 cases previously reported. We comment the evolution and the response to treatment. We reviewed other cases reported in children, with special attention to the therapeutic and the prognosis of the disease in childhood

Pénfigo vulgar - Caso clínico / Pénfigo vulgar - Caso clínico

El pénfigo vulgar es una enfermedad auto-inmune ampollosa, muco cutánea, de carácter crónico, donde las lesiones de la mucosa bucal pueden anteceder las lesiones cutáneas. Así, el odontólogo asume papel importante en la identificación de las lesiones, debiendo realizar el diagnóstico por la historia clínica i exámenes complementares adecuados. Este artículo relata un caso de pénfigo vulgar acometiendo un paciente del sexo masculino,47 años, con lesiones en la mucosa bucal y piel (AU)

Pemphigus vulgaris is an autoimmune blistering disease, mucocutaneous, chronic, where the lesions in the mouth; mucosa usually precede the skin lesions. Thus, the dentist has an important role in identifying the lesions, and the ;;diagnosis must be done through an adequate clinical history and complementary exams. This article reports a case of pemphigus vulgaris in a male patient, 47, suffering from lesions in the skin and mouth mucosa (AU)

Paraneoplastic pemphigus associated with pelvic inflammatory fibrosarcoma: a case report.

A 36-year-old African-American woman presented with an extensive stomatitis and pigmented cutaneous macules on the neck, axillae and hands. Subsequently she developed violaceus papules on the dorsa of the hands, histologically consistent with an interface dermatitis. After 18 months of progressive disease, paraneoplastic pemphigus was suspected and a search for an underlying neoplasm was initiated. An exploratory laparotomy revealed a pelvic mass and the histologic examination showed an inflammatory fibrosarcoma. The evidence of acantholysis on new cutaneous lesions and the positivity of indirect immunofluorescence with rodent urinary bladder epithelium reinforced the diagnostic criteria for paraneoplastic pemphigus, which is confirmed by the identification of strong protein bands at 210, 190 and 170 kd by immunoprecipitation. Paraneoplastic pemphigus should be considered when investigating atypical mucocutaneous manifestations of pemphigus vulgaris and lichen planus. Diagnostic screening for paraneoplastic pemphigus and a search for an underlying tumor should be performed.

Paraneoplastic pemphigus and bronchiolitis obliterans associated with a mediastinal mass: A rare case of Castleman's disease with respiratory failure requiring lung transplantation.

Castleman's disease is an infrequent and usually benign lymphoproliferative disorder. Resection of the tumor usually is curative. The immunostimulatory nature of the tumor can, in rare instances, result in paraneoplastic manifestations. The authors present a case of a 14 year old with mucocutaneous ulcerations and progressive dyspnea that was found to have a large mediastinal mass and circulating autoantibodies that were responsible for his paraneoplastic pemphigus and bronchiolitis obliterans. In spite of aggressive immunotherapy to control the autoimmune mucocutaneous lesions, the pulmonary fibrosis was irreversible and progressed to pulmonary failure necessitating lung transplantation. J Pediatr Surg 36:E22.

Trastornos cutáneos y no cutáneos tratadoscon fotoféresis extracorpórea / Cutaneous and noncutaneous disorders treated with extracorporeal photopheresis

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Thymoma with pemphigus foliaceus.

A 75-year-old Japanese woman was referred to us because of an anterior mediastinal mass. Crusts and shallow erosions developed 10 months earlier on her upper chest, back, and scalp. Pemphigus foliaceus was diagnosed based on histological examination of skin biopsy specimens and positivity for serum anti-desmoglein 1 antibody by enzyme-linked immunosorbent assay. Neurological examination and electromyography ruled out myasthenia gravis. Total thymectomy was performed, and the postoperative pathology studies showed mixed lymphoepithelial thymoma. One year after the resection, the eruption and alopecia improved and the serum anti-desmoglein 1 antibody titer decreased, suggesting a beneficial effect of thymectomy on thymoma-related pemphigus.

Carbon dioxide laser vaporization of recalcitrant symptomatic plaques of Hailey-Hailey disease and Darier's disease.

Carbon dioxide laser vaporization of localized, recalcitrant intertriginous plaques was done in two patients with Hailey-Hailey disease and two patients with Darier's disease. After treatment, only one patient had recurrence in one treatment site. All patients had significant clinical and symptomatic improvement. Destructive treatment must include the follicular infundibulum in Darier's disease, because histologically this was the focus of recurrent disease at one treated site. We suggest carbon dioxide laser vaporization for the treatment of chronic, localized, symptomatic plaques of Hailey-Hailey or Darier's disease, if medical therapy has been ineffective.