Proteomic Profiling of Central Centrifugal Cicatricial Alopecia Reveals Role of Humoral Immune Response Pathway and Metabolic Dysregulation.

Proteomic profiling on other primary cicatricial alopecias, such as frontal fibrosing alopecia and lichen planopilaris, have suggested a T helper 1-mediated inflammatory pathway, but in central centrifugal cicatricial alopecia (CCCA), the protein expression patterns are unknown. In this study, we sought to characterize protein expression patterns in CCCA to identify biomarkers of disease activity that will identify potential therapeutic avenues for treatment. Scalp protein quantification was performed to understand protein expression patterns in affected versus unaffected scalps in CCCA.

The role of adjuvant therapy in pemphigus: A systematic review and meta-analysis.

Background: The assumption that adjuvant modalities have added value to oral glucocorticoids in the treatment of pemphigus is intuitively sound but has not been conclusively proven.

Objective: We sought to compare the efficacy and safety of oral glucocorticoid treatment with or without adjuvants for pemphigus vulgaris and pemphigus foliaceus.

Methods: We performed a systematic review and meta-analysis of randomized controlled trials.

Mucosal pemphigus vulgaris anti-Dsg3 IgG is pathogenic to the oral mucosa of humanized Dsg3 mice.

There are two major clinical subsets of pemphigus vulgaris (PV)-mucosal PV (mPV) and mucocutaneous PV (mcPV). The mPV subset exhibits anti-human desmoglein (Dsg) 3 autoantibodies that fail to recognize murine Dsg3 (mDsg3); thus, passive transfer experiments of mPV IgG into wild-type (WT) mice have been unsuccessful at inducing disease. We therefore generated a fully humanized Dsg3 (hDSG3) murine model utilizing a hDsg3 transgenic animal crossed to the mDsg3 knockout line.

Pemphigus vulgaris autoantibody profiling by proteomic technique.

Pemphigus vulgaris (PV) is a mucocutaneous blistering disease characterized by IgG autoantibodies against the stratified squamous epithelium. Current understanding of PV pathophysiology does not explain the mechanism of acantholysis in patients lacking desmoglein antibodies, which justifies a search for novel targets of pemphigus autoimmunity. We tested 264 pemphigus and 138 normal control sera on the multiplexed protein array platform containing 701 human genes encompassing many known keratinocyte cell-surface molecules and members of protein families targeted by organ-non-specific PV antibodies.

Successful treatment of pemphigus with biweekly 1-g infusions of rituximab: a retrospective study of 47 patients.

Background: Rituximab is increasingly being appreciated as a remarkably effective treatment for pemphigus, mostly concomitantly with other immunosuppressive medications. The majority of studies have used a single cycle of rituximab with the same dosage as approved for the treatment of lymphomas, ie, 375 mg/m(2) weekly × 4 weeks. Rituximab is also approved for the treatment of rheumatoid arthritis, with a different dosing regimen: 1000 mg × 2, days 1 and 15.

High-dose cyclophosphamide without stem cell rescue in 207 patients with aplastic anemia and other autoimmune diseases.

High-dose cyclophosphamide has long been used as an anticancer agent, a conditioning regimen for hematopoietic stem cell transplantation, and a potent immunosuppressive agent in autoimmune diseases including aplastic anemia. High-dose cyclophosphamide is highly toxic to lymphocytes but spares hematopoietic stem cells because of their abundant levels of aldehyde dehydrogenase, the major mechanism of cyclophosphamide inactivation. High-dose cyclophosphamide therapy induces durable remissions in most patients with acquired aplastic anemia.

Treating pemphigus vulgaris with prednisone and mycophenolate mofetil: a multicenter, randomized, placebo-controlled trial.

Non-blinded trials of pemphigus vulgaris suggest that mycophenolate mofetil (MMF) may be beneficial. In a prospective, multicenter trial, outpatients with mild or moderate pemphigus vulgaris were randomized to MMF (2 or 3 g day(-1)) plus oral corticosteroids or placebo plus oral corticosteroids for 52 weeks. The primary end point was the proportion of patients in the placebo and combined MMF groups responding to treatment (absence of new, persistent oral or cutaneous lesions, and prednisone dose < or = 10 mg day(-1) from weeks 48 to 52).

Treatment of ocular mucous membrane pemphigoid with immunosuppressive drug therapy.

Purpose: To evaluate the effectiveness of immunosuppressive drug therapy in the treatment of ocular mucous membrane pemphigoid (MMP).

Design: Retrospective cohort study.

Participants: Ninety-four patients with biopsy-proven ocular MMP seen at the Pemphigoid Clinic at Wilmer Eye Institute from July 1984 through November 2006.

Transformation of cutaneous gamma/delta T-cell lymphoma following 15 years of indolent behavior.

Subcutaneous gamma/delta (gamma/delta) T-cell lymphoma is a rare lymphoma, characterized by its unique immunophenotype and clinical course. It has been shown to behave more aggressively than its counterpart bearing the alpha/beta receptor and has recently been removed from the subcutaneous panniculitis-like T-cell lymphoma category for this very reason. We present a case of a patient with a 15-year running diagnosis of panniculitis.

Rituximab therapy in severe juvenile pemphigus vulgaris.

Juvenile pemphigus vulgaris (PV) is a rare and often misdiagnosed condition. Although PV frequently is severe in children, a substantial portion of the morbidity and mortality associated with juvenile PV has been attributed to treatment. This report demonstrates the efficacy of rituximab therapy in juvenile PV.

Adalimumab therapy for recalcitrant pyoderma gangrenosum.

Objective: To describe a patient with treatment-refractory pyoderma gangrenosum and the outcome of a novel therapeutic approach.

Methods: Case report and review of the literature.

Results: A patient with inflammatory bowel disease developed severe pyoderma gangrenosum while receiving treatment with the chimeric anti-TNF-alpha antibody infliximab.

Role of electron microscopy in the diagnosis of ocular mucous membrane pemphigoid.

Purpose: To evaluate the role of electron microscopy (EM) for the diagnosis of ocular mucous membrane pemphigoid (MMP) among patients with cicatrizing conjunctivitis.

Design: Retrospective case series.

Participants: One hundred twenty-eight patients with cicatrizing conjunctivitis referred for the evaluation of possible ocular MMP between January 1985 and February 2002 who underwent conjunctival biopsy and evaluation with EM and direct immunofluorescent (DIF) techniques.

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.

Mucous membrane pemphigoid of the vulva.

Background: Mucous membrane pemphigoid is a rare autoimmune blistering disease primarily affecting mucosal surfaces. Blistering and scarring may occur in the eyes, mouth, esophagus, larynx, and on the vulva. Scarring can result in severe structural changes to the vulva that may mimic the findings of other inflammatory dermatologic disorders of the vulva, including lichen sclerosus and lichen planus.

Mucosal dominant pemphigus vulgaris with anti-desmoplakin autoantibodies.

Background: Anti-desmoplakin (DP) antibodies are present in paraneoplastic pemphigus (PNP) as a component of a complex humoral autoimmune reaction characterized by antibodies against proteins of the plakin family, desmogleins, and an unidentified 170 kd protein. Anti-DP antibodies have also been rarely identified in other blistering diseases. The significance of anti-DP antibodies in the pathogenesis of bullous diseases is unclear.

Neoplasms associated with paraneoplastic pemphigus: a review with emphasis on non-hematologic malignancy and oral mucosal manifestations.

The review included 163 cases of paraneoplastic pemphigus (PNP) reported between 1990 and 2003, including a new unique case of PNP associated with occult breast cancer and an ovarian cyst of borderline malignancy. Hematologic-related neoplasms or disorders were associated with 84% of the cases, with non-Hodgkin lymphoma (38.6%) as the most frequent, followed by chronic lymphocytic leukemia (18.

Mucous membrane pemphigoid and pseudopemphigoid.

Purpose: To describe the clinical characteristics of patients with mucous membrane pemphigoid (MMP) and pseudopemphigoid.

Design: Retrospective cohort study.

Participants: Two hundred eighty consecutive patients referred for the evaluation of possible ocular MMP from January 1, 1985, to December 31, 2001.

Differences and similarities among expert opinions on the diagnosis and treatment of pemphigus vulgaris.

Background: As a result of a lack of large-scale controlled studies, the diagnosis and management of pemphigus vulgaris (PV) has been solely on the basis of expert opinion, rather than on empirical evidence. We have completed a survey of worldwide experts on the diagnostic and therapeutic approaches to PV.

Methods: We conducted a telephone-based survey of 24 physicians from academic, tertiary care centers worldwide with an average of 20 years experience treating pemphigus.

Using rituximab (anti-CD20 antibody) in a patient with paraneoplastic pemphigus.

Paraneoplastic pemphaigus (PNP) is a rare autoimmune mucocutaneous blistering disease that is commonly associated with underlying B-cell neoplasms. There is no standard therapy for PNP. Potent immunosuppression has been the only potentially effective treatment in the setting of malignancy because there is no correlation between tumor burden and activity of disease.

Vesicular pemphigoid in a 16-year-old boy.

We describe a case of a 16-year-old African-American boy with bullous pemphigoid (BP), an acquired autoimmune blistering disease that is rarely seen in children. The patient's lesions, however, were distinctly herpetiform, complicating initial diagnosis and therapy. A diagnosis of BP was made by direct and indirect immunofluorescence.