ABSTRACT
BACKGROUND: Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease associated with lymphoproliferative neoplasms, and frequently with a very rare tumour, Castleman's disease. OBJECTIVES: To analyse the clinical history, immunopathological and histopathological findings in 28 patients with a confirmed diagnosis of PNP and Castleman's disease. METHODS: Sera from all patients were assayed by indirect immunofluorescence (IF) and immunoprecipitation (IP) for plakin autoantibodies, immunoblotting for detection of plectin autoantibodies, and enzyme-linked immunosorbent assay for detection of desmoglein (Dsg)1 and Dsg3 autoantibodies. RESULTS: Severe oral mucositis was observed in all patients, and lichenoid cutaneous lesions were seen in 19 of 28. Twenty cases of Castleman's disease were of the hyaline vascular type, four were of plasmacytoid type and four were of mixed type. Striking findings included pulmonary destruction leading to bronchiolitis obliterans in 26 patients and fatal outcome due to respiratory failure in 22 patients with pulmonary involvement. Histological findings included lichenoid and interface dermatitis with variable intraepithelial acantholysis. Direct IF showed deposition of IgG and C3 in the mouth and skin in 24 of 28 patients. However, indirect IF detected serum IgG autoantibodies in all patients. IP revealed IgG autoantibodies against desmoplakin I, envoplakin and periplakin in all cases, and against desmoplakin II and the 170-kDa antigen in 19 patients. Dsg3 and Dsg1 autoantibodies were present in 22 and 11 patients, respectively, and plectin autoantibodies in 23 patients. CONCLUSIONS: PNP in association with Castleman's disease presents with severe oral mucositis and cutaneous lichenoid lesions. Serum autoantibodies against plakin proteins are the most diagnostic markers. Pulmonary injury with respiratory failure is the cause of death in most cases.
Subject(s)
Castleman Disease/complications , Paraneoplastic Syndromes/complications , Pemphigus/complications , Adolescent , Adult , Aged , Antibodies, Anti-Idiotypic/analysis , Autoantibodies/analysis , Autoantibodies/blood , Cadherins/immunology , Castleman Disease/immunology , Child , Complement C3/immunology , Cytoskeletal Proteins/analysis , Cytoskeletal Proteins/blood , Female , Fluorescent Antibody Technique, Indirect , Humans , Immunoglobulin G/immunology , Male , Middle Aged , Mouth Mucosa/immunology , Mouth Mucosa/pathology , Paraneoplastic Syndromes/immunology , Pemphigus/immunology , Precipitin Tests , Protein Serine-Threonine Kinases/immunology , Skin/immunology , Skin/pathologyABSTRACT
Henoch-Schönlein purpura (HSP) is an IgA-mediated small vessel vasculitis which commonly involves the skin, gastrointestinal system and kidneys. Numerous HSP triggers have been identified, and pregnancy has been reported as an exacerbating factor. After a pregnant woman had been diagnosed as having new-onset HSP, we reviewed all cases of immunofluorescence-proven HSP evaluated by the Department of Dermatology at the Johns Hopkins Hospital between 1990 and 2002, and report three cases of HSP occurring during pregnancy. Two patients developed new-onset HSP, one at 16 weeks gestation and one at 22 weeks, while the third developed a recurrence of HSP at 12 weeks gestation after 19 years of remission. We conclude that pregnancy may be a trigger for HSP onset or recurrence in susceptible individuals.
Subject(s)
IgA Vasculitis/diagnosis , Pregnancy Complications, Hematologic/diagnosis , Adult , Female , Fluorescent Antibody Technique, Direct , Humans , IgA Vasculitis/immunology , Immunoglobulin A/analysis , Pregnancy , Pregnancy Complications, Hematologic/immunology , Pregnancy Trimester, Second , Retrospective Studies , Skin/immunologyABSTRACT
Cardiac conduction disease is an infrequent complication of Wegener's granulomatosis (WG). We describe a case demonstrating an association between WG and complete atrioventricular dissociation. This manifestation was initially interpreted as Lyme disease based on these cardiac findings, arthritis, myalgias and positive Lyme serology. The clinical overlap between these disorders and the appropriate use of respective serologies is discussed.
