Idiopathic Multicentric Castleman Disease with Severe Paraneoplastic Syndromes
Poster #: 203
Session/Time: B
Author:
Saif Fiaz, DO, MS
Mentor:
Waleed Kassabo, MD
Research Type: Case Report
Abstract
INTRODUCTION:
Castleman Disease (CD) is a rare constellation of diseases that has evolved in its definition since its first description in the 1950s by Benjamin Castleman. With broad presentation and low incidence of about 5,000 cases yearly, diagnosis can be delayed. CD consists of 4 main subtypes including unicentric CD (UCD), polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder and skin changes syndrome associated with MCD (POEMS-MCD), HHV8 associated MCD, and idiopathic MCD(iMCD). In this case we describe a severe case of iMCD with severe paraneoplastic syndromes in a young patient to include pemphigus vulgaris and eosinophilic pneumonia.
CASE INFORMATION:
A 22-year-old male with a past medical history of type 1 diabetes mellitus (T1DM) presented to outpatient dermatology with chief complaints of progressive odynophagia, dysphagia, genital pain, and diffuse pruritic eruptions of the trunk of one month. Initial outpatient workup revealed spongiform dermatitis and colonization with methicillin sensitive staph aureus(MRSA). The patient was started with topical treatments to include oral doxycycline, topical mupirocin, topical triamcinolone, oral fluconazole. Despite therapy, the patient's clinical picture worsened over the next 6 months for which he was sent to the hospital for workup. Pertinent negative workup included HIV, HBV, RPR titer, VZV, Rheumatoid factor, anti-nuclear antibody, P-ANCA, dsDNA. Pertinent positives include IgE, IL-6 levels, thrombocytosis, elevated ESR/CRP. Direct immunofluorescence of repeat skin biopsy revealed pemphigus vulgaris. Computed tomography of neck, chest, abdomen, pelvis revealed epiglottitis with associated lymphadenopathy, apical ground glass opacification of bilateral lungs, and large pelvic mass initially believed to be possibly prostatic rhabdomyosarcoma. The patient was treated symptomatically for epiglottitis, underwent bronchoscopy of lungs revealing findings most consistent with eosinophilic pneumonia. 2 CT-guided biopsies of intraabdominal mass were performed without specific findings, FISH and EBV testing were negative and revealed non-specific follicular hyperplasia. Ultimately the patient underwent intrapelvic mass resection with pathology revealing polytypic plasma cells with follicular and paracortical hyperplasia most consistent with diagnosis of CD. Ultimately the patient was diagnosed with HIV negative and HHV8 negative iMCD not otherwise specified (NOS) with concurrent paraneoplastic pemphigus vulgaris and paraneoplastic eosinophilic pneumonia. The patient was started on the first infusion of weekly rituximab and pending full course treatment.
DISCUSSION/CONCLUSION:
In this case we describe a severe presentation of a rare disease. Unique and highlighting features of this case include paraneoplastic pemphigus vulgaris, eosinophilic pneumonia, age at diagnosis, extent of pelvic mass, and absence of underlying identifiable cause. MCD presents on average in those in their 6th decade of life and is often associated with HHV8 and HIV. It also has several associated paraneoplastic syndromes associated with the disease, which include: pemphigus vulgaris, eosinophilic pneumonia, thrombocytopenia, bone marrow fibrosis, and polyneuropathy. The etiology is believed to be due to clonal neoplastic disease associated with lymph node stromal dendritic cells. 5 year overall survival with treatment of iMCD is 100%. Recovery in this case will likely be complicated by the extensive involvement of his skin and mucosal lesions. Treatment response is currently pending evaluation, and the clinical course is being tracked.
Castleman Disease (CD) is a rare constellation of diseases that has evolved in its definition since its first description in the 1950s by Benjamin Castleman. With broad presentation and low incidence of about 5,000 cases yearly, diagnosis can be delayed. CD consists of 4 main subtypes including unicentric CD (UCD), polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder and skin changes syndrome associated with MCD (POEMS-MCD), HHV8 associated MCD, and idiopathic MCD(iMCD). In this case we describe a severe case of iMCD with severe paraneoplastic syndromes in a young patient to include pemphigus vulgaris and eosinophilic pneumonia.
CASE INFORMATION:
A 22-year-old male with a past medical history of type 1 diabetes mellitus (T1DM) presented to outpatient dermatology with chief complaints of progressive odynophagia, dysphagia, genital pain, and diffuse pruritic eruptions of the trunk of one month. Initial outpatient workup revealed spongiform dermatitis and colonization with methicillin sensitive staph aureus(MRSA). The patient was started with topical treatments to include oral doxycycline, topical mupirocin, topical triamcinolone, oral fluconazole. Despite therapy, the patient's clinical picture worsened over the next 6 months for which he was sent to the hospital for workup. Pertinent negative workup included HIV, HBV, RPR titer, VZV, Rheumatoid factor, anti-nuclear antibody, P-ANCA, dsDNA. Pertinent positives include IgE, IL-6 levels, thrombocytosis, elevated ESR/CRP. Direct immunofluorescence of repeat skin biopsy revealed pemphigus vulgaris. Computed tomography of neck, chest, abdomen, pelvis revealed epiglottitis with associated lymphadenopathy, apical ground glass opacification of bilateral lungs, and large pelvic mass initially believed to be possibly prostatic rhabdomyosarcoma. The patient was treated symptomatically for epiglottitis, underwent bronchoscopy of lungs revealing findings most consistent with eosinophilic pneumonia. 2 CT-guided biopsies of intraabdominal mass were performed without specific findings, FISH and EBV testing were negative and revealed non-specific follicular hyperplasia. Ultimately the patient underwent intrapelvic mass resection with pathology revealing polytypic plasma cells with follicular and paracortical hyperplasia most consistent with diagnosis of CD. Ultimately the patient was diagnosed with HIV negative and HHV8 negative iMCD not otherwise specified (NOS) with concurrent paraneoplastic pemphigus vulgaris and paraneoplastic eosinophilic pneumonia. The patient was started on the first infusion of weekly rituximab and pending full course treatment.
DISCUSSION/CONCLUSION:
In this case we describe a severe presentation of a rare disease. Unique and highlighting features of this case include paraneoplastic pemphigus vulgaris, eosinophilic pneumonia, age at diagnosis, extent of pelvic mass, and absence of underlying identifiable cause. MCD presents on average in those in their 6th decade of life and is often associated with HHV8 and HIV. It also has several associated paraneoplastic syndromes associated with the disease, which include: pemphigus vulgaris, eosinophilic pneumonia, thrombocytopenia, bone marrow fibrosis, and polyneuropathy. The etiology is believed to be due to clonal neoplastic disease associated with lymph node stromal dendritic cells. 5 year overall survival with treatment of iMCD is 100%. Recovery in this case will likely be complicated by the extensive involvement of his skin and mucosal lesions. Treatment response is currently pending evaluation, and the clinical course is being tracked.