Correct!
5. More than one of the above
Castleman disease may be associated with various paraneoplastic syndromes and hence further investigation is warranted, including testing for autoimmune antibodies, particularly in light of some of the patient’s complaints that are seemingly unrelated to the pelvic mass. Repeat dermatologic consultation to establish an etiology for the oral and genital ulcers is appropriate. Pulmonary function testing is reasonable given the patient’s complaint of dyspnea on exertion and the rare association of Castleman disease with obstructive airway disease. However, bronchoscopic intervention is premature at this point.
The patient was seen by a dermatologist who again noted the ulcerating lesions on the patient’s tongue and labia. Biopsy of the labia was performed- the results showed slight acanthosis with spongiosis and eosinophils within the epidermis, as well as a lymphohistiocytic infiltrate in the papillary epidermis with a vacuolar interface reaction and occasional eosinophils and plasma cells. The results were interpreted as consistent with paraneoplastic pemphigus. Autoantibody testing showed primate esophagus IgG antibody positive (normal, negative), basement membrane IgG antibody negative, cell surface antibody IgG positive at 1:320 (normal, negative), primate split skin IgG negative, bullous pemphigoid 180 S = 5.06 U (normal, <9 U), bullous pemphigoid 230 S = 5.28 (normal, <9 U), desmoglein 1= 2.51 U (normal, <14 U), and desmoglein = 2.32 U (normal, <9 U). A repeat complete blood count and serum chemistries were within normal limits, and a urinalysis was unremarkable. Serum vascular endothelial growth factor measurement = 31 pg/mL (normal, 31 – 86 pg/mL).
Which of the following represents the most appropriate next step for the patient’s management? (click on the correct answer to be directed to the eighth of twelve pages)