though paraneoplastic pemphigus ( pnp ) and myasthenia gravis are rare , but can be associated with castleman 's disease .
its association with various autoimmune diseases is well known and hence presentation can vary . this case report is an attempt to create awareness among us not to neglect a common symptom like oral ulcers which are resistant to treatment .
a 25-year - old lady with no significant medical history including allergic diseases such as bronchial asthma was admitted to a tertiary care hospital of south india because of recalcitrant erosive lesions on her oral mucosa and lip that had begun 3 months earlier .
the patient was treated for steven johnson syndrome in a private hospital with steroids prior to the admission . on admission
, there were extensive erosions in the oral mucosa and tongue with thick hemorrhagic crusts on the lip [ figure 1 ] and erosive lesions over labia majora .
histological examination of a biopsy taken from a violaceous , indurated lesion of the lower lip showed a band - like infiltrate of lymphocytes and histiocytes throughout the papillary dermis , hydropic degeneration of basal keratinocytes , and necrotic keratinocytes within the hypertrophic epidermis .
skin biopsy was suggestive of interface dermatitis suggestive of pemphigus vulgaris [ figure 2 ] .
biopsy from un - involved buccal mucosa for direct immunofluorescence was negative for antibodies . during hospital
workup for respiratory weakness with limb weakness with preserved deep tendon reflexes was done with neostigmine test .
positive response to the test was suggestive of myasthenia gravis ; hence , pyridostigmine and steroids were started with which she was off ventilator in next 48 hours
. ultrasound abdomen and subsequently computed tomography ( ct ) imaging of the abdomen done revealed a solid tumor of 8.5  4  5.5 cm of size in right paraspinal area near the hilum of right kidney [ figure 3 ] .
mild hyperinflation without infiltrates was visible on chest radiographs , but conventional ct of the chest showed no remarkable abnormalities .
she underwent surgery for the retroperitoneal tumor and highly vascular tumor of 8  6  5 cm was removed without any intraoperative and post - operative complications .
histopathology revealed castleman 's disease of hyaline vascular variety [ figures 4 - 6 ] .
skin lesions improved at the end of 2 weeks post - operatively [ figure 7 ] and she was discharged with an advise to tapper her steroids and to continue pyridostigmine for myasthenia gravis . crusted and exfoliating dark brown velvety lesions on the oral cavity skin biopsy showing interface dermatitis suggestive of pemphigus vulgaris cect abdomen showing paraspinal mass lesion of 8.5  4  5.5 cm gross specimen showing cut surface of lymph node with pale brown homogenous appearance with intact capsule microphotograph showing pale follicle with concentric onion peel - like lymphocytes and a follicle traversed by a hyalinized vessel microphotograph showing pale follicle with concentric onion peel - like lymphocytes and a follicle traversed by a hyalinized vessel post - operative picture showing marked resolution of the lesions
three histopathological variants are hyaline vascular variant , plasma cell variant , and mixed variants .
thoracic site is most common constituting 70% cases ; among them around 80% is in mediastinum .
other common associations are hiv , kaposi sarcoma , non - hodgkin lymphoma , and polyneuropathy , organomegaly , endocrinopathy , monoclonal gammopathy , and skin changes syndrome .
paraneoplastic pemphigus ( pnp ) , an autoimmune syndrome that was first described by anhalt et al .
in 1990 , are mostly associated with lymphoproliferative neoplasms , i.e. , non - hodgkin 's lymphoma , chronic lymphocytic leukemia , castleman 's disease and , less commonly , thymoma and retroperitoneal sarcomas .
presence of intractable stomatitis and anti - plakin protein autoantibodies is a constant feature ; however , there have been reports of antibody negative pnp in literature .
progression of disease and death occurs frequently despite treatment of the associated neoplasm and regardless of therapeutic intervention for the autoimmune disease . even after complete resection of castleman 's disease
any recurrence of oral lesions or rise in antibody levels would signal recurrence or new lesions of castleman 's disease .
a study of 28 patients presenting with stomatitis with lichenoid skin lesions who later on were found to have castleman 's disease on follow - up showed fatality among 22 patients over a period of 2 years secondary to respiratory failure .
post - mortem revealed evidence of progressive bronchiolitis obliterans in those cases indicating progressive autoimmune damage to the lung .
treatment with anti - il-6 has been shown to improve platelet counts among patients with autoimmune thrombocytopenia with castleman 's disease ; however , there has been no study among patients with pnp . in our patient ,
diagnosis of myasthenia was made on the basis of neostigmine test where 0.04 mg / kg is given intramuscularly or 0.02 mg / kg intravenously ( one time only ) observing for clinical improvement of weakness .
 our patient had a rare combination of pnp with extrathoracic castleman 's disease with myasthenia gravis .
castleman 's disease being hyaline vascular variety presenting with systemic manifestation is also a rare presentation . on follow - up ,
she developed progressive hypoxia and succumbed to ventricular arrhythmia at the end of 1 year .
castleman 's disease , though described as benign entity , its varied presentation with myasthenia , pnp , and progressive lung damage resulting in bronchiolitis obliterans secondary to autoimmune injury makes it lethal .
unicentric hyaline vascular variety with systemic manifestations behaves similar to multicentric and hence is associated with bad prognosis as in our case .
any non - healing oral ulcers with or without skin lesions should alert the treating physician for extensive workup and to intervene at the right time .
presentation of unicentric hyaline vascular variant castleman 's disease with systemic manifestations like paraneoplastic pemphigus ( pnp ) and myasthenia is uncommon .
 castleman 's disease is a rare autoimmune disorder with varied clinical presentations . 
 castleman 's commonly involves mediastinum and hence it is thoracic in most of the reported cases . 
 paraneoplastic pemphigus ( pnp ) and myasthenia gravis can be associated with multicentric castleman 's disease . 
 its association with hiv , kaposi sarcoma , and lymphoma is also well known . 
 we report a rare combination of unicentric , extrathoracic castleman 's disease with pnp and myasthenia gravis . 
Introduction
Case Report
Discussion
Conclusion