castleman 's disease ( cd ) or giant lymph node hyperplasia is a rare disorder first explained by castleman et al . , in 1954
. it can be unicentric or multicentric and mostly involves mediastinum ( 60% ) , retroperitoneum ( 11% ) , and axilla ( 4% ) .
multicentric castleman 's disease ( mcd ) is manifested by generalized lymphadenopathy , hepatosplenomegaly , and polyclonal hypergammaglobulinemia , high erythrocyte sedimentation rate ( esr ) , anemia , thrombocytopenia , and constitutional symptoms .
human herpes virus 8 ( hhv-8 ) infection has been identified in nearly 100% of hiv - positive and 50% of hiv - negative mcd patients .
this virus predisposes patients to much higher risk of other malignancies , including kaposi 's sarcoma ( ks ) ( 13% ) and non - hodgkin 's lymphoma ( 18% ) .
kaposi 's sarcoma is a vascular neoplasm with a compound and unknown histogenesis in which most of the spindle cell lesions express cd31 and cd34 .
castleman 's disease is histologically divided into three types : hyaline vascular type or angiofollicular type , plasma cell ( pc ) type , and mixed type .
co - incidence of mcd with ks and some common features of them led us to report this case .
it is unknown whether ks is a consequence of immunosuppression caused by mcd or ks is a clinical feature of mcd .
a 77-year - old man was admitted to our dermatology clinic with fever , anorexia , weight loss , generalized pruritus , and multiple cutaneous lesions on the legs .
the patient 's complaints started 1 year ago ; however , the legs edema was developed during the recent 6 months .
a physical examination revealed cachexia , pallor , xerosis with excoriation marks , skin lesions , and generalized lymphadenopathy .
the lymph nodes were discrete , mobile , firm , and non - tender ranging in size from 0.5 to 6 cm .
cutaneous lesions were violaceous indurated papules and plaques on the anterior aspects of both legs in the previous site of saphenous vein angioplasty from 3 years ago .
these lesions clinically resembled ks [ figures 1 and 2 ] . additionally , laboratory findings showed anemia ( hb : 9.6 mg / dl ) , increased esr ( 98 mm / h ) , elevated wbc count ( 11100 mm ) with 12% eosinophilia , and also negative viral markers ( hhv-8 , hiv , hcv , hbv ) .
notably , chest ct scan demonstrated extensive bilateral axillary adenopathy , pretracheal and antrosuperior mediastinal adenopathy , subpleural wedge - shaped consolidation on the base of the left lung , and subpleural nodules on the right lung recommended to rule out of lymphoma .
abdominopelvic ultrasound revealed mild hepatosplenomegaly with a hyperechoic solid mass in the mid portion of spleen suggesting hemangioma whereas retroperitoneal lymphadenophathy was not detected . also , bone marrow aspiration was normal .
brown - bluish papules and nodules of kaposi 's sarcoma violaceous indurated lesions on the legs in the previous location of saphenous angioplasty a skin biopsy of the lesions showed hypercellular neoplasm composed of proliferated spindle - shape cells arranged in a whorled and fascicular pattern with slit - like spaces containing red blood cell with extravasation [ figure 3 ] .
an axillary lymph node biopsy demonstrated follicular hyperplasia with marked vascular proliferation , hyalinization , and few tight concentric layers of lymphocytes , arranged in onion skin appearance [ figure 4 ] .
the patient was treated with granulocyte colony - stimulating factor ( g - csf ) for four sessions , alpha interferon 2b ( ifn-2b ) subcutaneously for 5 months and vinblastin weekly .
eventually , patient 's pruritus relieved after 2 months , lymphadenopathy regressed , general condition became good , and also , the patient went into the clinical remission . moreover , radiotherapy within 5 months and ks lesions regressed .
the patient received 30 sessions of irradiation on the skin lesions resulting in complete regression .
proliferation of spindle cells arranged in a whorled and fascicular pattern with slit - like spaces containing red blood cell extravasation ( hematoxylin and eosin40 ) follicular hyperplasia with prominent vascular proliferation , hyalinization and few tight concentric layers of lymphocytes , arranged in onion skin appearance ( hematoxylin and eosin100 )
castleman 's disease is divided into localized and multicentric type and histologically , almost the entire multicentric variants are pc type .
the standard curative treatment of localized cd is surgical excision with a 5-year survival rate of 100% and excellent prognosis .
additionally , corticosteroids , chemotherapic agents and radiation therapy are the other modalities for the treatment of mcd . in 2003 , boller et al . , reported simultaneous of mcd and ks in a 17-year - old patient after treatment with cyclosporine for nephropathy .
in addition , de rosa et al . described a woman affected by mcd and complicated by ks .
they discussed that mcd and ks might be presented in a same immunologically patient or ks was a consequence of immunosuppression .
our patient shared many similarities to the previous literature reports and demonstrated mcd findings such as fever , anorexia , weight loss , peripheral and mediastinal lymphadenopathy , and also hepatosplenomegaly .
he had simultaneously violaceous papules and plaques over both legs and the histopathology findings were consistent with co - existence of mcd and ks .
notably , mcd has been reported in adults usually before age 30 and shows an association with hhv-8 and hiv infections .
in contrast , our patient was a 77-year - old man and compared with the prior literature findings , he had hhv-8 and hiv - negative serology .
co - incidence of mcd with ks in our case report can be interpreted that mcd may be complicated by ks or the latter is a manifestation of mcd .
finally , mcd should be kept in mind as a differential diagnosis in a patient with ks .
mcd should be kept in mind as a differential diagnosis in a patient with ks .
 castleman 's disease ( cd ) or giant lymph node hyperplasia is a rare disorder that can be unicentric or multicentric . 
 multicentric castleman 's disease ( mcd ) is manifested by generalized lymphadenopathy , hepatosplenomegaly , polyclonal hypergammaglobulinemia , hematological abnormality , and constitutional symptoms . 
 human herpesvirus 8 ( hhv-8 ) infection is present in nearly 100% mcd associated with hiv-1 infection , but in about 50% of cases of hiv negative . herein , we report a 77-year - old man with systemic involvement and skin lesions on the anterior aspect of both legs in the previous site of saphenous vein angioplasty . 
 co - existence of mcd with kaposi 's sarcoma ( ks ) led us to present this rare case . 
Introduction
Case Report
Discussion