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Inguinal lymphadenopathy as the initial presentation of sarcoidosis.


A 50-year-old African American woman with iron deficiency anemia secondary to menorrhagia and uterine fibroids was admitted for symptomatic anemia, requiring blood transfusions. The patient reported worsening right groin swelling and right leg swelling over the last 10 years with progressive pain with activity. A brother had lymphoma.

On admission, she was afebrile and normotensive. Her right leg was 1.5 times larger in circumference than her left leg with nonpitting edema and a negative Homan's sign. A 7 x 4 cm nodular mass was palpable in her right groin. There was no associated erythema or tenderness to palpation. Her white blood cell count was 4.6 K/uL, hemoglobin 6.5 g/dL, and platelets 331 K/uL. She had a positive antineutrophil antibody result with a 1:320 titer, an angiotensin-converting enzyme level of 25 mg/L, a vitamin D level of 52.9 ng/mL, an erythrocyte sedimentation rate of 47 mm/hr, a C-reactive protein level of 4.8 mg/ dL, a complement 3 (C3) level of 147 g/L, and a complement 4 (C4) level of 48.4 g/L. A computed tomography (CT) scan of the chest, abdomen, and pelvis revealed right deep pelvic and inguinal bulky adenopathy, with an 8.1 x 5.3 cm right pelvic sidewall mass, multiple enlarged right inguinal lymph nodes measuring 6 cm, and a large left uterine mass, measuring 8 cm (Figure 1). No abnormalities were noted in the chest or abdomen. A dilatation and curettage with endometrial biopsy was completed. An ultrasound-guided core needle biopsy was performed to further evaluate her inguinal mass. Her endometrial biopsy showed numerous myometrial tissue fragments and acute and chronic inflammation with squamous metaplasia, all suggestive of underlying submucosal leiomyoma and atrophy. The tissue sample was negative for granulomas, atypia, or malignancy. The ultrasound-guided core needle biopsy of the large right inguinal lymph node showed noncaseating granulomatous inflammation with special stains negative for acid-fast bacilli and fungi (Figure 2). Flow cytometric analysis showed no evidence of a malignant hematolymphoid process.



The patient was started on prednisone 40 mg daily and discharged home. She experienced an initial yet short-lived improvement in her right groin and lower extremity swelling. Attempts to taper the prednisone dose below 20 mg daily resulted in worsening right lower leg lymphedema. Multiple ultrasounds of her right lower extremity were done to rule out deep venous thrombosis, all of which were negative.

A repeat CT scan of her chest, abdomen, and pelvis 5 months after her initial diagnosis suggested progressive sarcoidosis with increasing pelvic and inguinal lymphadenopathy, new low-density areas within the spleen, and multiple subcentimeter scattered pulmonary nodules. One month later, she died. The cause of death was not known.


Three main mechanisms of sarcoidosis causing asymmetric peripheral edema have been described. The first is lymphatic obstruction due to peripheral lymphadenopathy. Our patient's edema illustrated this mechanism and, to our knowledge, is only the sixth case reported to date. Of these cases, four (including ours) were described in black patients, one in an Indian patient, and one in an Asian patient (1-5). This distribution is consistent with findings from a US study that demonstrated that extrathoracic lymph node involvement is more common in black patients than in white patients (6). The location of the obstructing lymph nodes was inguinal in three cases and retroperitoneal in two (1-5). In these cases, systemic steroid treatment resulted in prompt resolution of the lymphadenopathy and associated edema within 2 months.

A second mechanism by which sarcoidosis can cause peripheral edema is by direct infiltration into surrounding tissues. Two cases displaying this mechanism have been reported. One involved a 39-year-old black man (7). He had known sarcoidosis with pulmonary and skin involvement and presented with asymmetric lower-extremity edema. A CT scan revealed "diffuse, infiltrative masses involving the subcutaneous tissues of the right leg" (7). His symptoms abated within 1 month following systemic steroids. A second case involved a 59-year-old woman with cutaneous nodules, bilateral lower-extremity edema, and ulceration (8). Her symptoms improved with prednisolone but recurred when doses were decreased below 15 mg.

A third mechanism is tenosynovitis, producing distal peripheral edema. In one case series, five patients presented with lower-extremity pitting edema localized to the dorsal foot and ankle associated with acute sarcoidosis (9). Magnetic resonance imaging showed extensive tenosynovitis. As with previous cases, all responded quickly to systemic steroid treatment.


The authors thank Paul Bannister, MD, from the Department of Pathology at Baylor Medical Center at Garland, for providing histology images.

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(2.) Nathan MP, Pinsker R, Chase PH, Elguezabel A. Sarcoidosis presenting as lymphedema. Arch Dermatol 1974;109(4):543-544.

(3.) Silver HM, Tsangaris NT, Eaton OM. Lymphedema and lymphography in sarcoidosis. Arch Intern Med 1966;117(5):712-714.

(4.) Sweeney T, Ramsby G, Keohane M. Edema of the lower extremities secondary to obstructive sarcoidosis. Angiology 1980;31(1):69-71.

(5.) Mahajan VK, Sharma NL, Sharma RC, Sharma VC. Cutaneous sarcoidosis: clinical profile of 23 Indian patients. Indian J Dermatol Venereol Leprol 2007;73(1):16-21.

(6.) Baughman RP, Teirstein AS, Judson MA, Rossman MD, Yeager H, Brsnitz EA. Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med 2001;164(10):1885-1889.

(7.) Hoover RD Jr, Stricklin G, Curry TW, Carmichael LC. Unilateral lower extremity edema caused by infiltrative sarcoidosis. J Am Acad Dermatol 1994;30(3):498-500.

(8.) Muhlemann MF, Walker NP, Tan LB, Champion RH. Elephantine sarcoidosis presenting as ulcerating lymphoedema. J R Soc Med 1985;78(3):260-261.

(9.) Cantini F, Niccoli L, Olivieri I, Barozzi L, Pavlica P, Bozza A, Macchioni PL, Padula AA, Salvarani C. Remitting distal lower extremity swelling with pitting oedema in acute sarcoidosis. Ann Rheum Dis 1997;56(9):565-566.

Jim George, MD, Reese Graves, MD, and Robert Meador Jr., MD

From the Department of Family Medicine (George, Graves), and the Division of Rheumatology, Department of Internal Medicine (Meador), Baylor Medical Center at Garland, Garland, Texas.

Corresponding author: Jim George, MD, 1009 East Grubb Drive, Mesquite, TX 75149 (e-mail:
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Author:George, Jim; Graves, Reese; Meador, Robert, Jr.
Publication:Baylor University Medical Center Proceedings
Date:Apr 1, 2013
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