Recognizing Kaposi’s Sarcoma In The Lower Extremity

Given the varying clinical presentations of Kaposi’s sarcoma, these authors offer pertinent insights in diagnosing these lesions in the foot.

Kaposi’s sarcoma is an angioproliferative disorder characterized by infection with human herpesvirus (HHV-8). The disease is multifocal and can range from indolent to aggressive with visceral involvement. There are four types of the disease based on clinical presentation. They include classic, endemic, iatrogenic and AIDS-associated Kaposi’s sarcoma.¹

Classic. This occurs in Mediterranean or Eastern European patients, and commonly involves the lower extremities. It is rarely aggressive and rarely involves visceral organs.

Endemic. This arises in patients of African descent and can be aggressive in children. Internal organ involvement may be present in adult patients and is common in children.

Iatrogenic. This occurs secondary to exogenous immunosuppression. It affects the distal lower extremities with common visceral involvement. This can be aggressive but can regress with modification of exogenous immunosuppression.

AIDS-associated. This type is localized or disseminated with visceral involvement common in the setting of poor human immunodeficiency virus (HIV) control. It may regress with optimal HIV treatment.

The clinical presentation can vary based on the type of Kaposi’s sarcoma. General symptoms include fever, fatigue, and non-tender skin lesions mainly on the lower extremity, face and neck. The lesions can be of varying sizes and plaque-like, papular or nodular with violaceous pigmentation. Lymphedema is often present and can be severe. If visceral involvement is present, symptoms and signs will be organ specific.²

Differential diagnoses for Kaposi’s sarcoma include hemangioma, molluscum contagiosum, angiosarcoma, angioma, hematoma, dermatofibroma, pyogenic granuloma, and bacterial, viral and fungal infections.²

Definitive diagnosis is based on tissue biopsy and the histopathological evaluation should reveal leukocytic infiltration and neovascularization with proliferation of small lymph or blood vessels, and spindle-shaped cells.

The workup for Kaposi’s sarcoma should initially involve a thorough physical examination. One should perform a biopsy of suspicious lesions and send it for histopathological examination. Clinicians should gear diagnostic tests toward assessing HIV status, determining visceral involvement and evaluating for immunodeficiency.

Treatment can be local or systemic, and often depends on the type and aggressiveness of the Kaposi’s sarcoma.³ Local treatments aim at destruction of skin lesions and can include cryotherapy, laser treatment, radiation therapy, chemotherapy and edema control. Physicians often utilize systemic treatments for patients with aggressive Kaposi’s sarcoma and these treatments may include chemotherapeutic agents such as doxorubicin and highly active antiretroviral therapy (HAART) for AIDS-associated Kaposi’s sarcoma.⁴

Case Study: When An Older Patient Presents With Hypercalcemia And Well-Circumscribed, Non-Draining Lesions Of Varying Sizes

An 81-year-old male with a history of hypercalcemia and lymphadenopathy presented for hospital admission with hypercalcemia and fever. There was a subsequent podiatry consult to evaluate bilateral foot and ankle lesions. The lesions were well-circumscribed macules and plaques of varying sizes and stages, were red to purple in color, and present along the left plantar lateral forefoot, bilateral medial heel and right plantar arch.

The lesions did not exhibit any drainage, fluctuance or signs of infection. Pedal pulses were palpable bilaterally. Pitting edema (+2) was present on the left lower extremity and trace edema was present to the right lower extremity. Gross sensation was intact bilaterally and the lesions were tender to palpation. The patient was non-ambulatory secondary to generalized weakness due to hypercalcemia. His vitals were stable except for febrile episodes with a maximum temperature of 103.3ºF.

The patient’s past medical history was notable for hypercalcemia, lymphadenopathy, hypertension, traumatic brain injury, abdominal aortic aneurysm, urinary tract infection, cerebrovascular accident, subdural hematoma and possible sarcoidosis. The patient’s social history revealed former tobacco use. His family history was unremarkable.

The initial workup included a complete blood count with differential, complete metabolic panel, rapid plasma reagin, hepatitis panel, HIV antigen/antibody, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), immunoelectrophoresis, computed tomography (CT) scan of the chest/abdomen/pelvis, bilateral foot radiographs and biopsies of the lesions. After the use of local anesthesia for these areas, we took 3 mm punch biopsies from the left medial heel lesion and right plantar arch lesion. The diagnostic workup also included inguinal lymph node biopsies with an immunohistochemistry assay including HHV-8.

The results included negative HIV antigen antibody testing, multifactorial anemia, thrombocytopenia, an ESR reading of 23, and a CRP reading of 5. Bilateral radiographs of the feet were unremarkable. The CT chest/abdomen/pelvis scan revealed enlarged left subpectoral, bilateral axillary, mediastinal, hilar, paracardiac, hepatogastric, porta hepatis, perisplenic, retroperitoneal, left iliac, internal iliac and external iliac lymph nodes. Punch biopsy results of the feet were remarkable for Kaposi’s sarcoma bilaterally. The inguinal lymph node analysis was negative for HHV-8.

The diagnosis was Kaposi’s sarcoma without strong evidence of immunodeficiency. Further analysis of laboratory and clinical findings resulted in a diagnosis of Castleman disease with associated Kaposi’s sarcoma.

Case Study: When A Patient Presents With A Unilateral Draining Rash Of Six Months In Duration On The Left Lower Extremity

A 41-year-old male with no significant medical history presented with a unilateral nodular, draining rash along the left lower extremity that he had for six months. Nodular lesions extended from the ankle to the thigh. The patient reported serosanguineous drainage but denied purulent drainage. He noted that the entire area was tender to palpation.

The lesions were nodular with an erythematous base, red to purple in color and of varying stages. The left lower extremity was edematous in comparison to the right lower extremity. Pedal pulses were palpable bilaterally. Gross protective sensation was intact bilaterally. There appeared to be no restriction to range of motion.

The patient reported no past medical history. He related recent travel to Trinidad and the Cayman Islands. He reported current tobacco, alcohol and marijuana use. His family history was unremarkable. The patient’s vital signs were stable for the duration of the admission. We initially placed him on vancomycin secondary to concern for a possible infectious process.

Initial testing included complete blood count with differential, complete metabolic panel, rapid plasma reagin, HIV antigen/antibody, ESR, CRP, immunoelectrophoresis, and wound cultures taken from the draining lesion. We took a 4 mm punch biopsy of one of the left leg lesions. Laboratory findings included leukopenia, macrocytic anemia, an ESR reading of 15 and a CRP reading of 1.7. The patient was HIV positive and the biopsy results revealed papulonodular Kaposi’s sarcoma.

In Conclusion

Given the variability of clinical presentation, Kaposi’s sarcoma can be difficult to diagnose. The aforementioned cases involved patients of differing demographics and medical history. In these two cases, the clinical appearance of the cutaneous lesions differed and presented in different anatomic locations and distributions. Given the uncertainty of clinical presentation, biopsy of cutaneous lesions is of significant importance. After making a diagnosis, refer patients to the appropriate specialty (oncology, infectious disease, dermatology) for further workup and treatment.

Dr. Cornell is a Diplomate of the American Board of Foot and Ankle Surgery. She is fellowship trained in diabetic limb salvage and currently in private practice in Havertown, Pa. She is an attending physician with the Crozer-Keystone Health System Podiatric Residency program.

Dr. Patel is a second-year resident within the Division of Podiatric Surgery at Crozer-Keystone Health System in Pennsylvania. 

References

1. Martin JN, Ganem DE, Osmond DH, et al. Sexual transmission and the natural history of human herpesvirus 8 infection. N Engl J Med. 1998; 338(14):948-54.

2. Schwartz RA. Kaposi's sarcoma: an update. J Surg Oncol. 2004; 87(3):146-51.

3. Von Roenn JH, Cianfrocca M. Treatment of Kaposi's sarcoma. Cancer Treat Res. 2001; 104:127.

4. Castiñeiras I, Almagro M, Rodríguez-Lozano J, et al. Disseminated classic Kaposi's sarcoma. Two cases with excellent response to pegylated liposomal doxorubicin. J Dermatolog Treat. 2006; 17(6):377-80.