Dermatologic Manifestations of Kaposi Sarcoma Clinical Presentation

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Feb 22, 2012
 

History

Kaposi sarcoma (KS) is a neoplasm that often manifests with multiple vascular nodules in the skin and other organs.

Although true metastases appear to occur, a multifocal origin is most common.

The pattern of Kaposi sarcoma is variable, with a course ranging from indolent (only skin manifestations) to fulminant (extensive visceral involvement).

Kaposi sarcoma also may arise primarily in the oral mucosa, lymph nodes, and/or viscera without skin involvement. See the image below.

Oral Kaposi sarcoma. Oral Kaposi sarcoma.

Kaposi sarcoma initially may be evident in any organ of the body.

Chronic lymphedema may precede Kaposi sarcoma.

Professor Ruocco's term isotopic response applies to the occurrence of Kaposi sarcoma with new cutaneous nodules at the site of other, unrelated, and already healed skin diseases.[29]

Although primary penile Kaposi sarcoma is uncommon in HIV negative men, one should consider this possibility when treating nonspecific penile lesions.[30] A minimal penile lesion with nondistinctive clinical features may be the exclusive manifestation of Kaposi sarcoma. In addition, it may appear as a skin-colored nodule suggestive of a primary squamous cell carcinoma.[31] Clearly, in both cases, histologic evaluation is mandated to establish the diagnosis.

Vitiligo and other autoimmune disorders are sometimes evident in patients with AIDS Kaposi sarcoma. Rarely, vitiligo has been observed developing around nodules of Kaposi sarcoma.[32]

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Physical

Kaposi sarcoma (KS) is described in 3 forms including localized nodular, locally aggressive, and generalized Kaposi sarcoma. Kaposi sarcoma typically occurs in these 3 forms and in 6 stages including patch, plaque, nodular, exophytic, infiltrative, and lymphadenopathic.

Cutaneous Kaposi sarcoma usually begins as discrete red or purple patches that are bilaterally symmetric and initially tend to involve the lower extremities.

Patches become elevated, evolving into nodules and plaques.

Nodules may be spongy to the touch. See the images below.

Man who is homosexual and has HIV infection and KaMan who is homosexual and has HIV infection and Kaposi sarcoma.Man who is homosexual and has HIV infection and KaMan who is homosexual and has HIV infection and Kaposi sarcoma. Man who is homosexual and has HIV infection and KaMan who is homosexual and has HIV infection and Kaposi sarcoma. Man who is homosexual and has HIV infection and KaMan who is homosexual and has HIV infection and Kaposi sarcoma. Man who is homosexual and has HIV infection and KaMan who is homosexual and has HIV infection and Kaposi sarcoma. Man who is homosexual and has HIV infection and KaMan who is homosexual and has HIV infection and Kaposi sarcoma.

Kaposi sarcoma also occurs as a large infiltrating mass or as multiple cone-shaped friable tumors. These 2 variants, termed locally aggressive Kaposi sarcoma, may adhere firmly to underlying anatomic structures including bone.

See the images below.

A 35-year-old man with dome-shaped locally aggressA 35-year-old man with dome-shaped locally aggressive tumors, an example of exophytic Kaposi sarcoma with cavernous hemangiomalike histology. Elderly man of Mediterranean lineage with hyperkerElderly man of Mediterranean lineage with hyperkeratotic nodule of Kaposi sarcoma on sole of foot.

Early Kaposi sarcoma may appear as violaceous patches (patch stage Kaposi sarcoma), which occasionally resemble large junctional melanocytic nevi or may appear as irregular-shaped patches similar to the nevus flammeus.

More commonly, Kaposi sarcoma is evident as violaceous plaques or nodules on the lower extremities. The nodules tend to enlarge into dome-shaped tumors.

Cutaneous Kaposi sarcoma rarely may be infiltrative or exophytic. To the authors' knowledge, infiltrative Kaposi sarcoma has not been described outside of Africa. Exophytic Kaposi sarcoma may erode downward into bone.

Although rare, isolated osseous Kaposi sarcoma with osteolytic bone changes has been described.[33]

Lymphadenopathic Kaposi sarcoma may demonstrate skin lesions.

At times, a Köbner phenomenon appears evident, with nodules at sites of trauma.

A few unusual varieties of Kaposi sarcoma also exist.

Telangiectatic Kaposi sarcoma is an eruption of pink translucent nodules with prominent telangiectasia.

Ecchymotic Kaposi sarcoma appears as periorbital ecchymoses. Histologically, there is a large amount of extravasated red blood cells, no evidence of amyloidosis, and a dermis containing foci of proliferating moderately atypical spindle cells, vascular slits, erythrophagocytosis, and other features of Kaposi sarcoma. On the trunk, lesions often follow skin tension lines.

See the image below.

Ecchymotic Kaposi sarcoma in a man who is homosexuEcchymotic Kaposi sarcoma in a man who is homosexual.

Keloidal Kaposi sarcoma is evident as somewhat brown-to-violaceous keloidal nodules. Histologically, these are Kaposi sarcoma nodules with a keloidal component.

Cavernous Kaposi sarcoma is a rare type of locally aggressive Kaposi sarcoma characterized by cutaneous tumors that histologically resemble cavernous hemangiomas; however, the endothelial cells and their nuclei are large and prominent, bulging into the cavity.

Lymphangiomalike Kaposi sarcoma is a rare variant in which dilated vascular spaces produce a bullous-appearing eruption, typically on the lower legs. The lesions are easily compressible and appear clinically to be fluid-filled. The vascular channels are lined by banal-appearing endothelial cells permeating the dermis in the absence of spindle cell proliferation.

Occasionally, Kaposi sarcoma may be first evident as a cutaneous horn.[34]

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Causes

HHV-8 has been linked convincingly with all 4 types of KS, an association that is necessary, but not sufficient, to develop KS; therefore, other factors also are important. At this point, immunosuppression appears to be the most significant cofactor.

The use of prednisolone as an adjunct to treatment in HIV-1–associated pleural tuberculosis in Uganda was associated with a significantly higher incidence of KS (4.2 cases per 100 person-years, compared with 0 cases per 100 person-years [P = .02]),[35] which is a dramatic example of the induction of KS by immunosuppressive therapy with corticosteroids.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

W Clark Lambert, MD, PhD  Professor and Head, Dermatopathology, Departments of Pathology and Dermatology, UMDNJ-New Jersey Medical School

W Clark Lambert, MD, PhD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Dermatological Association, American Society of Dermatopathology, International Academy of Pathology, Medical Society of New Jersey, Sigma Xi, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Abby S Van Voorhees, MD  Assistant Professor, Director of Psoriasis Services and Phototherapy Units, Department of Dermatology, University of Pennsylvania School of Medicine, Hospital of the University of Pennsylvania

Abby S Van Voorhees, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, National Psoriasis Foundation, Phi Beta Kappa, Sigma Xi, and Women's Dermatologic Society

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Merck Salary Management position; Abbott Honoraria Speaking and teaching; Amgen Honoraria Review panel membership; Centocor Honoraria Consulting; Leo Consulting; Merck None Other

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Christen M Mowad, MD  Associate Professor, Department of Dermatology, Geisinger Medical Center

Christen M Mowad, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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Elderly American man of Armenian origin with characteristic violaceous plaques of the legs, a good example of classic Kaposi sarcoma.
Man who is homosexual and has HIV infection and Kaposi sarcoma.
Man who is homosexual and has HIV infection and Kaposi sarcoma.
Man who is homosexual and has HIV infection and Kaposi sarcoma.
Elderly man of Mediterranean lineage with hyperkeratotic nodule of Kaposi sarcoma on sole of foot.
Man who is homosexual and has HIV infection and Kaposi sarcoma.
Man who is homosexual and has HIV infection and Kaposi sarcoma.
Man who is homosexual and has HIV infection and Kaposi sarcoma.
A 35-year-old man with dome-shaped locally aggressive tumors, an example of exophytic Kaposi sarcoma with cavernous hemangiomalike histology.
Ecchymotic Kaposi sarcoma in a man who is homosexual.
Intermediate lesion showing moderately enlarged spindle cells, some of which line poorly formed blood vessels that open into the interstitium with extravasation of erythrocytes. Erythrophagocytosis by these spindle cells is noted in places (hematoxylin and eosin, magnification X80).
Early lesion showing increased cellularity of the dermis with slightly enlarged spindle cells of focally fine stellate blood vessels that open at their corners into the interstitium (hematoxylin and eosin, magnification X40).
Keloidal Kaposi sarcoma demonstrating features both of keloids and Kaposi sarcoma (hematoxylin and eosin, magnification X80).
Oral Kaposi sarcoma.
Kaposi sarcoma of the leg.
Late lesion showing masses of moderately atypical spindle cells in a haphazard array, some of which line poorly formed blood vessels. Many of the spindle cells show erythrophagocytosis (hematoxylin and eosin, magnification X40).
Late lesion showing atypical spindle cells, some of which are lining poorly formed blood vessels, with prominent erythrophagocytosis by the cells (hematoxylin and eosin, magnification X80).
 
 
 
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