Sections

Dermatologic Manifestations of Kaposi Sarcoma

Sections Dermatologic Manifestations of Kaposi Sarcoma
Overview
Presentation
DDx
Workup
Treatment
Medication
Questions & Answers
Media Gallery
References

Overview

Practice Essentials

In 1981, Kaposi sarcoma seen in AIDS (KS-AIDS) in America was identified in 3 reports of Kaposi sarcoma as an original defining element of what later became known as AIDS (plus an important editorial and a Centers for Disease Control and Prevention Morbidity and Mortality Weekly Report bulletin). These original descriptions were by Borkovic and Schwartz in San Francisco, Friedman-Kien from New York City,^[1] and Gottlieb and associates in Los Angeles.^[2] For some time, Kaposi sarcoma was seen in 30-40% of patients with AIDS, often as the presenting sign. The incidence of Kaposi sarcoma has fallen markedly in recent times, although its prevalence has not. The challenge remained to explain the reason male patients who have sex with males and have AIDS exhibited Kaposi sarcoma much more commonly than did patients with AIDS unassociated with homosexuality, with the exception of small foci of this group in isolated midwestern communities.

The breakthrough came in 1994, when the Kaposi sarcoma–associated herpes virus (human herpesvirus type 8 [HHV-8]) was identified using representational difference analysis. HHV-8 has been linked closely with all 4 types of Kaposi sarcoma, ie, classic (traditional), endemic (African), epidemic (AIDS related), and iatrogenic (related to immunosuppression).^[3] Since then, much research has shown that HHV-8 appears to be necessary to, but not sufficient for, the development of Kaposi sarcoma.^[4]

Nevertheless, two critical questions remain. Is Kaposi sarcoma a hyperplasia or a neoplasm? Is it always multicentric or can it be metastatic as well? The authors favor the latter interpretation of both points.

Kaposi sarcoma and its causative agent, Karposi sarcoma–associated herpesvirus, have distinctive largely unexplained geographic distributions.^[5] The "oncoweed" hypothesis states that biologic plants in the environment account for the lytic reactivation of human herpesvirus 8. Quinine and its derivatives might better explain the epidemiology of Kaposi sarcoma in Africa than oncoweeds. Thus, an "oncodrug" hypothesis has been proposed, specifically with regard to quinine and its derivatives for African Kaposi sarcoma.

See the image below.

Elderly American man of Armenian origin with characteristic violaceous plaques of the legs, a good example of classic Kaposi sarcoma.

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Signs and symptoms

See Presentation.

Diagnostics

See Workup for a full discussion.

Evaluation for Kaposi sarcoma should include a complete physical examination and a biopsy of suspected lesions including lymph nodes. CT scan used to guide a fine needle for tissue specimens appears promising.

Management

Also see Treatment and Medication.

Solitary Kaposi sarcoma lesions may be excised surgically or removed using laser surgery.

Because the natural history of Kaposi sarcoma (KS) is variable, assessment of therapy may be difficult. Treatment usually is based on the extent of disease and the patient's immune status. The optimal therapy of KS-AIDS is yet to be determined. The challenge is to treat KS-AIDS effectively without immunocompromising the patient further, or better, with reconstitution of the immune system.

Management modalities for Kaposi sarcoma include nonintervention, surgical removal of skin nodules or severely affected areas (eg, areas of the extremities, intussuscepted bowel), laser surgery, conventional and megavoltage radiotherapy, chemotherapy, immunotherapy, antiviral drugs, and cessation of immunosuppressive therapy in iatrogenically immunosuppressed patients.

Indolent skin tumors in elderly white patients may not require specific therapy early in the course of the disease; however, systemic vinblastine (or other chemotherapy) attacks both cutaneous and visceral lesions.

Localized nodular disease may respond well to surgical excision, radiotherapy, and intralesional and outpatient low-dose vinblastine chemotherapy. The latter combination of local and systemic regimens may be preferable. The authors usually inform patients that this is a multicentric disease that has silent gut lesions that also may regress with the systemic approach. Although the authors prefer intralesional vinblastine, intralesional vincristine as first-line therapy for nodules in classic Kaposi sarcoma has also been recommended.^[6]

The efficacy of taxanes (eg, paclitaxel, docetaxel), as agents with antiangiogenic properties, has been shown for patients with AIDS-associated Kaposi sarcoma and in those with refractory or life-threatening Kaposi sarcoma without HIV infection.^[7] Pegylated liposomal doxorubicin is now being used as a second-line therapy in the treatment of patients with advanced classic Kaposi sarcoma.^[8]

Limited experience for classic and HIV-related cutaneous Kaposi sarcoma treated with 0.1% topical timolol gel has shown that it can be beneficial.^[9]

Radiotherapy is an option for some Kaposi sarcoma patients. Radiotherapy often produces good therapeutic results with classic nodular Kaposi sarcoma but tends to be only palliative in patients with Kaposi sarcoma and AIDS. In localized nodular Kaposi sarcoma, conventional radiotherapy is highly effective.

Electron-beam radiotherapy, which has limited penetration beyond the dermis, may be a good modality for superficial lesions.

Deeper or unresponsive Kaposi sarcoma may be treated using standard non–electron-beam radiation or other options.

Initial response to radiotherapy usually is complete or demonstrates marked regression of the nodules. The more extensive the involvement, the less responsive it tends to be.

Radiotherapy may be more effective on new, rather than chronic, lesions and may provide local Kaposi sarcoma control in patients with KS-AIDS.

Radioisotope scanning using technetium (Tc) 99m may detect occult Kaposi sarcoma infiltration in the subcutaneous and muscular tissues and draining lymph nodes. This allows improved efficiency of large-field radiotherapy.

Argon laser photocoagulation therapy also may be beneficial in classic Kaposi sarcoma lesions. Similarly, classic Kaposi sarcoma may favorably respond to long-pulse neodymium-doped yttrium aluminum garnet laser therapy.^[10]

Background

In 1872, Moritz Kaposi (1837-1902) of Kaposvar, Hungary, a dermatology faculty member at the University of Vienna, first described idiopathisches multiples Pigmentsarkom der Haut, which has become known as Kaposi sarcoma (KS). Kaposi sarcoma had brownish red–to–bluish red cutaneous nodules that tended to enlarge into dome-shaped tumors. Kaposi observed similar neoplasms of the mucosa, especially of the larynx, trachea, stomach, liver, and colon. Kaposi's original 1872 description of 5 patients is more similar to the Kaposi sarcoma seen in AIDS (KS-AIDS) than the Kaposi sarcoma expected in elderly men of Italian, Jewish, or Mediterranean linkage, in whom the disease behavior is benign. Kaposi's original 5 patients died within 2-3 years. Kaposi later updated his data, noting that all of his 16 patients were men with a prognosis that remained unfavorable.

In 1882, Tommaso De Amici, Professor and Head, Dermatology, University of Naples, Italy published in monograph form a detailed analysis of 12 patients with Kaposi sarcoma.^{[11, 12]}

For most of the first 3 quarters of the 20th century, Kaposi sarcoma was viewed as an indolent slowly growing cancer, and patients were expected to die with, rather than of, Kaposi sarcoma. The aggressive course originally noted by Kaposi has become part of the devastation of AIDS, especially among men who have sex with men.^{[13, 14]}

Pathophysiology

HIV transactivating (tat) gene, cytokine, and HHV-8 stories in Kaposi sarcoma (KS) are fascinating. Each begins with a classic study. In 1988, the human immunodeficiency virus type 1 (HIV-1) tat gene was introduced into transgenic mice, inducing nodules that resembled Kaposi sarcoma in 33 of 37 males but in none of 15 females. Therefore, it appeared that HIV could play a direct role in causing Kaposi sarcoma. The medication quinine may be a potentially overlooked triggering factor in millions of Africans with Kaposi sarcoma.^[15]

The second saga was a result of efforts to grow Kaposi sarcoma cells in culture, requiring a long-term growth factor. Conditioned medium from T cells infected with human T-cell leukemia virus type II (rather than HIV-1 or human T-cell leukemia virus type I) best supported the growth and long-term culture of Kaposi sarcoma cells derived from KS-AIDS lesions. In 1992, this growth factor proved to be a cytokine previously termed oncostatin M, since it had been identified earlier for its inhibitory effects on a variety of cancer cells. Another cytokine scatter factor was found in large quantities in this medium, inducing endothelial cells to demonstrate a Kaposi sarcoma tumor cell-like phenotype. The importance of oncostatin M, scatter factor, and the tat protein has been shown in the pathogenesis of Kaposi sarcoma.

Other cytokines, including interleukin 1 (IL-1), tumor necrosis factor, interleukin 6 (IL-6), and basic fibroblastic growth factor (bFGF), may work synergistically with the HIV tat gene product. Scatter factor may be involved both in initiation and in maintenance of Kaposi sarcoma. Scatter factor stimulates endothelial cells to migrate nearby and become factor XIIIa–positive c-Met- expressing spindle-shaped Kaposi sarcoma cells. The cells further expand neovascularization by producing cytokines and promoting autocrine-mediated and paracrine-mediated growth of Kaposi sarcoma cells. The scatter factor receptor, c-Met proto-oncogene, is expressed by Kaposi sarcoma cells; the oncogene int-2 of the fibroblast growth factor family also may be evident.

Herpes-type viruses have been linked with Kaposi sarcoma for more than 3 decades. A landmark study showed short DNA sequences of a unique human herpesvirus in Kaposi sarcoma tissues via a new molecular biological technique termed representational difference analysis. They resembled herpesvirus saimiri but proved to be a new type of human herpesvirus now termed HHV-8. This virus appears to interact with the HIV tat protein, excess levels of basic fibroblast growth factor, scatter factor, and IL-6. For example, HHV-8–encoded IL-6 has been found to induce endogenous human IL-6 secretion. An HHV-8 oncogene, Kaposin (ORF K12), has been characterized; however, additional factors remain to be found. For example, a 53% prevalence of HHV-8 subtype E in Brazilian Indians does not appear to be linked with the development of Kaposi sarcoma in this population.^[16]

Classic Kaposi sarcoma is seen in Italy with hot spots being in the Po River Valley, Sardinia, and southern Italy. It has been suggested that volcanic soil or birthplace/residency in areas abundant with bloodsucking insects may be a risk factor.^[17]A survey evaluated the correlation between HHV-8 infection and classic Kaposi sarcoma incidence in northern Sardinia.^[18]It revealed that seroprevalence was 35%, within a range of 15.3-46.3% in the five areas. Age was as an important risk factor. Subjects aged older than 50 years had a higher seroprevalence to HHV-8 as compared with younger individuals. A strong direct correlation between HHV-8 prevalence and classic Kaposi sarcoma incidence was also observed.

Kaposi sarcoma–associated herpesvirus (KSHV), or human herpesvirus 8 (HHV-8), is the most frequent cause of malignancy in patients with AIDS.^[19]KSHV and related herpesviruses have pirated cellular cDNAs from the host genome. Many of the viral regulatory homologs encode proteins that directly inhibit host adaptive and innate immunity. Other viral proteins may target retinoblastoma protein and p53 control of tumor suppressor pathways, which play key effector roles in intracellular immune responses. The immune evasion strategies used by KSHV in targeting tumor suppressor pathways activated during immune system signaling, may lead to inadvertent cell proliferation and tumorigenesis in susceptible hosts.

HHV-8 variants have been found heterogeneously distributed in diverse geographic regions, but their pathogenicity in Kaposi sarcoma development is unknown.^[20]The frequency of KSHV genotypes has been evaluated in numerous settings.^{[21, 22]}The frequency of these genotypes isolated from Kaposi sarcoma lesions among 50 patients from one center in Brazil was evaluated. The most frequently detected viral genotypes were A (50%) and C (48%); the B genotype was isolated only in one case.^[23]A genotype was predominant in those HIV-positive, whereas the C genotype was mostly in those in the HIV-negative group. An association between E genotype and Kaposi sarcoma development was observed in Peru.

The origin of the spindle cell, the hallmark cell of Kaposi sarcoma, is unknown. Most research favors a lymphatic endothelial cell or an endothelial-cell precursor evolving into a lymphatic phenotype, both preferentially targeted by KSHV.^[24]

Etiology

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]),^[25]which is a dramatic example of the induction of KS by immunosuppressive therapy with corticosteroids.

Frequency

United States

The incidence of Kaposi sarcoma (KS) has been estimated at 0.02-0.06%. Traditional Kaposi sarcoma of middle-aged and older American men of Mediterranean and Eastern European (Ashkenazi) Jewish lineage represents approximately 0.2% of cancer cases in the United States. Iatrogenic Kaposi sarcoma has a 400% increased incidence compared to the American population at large. The incidence of Kaposi sarcoma among American renal transplant recipients is approximately 0.4%. In the AIDS grouping, Kaposi sarcoma originally accounted for as many as 35% of patients, an incidence that has been declining with early detection of AIDS, although Kaposi sarcoma prevalence may remain high. Nevertheless, it may develop in HIV patients with well-controlled HIV disease and may be a significant factor in morbidity and mortality.^[26] In the United States, a few emigrants arrive from Kaposi sarcoma–endemic regions (primarily Africa). The largest immigrant population in this category may be Haitians; however, recent evidence suggests that Haitian AIDS originated directly in New York rather than Africa.

International

Four groups are predisposed to Kaposi sarcoma including (1) older men of Mediterranean and Jewish lineage; (2) Africans from areas including Uganda, the Congo Republic, Congo (Brazzaville), and Zambia; (3) persons who are iatrogenically immunosuppressed; and (4) men who are have sex with men. Kaposi sarcoma traditionally is an uncommon disease in middle-aged and elderly European men of Mediterranean or Jewish lineage.

A similar focus of Kaposi sarcoma exists in the same age and sex groups in Africa. If a crudely calculated incidence of 28 cases of Kaposi sarcoma per 100,000 in the Arabian population is correct, Kaposi sarcoma may be more common among Arabians than among Mediterranean people. A previously unrecognized genetic predisposition for Kaposi sarcoma among Arabians has been suggested. The incidence of Kaposi sarcoma among renal transplant recipients may be as high as 3.5% or higher in regions endemic for Kaposi sarcoma, which is significantly higher than the 0.4% incidence renal transplant recipients in the United States and Western Europe.

Endemic African Kaposi sarcoma has accounted for 10% of cancers and has been seen in a male-to-female ratio of 15:1. The Kampala Cancer Registry, one of the continent's first and foremost, has shown a significant alteration in the incidence of Kaposi sarcoma in the era of AIDS. In Uganda, Kaposi sarcoma has caused almost one half (48.9%) of cancer cases in men and 17.9% in women. The incidence in men (30.1 cases per 100,000) represents a more than 10-fold increase in men since the 1950s and is approximately 3 times the incidence found in women (11 cases per 100,000). The incidence in boys and girls was approximately the same in childhood (birth to 14 y), with small peaks in girls younger than 5 years and boys aged 5-9 years. Subsequently, a progressive rise in incidence peaked in women aged 25-29 years and in men aged 35-39 years. Lymphadenopathic Kaposi sarcoma affected 12% of total cases; 42% of childhood cases were of this type.

Of 73 Ugandan children with epidemic Kaposi sarcoma from the Uganda Cancer Institute in Kampala (from 2004-2007), 37 were boys and 36 were girls, with a median age of 10.1 years (range 2-18 y).^[27]

In neighboring Zambia, the disorder was particularly aggressive among children, more than 80% of whom were HIV seropositive. Kaposi sarcoma was found to represent as much as 25% of childhood cancers. The average male-to-female ratio was 1.76:1, with male predominance higher in children older than 5 years (2.5:1 ratio) than in children younger than 5 years (1.4:1 ratio). The prevalence of HIV-related Kaposi sarcoma seems to be increasing in Nigeria, probably owing to more females having HIV disease.^[28]

In Italy, KS-AIDS has produced notable epidemiologic changes. A doubling of Kaposi sarcoma incidence rates was noted in Italian men younger than 50 years from 1976-1984 to 1985-1990; however, no change, or possibly a decline, was observed in older men. The incidence of Kaposi sarcoma was estimated in the region around Venice, Italy, with rates higher in the coast and alpine valleys; in the latter, there was an excess of cases for both sexes combined (SIR = 191.1; CI = 113.2-302.0).^[17]The standardized incidence rate for Kaposi sarcoma in Italy declined markedly in the highly active antiretroviral treatment (HAART) period.^[29]

Classic Kaposi sarcoma in Greece seems to have an older age of onset; lower male-to-female ratio; endemic clustering; and disseminated skin disease at diagnosis, often accompanied by lymphedema and not unusual visceral or lymph node involvement.^{[30, 31, 32]}A clustering was noted, with a high proportion of the patients being born in Peloponnesos (42.42%) and residing in Athens (51.51%) or in Peloponnesos (24.24%).

The incidence rates of classic Kaposi sarcoma in Italy after the spread of AIDS was evaluated.^[18]The rates were 1 case per 100,000 population in men and 0.4 case per 100,000 population in women, varying from 0.3 cases per 100,000 population for men in Umbria and 4.7 cases per 100,000 population for men in Sassari in men and from 0.1 case per 100,000 population for women in Parma and 1.7 cases per 100,000 population for women in Sassari.

The rate of classic Kaposi sarcoma in southern Sardinia (Italy) from 1998-2002 was found to be 2.49 cases per 100 000 population per year (standardized), which was the highest rate recorded in the island.^[33]

A Finnish nationwide registry–based analysis of cancer clustering detected a strong familial occurrence of Kaposi sarcoma. This study found Kaposi sarcoma had a very high cluster score, with Kaposi sarcoma patients forming clusters of close relatives.^[34]One family was documented with 5 affected individuals in 2 generations. Several other families had 2 first-degree relatives with Kaposi sarcoma.

The increasing availability of antiretroviral therapy in Latin America has produced a marked decrease in Kaposi sarcoma since 2000, with its incidence there stabilizing and the risk being highest before and shortly after antiretroviral therapy initiation.^[35]

Race

Kaposi sarcoma is an uncommon disease of middle-aged and elderly American and European men of Mediterranean or Jewish lineage. This propensity also is seen in individuals with iatrogenically induced Kaposi sarcoma but not among persons in the KS-AIDS group. Kaposi sarcoma is rare in American Blacks, despite its large foci among Blacks in certain regions of Africa.

Sex

Classic Kaposi sarcoma has an overwhelming male predominance, with a male-to-female ratio of approximately 10-15:1.

For endemic Kaposi sarcoma in Central Africa, the male-to-female ratio is near unity in childhood Kaposi sarcoma cases but often rises in puberty to 15:1.

In Corsica and Sardinia (where classic Kaposi sarcoma is endemic), with the arrival of AIDS, the ratio of male-to-female cases has dropped from 10:1 to 3:1. Children of women who are HIV-1 seropositive without Kaposi sarcoma have an aggressive form of childhood HIV-associated Kaposi sarcoma. A male-to-female ratio of 1.5:1 was observed.

A male-to-female ratio of 1.5:1 also was evident among renal transplant recipients in Arabia.^[36]

In Zambia, Kaposi sarcoma represents up to 25% of childhood cancers and has an average male-to-female ratio of 1.76:1, with male predominance higher in children older than 5 years (2.5:1) than in children younger than 5 years (1.4:1).

Clinical manifestations and therapeutic response in men and women with AIDS-associated KS in Uganda differed between men and women.^[37]At presentation, the median CD4 T-cell count was significantly lower in women than men, with women more likely than men to initially develop Kaposi sarcoma of the face and hard palate and more likely to develop lesions on the lower extremity. In addition, women were less likely than men to demonstrate clinical improvement.

Age

Age distribution depends on the type of Kaposi sarcoma.

In the United States and Europe, traditional Kaposi sarcoma has a peak incidence between 40-70 years, with a wide range of up to 89 years.

Young men with KS-AIDS who are engage in sex with men also show a wide age range but tend to be much younger, averaging 20-40 years at age of onset.

For endemic Kaposi sarcoma in Uganda, the incidence in boys and girls was approximately the same in childhood (birth to 14 y), with a small peak in girls younger than 5 years and boys aged 5-9 years. Subsequently, a progressive rise in incidence peaked in women aged 25-29 years and in men aged 35-39 years.

Prognosis

Clinical classification of Kaposi sarcoma may be the best prognosticator, comparing localized nodular disease, locally aggressive disease, and generalized Kaposi sarcoma.

Cutaneous skin testing for anergy may correlate with clinical disease type and prognosis.

Prognosis appears to correlate with the CD4 count.

Localized nodular Kaposi sarcoma has the best prognosis, with few deaths directly attributable to Kaposi sarcoma.

Locally aggressive Kaposi sarcoma has an intermediate prognosis.

The authors do not disagree with an old African estimate of a 3-year survival rate of 64%. Generalized Kaposi sarcoma, the form seen most commonly in patients with KS-AIDS, has a 3-year survival rate closer to 0% without therapy.

The low level of access to antiretroviral drugs in HIV-infected patients explains the morbidity and mortality from AIDS-associated Kaposi sarcoma in much of Africa, including Togo.^[38]

Seventy children with Kaposi sarcoma and HIV infection were evaluated in South African hospitals.^[39]The mortality was high despite use of antiretrovirals and cytostatics, with 32 patients surviving only 4 months on average; 10 were lost to follow-up, and 28 were alive, with an average follow-up of 16 months.

Significant morbidity and mortality has been seen in those with Kaposi sarcoma IRIS.^{[40, 41]}

Mortality/morbidity

Patients with traditional Kaposi sarcoma (KS) tend to die with Kaposi sarcoma rather than of Kaposi sarcoma. Patients with KS-AIDS usually die from associated opportunistic infections or from gastrointestinal Kaposi sarcoma with hemorrhage. The mean survival rate of patients with KS-AIDS has been approximately 15-24 months, although the introduction into the United States of apparent immune system reconstitution using highly active antiretroviral therapy (HAART) has extended survival substantially. Kaposi sarcoma also may be fatal as a result of gut perforation, cardiac tamponade, massive pulmonary obstruction or, rarely, brain metastases.

In Kaposi's original description, death usually ensued within 3 years and was linked to fever, diarrhea, and hemoptysis. Inanition may be an important factor, and death may ensue as a result of bulky tumor obstructing the bronchi or larynx.

Patients with AIDS-related Kaposi sarcoma often have widespread visceral Kaposi sarcoma, although Kaposi sarcoma limited to the skin also is common.

Patients with iatrogenic Kaposi sarcoma tend to have gut bleeding resulting from Kaposi sarcoma, although termination or reduction of immunosuppression often, but not always, results in regression of Kaposi sarcoma.

Clinical Presentation

Friedman-Kien AE. Disseminated Kaposi's sarcoma syndrome in young homosexual men. J Am Acad Dermatol. 1981 Oct. 5(4):468-71. [QxMD MEDLINE Link].
Gottlieb MS, Schroff R, Schanker HM, Weisman JD, Fan PT, Wolf RA, et al. Pneumocystis carinii pneumonia and mucosal candidiasis in previously healthy homosexual men: evidence of a new acquired cellular immunodeficiency. N Engl J Med. 1981 Dec 10. 305(24):1425-31. [QxMD MEDLINE Link].
Allan AE, Shoji T, Li N, Burlage A, Davis B, Bhawan J. Two cases of Kaposi's sarcoma mimicking Stewart-Treves syndrome found to be human herpesvirus-8 positive. Am J Dermatopathol. 2001 Oct. 23(5):431-6. [QxMD MEDLINE Link].
Schwartz RA, Micali G, Nasca MR, Scuderi L. Kaposi sarcoma: a continuing conundrum. J Am Acad Dermatol. 2008 Aug. 59(2):179-206; quiz 207-8. [QxMD MEDLINE Link].
Ruocco V, Ruocco E, Schwartz RA, Janniger CK. Kaposi sarcoma and quinine: A potentially overlooked triggering factor in millions of Africans. J Am Acad Dermatol. 2011 Feb. 64(2):434-6. [QxMD MEDLINE Link].
Brambilla L, Bellinvia M, Tourlaki A, Scoppio B, Gaiani F, Boneschi V. Intralesional vincristine as first-line therapy for nodular lesions in classic Kaposi sarcoma: a prospective study in 151 patients. Br J Dermatol. 2009 Nov 25. [QxMD MEDLINE Link].
Fardet L, Stoebner PE, Bachelez H, et al. Treatment with taxanes of refractory or life-threatening Kaposi sarcoma not associated with human immunodeficiency virus infection. Cancer. 2006 Apr 15. 106(8):1785-9. [QxMD MEDLINE Link].
Di Lorenzo G, Di Trolio R, Montesarchio V, et al. Pegylated liposomal doxorubicin as second-line therapy in the treatment of patients with advanced classic Kaposi sarcoma: a retrospective study. Cancer. 2008 Mar 1. 112(5):1147-52. [QxMD MEDLINE Link].
Abdelmaksoud A, Filoni A, Giudice G, Vestita M. Classic and HIV-related Kaposi sarcoma treated with 0.1% topical timolol gel. J Am Acad Dermatol. 2017 Jan. 76 (1):153-155. [QxMD MEDLINE Link].
Özdemir M, Balevi A. Successful Treatment of Classic Kaposi Sarcoma With Long-Pulse Neodymium-Doped Yttrium Aluminum Garnet (Nd: YAG) Laser: A Preliminary Study. Dermatol Surg. 2017 Feb 1. [QxMD MEDLINE Link].
Ronchese F. Kaposi's sarcoma; an overlooked essay of 1882. AMA Arch Derm. 1958 May. 77(5):542-5. [QxMD MEDLINE Link].
Schiavo AL, Ruocco V, Marino F, Ferraiolo S, Pinto F, Orlando G. Tommaso de Amicis, Augusto Ducrey, Lodovico Tommasi: three Neapolitan stars in the dermatovenereology firmament. Int J Dermatol. 1996 Jan. 35(1):57-62. [QxMD MEDLINE Link].
Borkovic SP, Schwartz RA. Kaposi's sarcoma presenting in the homosexual man -- a new and striking phenomenon!. Ariz Med. 1981 Dec. 38(12):902-4. [QxMD MEDLINE Link].
Borkovic SP, Schwartz RA. Kaposi's sarcoma. Am Fam Physician. 1982 Oct. 26(4):133-7. [QxMD MEDLINE Link].
Ruocco V, Ruocco E, Schwartz RA, Janniger CK. Kaposi’s sarcoma and quinine: a potentially overlooked triggering factor in millions of Africans. J Am Acad Dermatol. 2010. in press:
Biggar RJ, Whitby D, Marshall V, Linhares AC, Black F. Human herpesvirus 8 in Brazilian Amerindians: a hyperendemic population with a new subtype. J Infect Dis. 2000 May. 181(5):1562-8. [QxMD MEDLINE Link].
Ascoli V, Zambon P, Manno D, et al. Variability in the incidence of classic Kaposi's sarcoma in the Veneto region, Northern Italy. Tumori. 2003 Mar-Apr. 89(2):122-4. [QxMD MEDLINE Link].
Santarelli R, De Marco R, Masala MV, et al. Direct correlation between human herpesvirus-8 seroprevalence and classic Kaposi's sarcoma incidence in Northern Sardinia. J Med Virol. 2001 Oct. 65(2):368-72. [QxMD MEDLINE Link].
Moore PS, Chang Y. Kaposi's sarcoma-associated herpesvirus immunoevasion and tumorigenesis: two sides of the same coin?. Annu Rev Microbiol. 2003. 57:609-39. [QxMD MEDLINE Link].
Tornesello ML, Biryahwaho B, Downing R, et al. Human herpesvirus type 8 variants circulating in Europe, Africa and North America in classic, endemic and epidemic Kaposi's sarcoma lesions during pre-AIDS and AIDS era. Virology. 2010 Mar 15. 398(2):280-9. [QxMD MEDLINE Link].
Serwin AB, Mysliwiec H, Wilder N, Schwartz RA, Chodynicka B. Three cases of classic Kaposi's sarcoma with different subtypes of Kaposi's sarcoma-associated herpesvirus. Int J Dermatol. 2006 Jul. 45(7):843-6. [QxMD MEDLINE Link].
Cassar O, Blondot ML, Mohanna S, et al. Human herpesvirus 8 genotype E in patients with Kaposi sarcoma, Peru. Emerg Infect Dis. 2010 Sep. 16(9):1459-62. [QxMD MEDLINE Link].
Ramos da Silva S, Ferraz da Silva AP, Bacchi MM, Bacchi CE, Elgui de Oliveira D. KSHV genotypes A and C are more frequent in Kaposi sarcoma lesions from Brazilian patients with and without HIV infection, respectively. Cancer Lett. 2011 Feb 1. 301(1):85-94. [QxMD MEDLINE Link].
Dupin N, Grange PA. Looking for the target cell of Kaposi's sarcoma-associated herpesvirus. J Invest Dermatol. 2006 Mar. 126(3):545-7. [QxMD MEDLINE Link].
Elliott AM, Luzze H, Quigley MA, et al. A randomized, double-blind, placebo-controlled trial of the use of prednisolone as an adjunct to treatment in HIV-1-associated pleural tuberculosis. J Infect Dis. 2004 Sep 1. 190(5):869-78. [QxMD MEDLINE Link].
Hwang A, Iskandar AS, Kerr WT, Farrell S, Plaxe SC, Dasanu CA. Clinico-epidemiologic characteristics and patterns of care in Kaposi's sarcoma: Data from a single-institution series. J Oncol Pharm Pract. 2019 Apr 2. 1078155219838614. [QxMD MEDLINE Link].
Gantt S, Kakuru A, Wald A, et al. Clinical presentation and outcome of epidemic Kaposi sarcoma in Ugandan children. Pediatr Blood Cancer. 2010 May. 54(5):670-4. [QxMD MEDLINE Link]. [Full Text].
Onunu AN, Okoduwa C, Eze EU, Adeyekun AA, Kubeyinje EP, Schwartz RA. Kaposi's sarcoma in Nigeria. Int J Dermatol. 2007 Mar. 46(3):264-7. [QxMD MEDLINE Link].
Polesel J, Franceschi S, Suligoi B, Crocetti E, Falcini F, Guzzinati S, et al. Cancer incidence in people with AIDS in Italy. Int J Cancer. 2010 Jan 4. [QxMD MEDLINE Link].
Stratigos JD, Potouridou I, Katoulis AC, et al. Classic Kaposi's sarcoma in Greece: a clinico-epidemiological profile. Int J Dermatol. 1997 Oct. 36(10):735-40. [QxMD MEDLINE Link].
Barco D, Alegre M, Alomar A. [Classic kaposi sarcoma associated with lymphedema following arterial catheterization]. Actas Dermosifiliogr. 2008 Oct. 99(8):665-7. [QxMD MEDLINE Link].
Ruocco V, Schwartz RA, Ruocco E. Lymphedema: an immunologically vulnerable site for development of neoplasms. J Am Acad Dermatol. 2002 Jul. 47(1):124-7. [QxMD MEDLINE Link].
Atzori L, Fadda D, Ferreli C, et al. Classic Kaposi's sarcoma in southern Sardinia, Italy. Br J Cancer. 2004 Oct 4. 91(7):1261-2. [QxMD MEDLINE Link].
Kaasinen E, Aavikko M, Vahteristo P, Patama T, Li Y, Saarinen S, et al. Nationwide registry-based analysis of cancer clustering detects strong familial occurrence of kaposi sarcoma. PLoS One. 2013. 8(1):e55209. [QxMD MEDLINE Link].
Castilho JL, Kim A, Jenkins CA, et al. Antiretroviral therapy and Kaposi's sarcoma trends and outcomes among adults with HIV in Latin America. J Int AIDS Soc. 2021 Jan. 24 (1):e25658. [QxMD MEDLINE Link].
Qunibi W, Al-Furayh O, Almeshari K, et al. Serologic association of human herpesvirus eight with posttransplant Kaposi's sarcoma in Saudi Arabia. Transplantation. 1998 Feb 27. 65(4):583-5. [QxMD MEDLINE Link].
Phipps W, Ssewankambo F, Nguyen H, et al. Gender differences in clinical presentation and outcomes of epidemic Kaposi sarcoma in Uganda. PLoS One. 2010 Nov 12. 5(11):e13936. [QxMD MEDLINE Link]. [Full Text].
Pitche PT, Kombate K, Owono F, Tchangai-Walla K. Kaposi's sarcoma in a hospital setting in Lome (Togo): a study of 93 cases. Int J Dermatol. 2007 Oct. 46 Suppl 1:42-4. [QxMD MEDLINE Link].
Stefan DC, Stones DK, Wainwright L, Newton R. Kaposi sarcoma in South African children. Pediatr Blood Cancer. 2011 Mar. 56(3):392-6. [QxMD MEDLINE Link].
Achenbach CJ, Harrington RD, Dhanireddy S, Crane HM, Casper C, Kitahata MM. Paradoxical Immune Reconstitution Inflammatory Syndrome in HIV-Infected Patients Treated With Combination Antiretroviral Therapy After AIDS-Defining Opportunistic Infection. Clin Infect Dis. 2012 Feb. 54(3):424-33. [QxMD MEDLINE Link]. [Full Text].
Stover KR, Molitorisz S, Swiatlo E, Muzny CA. A Fatal Case of Kaposi Sarcoma Due to Immune Reconstitution Inflammatory Syndrome. Am J Med Sci. 2012 Jan 6. [QxMD MEDLINE Link].
Wolf R, Brenner S, Ruocco V, Filioli FG. Isotopic response. Int J Dermatol. 1995 May. 34(5):341-8. [QxMD MEDLINE Link].
Micali G, Nasca MR, De Pasquale R, Innocenzi D. Primary classic Kaposi's sarcoma of the penis: report of a case and review. J Eur Acad Dermatol Venereol. 2003 May. 17(3):320-3. [QxMD MEDLINE Link].
Schwartz RA, Cohen JB, Watson RA, et al. Penile Kaposi's sarcoma preceded by chronic penile lymphoedema. Br J Dermatol. 2000 Jan. 142(1):153-6. [QxMD MEDLINE Link].
Farshidpour M, Marjani M, Baghaei P, Tabarsi P, Masjedi H, Asadi Kani ZF, et al. Disseminated Kaposi's Sarcoma with the Involvement of Penis in the Setting of HIV Infection. Indian J Dermatol. 2015 Jan-Feb. 60(1):104. [QxMD MEDLINE Link]. [Full Text].
Elsaie ML, Shehu YM, Sani MU, Borodo MM, Mohammed AZ. Disseminated vitiligo developing around Kaposi sarcoma nodules in an AIDS patient. Cutis. 2011 Nov. 88(5):237-9. [QxMD MEDLINE Link].
Shah AS, Abu Saleh OM. Kaposi Sarcoma Masquerading as a Tooth Abscess in a Patient With Human Immunodeficiency Virus. Mayo Clin Proc. 2019 Feb. 94 (2):370. [QxMD MEDLINE Link].
Satta R, Biondi G, Cottoni F. Rainbow pattern: practical applications of dermoscopy in Kaposi's sarcoma. G Ital Dermatol Venereol. 2016 Apr. 107 (2):206-7. [QxMD MEDLINE Link].
McClain CM, Haws AL, Galfione SK, Rapini RP, Hafeez Diwan A. Pyogenic Granuloma-Like Kaposi's Sarcoma. J Cutan Pathol. 2016 Jun. 43 (6):549-51. [QxMD MEDLINE Link].
Korekawa A, Kaneko T, Nakano H, Sawamura D. Pyogenic granuloma-like Kaposi's sarcoma on the first toe. J Dermatol. 2018 Feb 14. [QxMD MEDLINE Link].
Megaly M, Boshra N. Pyogenic granuloma-like Kaposi's sarcoma. Lancet. 2022 Apr 23. 399 (10335):e38. [QxMD MEDLINE Link].
Aïm F, Rosier L, Dumontier C. Isolated Kaposi sarcoma of the finger pulp in an AIDS patient. Orthop Traumatol Surg Res. 2012 Feb. 98(1):126-8. [QxMD MEDLINE Link].
Schuch LF, Kovalski LNS, Leite AA, et al. Oral lymphangioma-like Kaposi sarcoma: a Brazilian case report in a scenario of a still high number of HIV infections. Oral Maxillofac Surg. 2022 Mar. 26 (1):171-176. [QxMD MEDLINE Link].
Silapunt S, Jordon RE, Piao Y, Tsai KY. Kaposi sarcoma presenting as a cutaneous horn. J Am Acad Dermatol. 2011 Feb. 64(2):447-8. [QxMD MEDLINE Link].
Manzardo C, Guardo AC, Letang E, Plana M, Gatell JM, Miro JM. Opportunistic infections and immune reconstitution inflammatory syndrome in HIV-1-infected adults in the combined antiretroviral therapy era: a comprehensive review. Expert Rev Anti Infect Ther. 2015 Apr 10. 1-17. [QxMD MEDLINE Link].
Vanousova D, Jilich D, Machala L, et al. [Diagnostic pitfalls of HIV-associated Kaposi's sarcoma]. Klin Onkol. 2010. 23(5):285-92. [QxMD MEDLINE Link].
Straka L, Adamicová K. [Reactive angioendotheliomatosis--a rare disease mimicking Kaposi sarcoma: a case report]. Cesk Patol. 2004 Oct. 40(4):162-6. [QxMD MEDLINE Link].
Croteau SE, Kozakewich HP, Perez-Atayde AR, Fishman SJ, Alomari AI, Chaudry G, et al. Kaposiform lymphangiomatosis: a distinct aggressive lymphatic anomaly. J Pediatr. 2014 Feb. 164(2):383-8. [QxMD MEDLINE Link]. [Full Text].
Mansur AT, Demirci GT, Ozbal Koc EA, Yildiz S. An unusual lesion on the nose: microvenular hemangioma. Dermatol Pract Concept. 2018 Jan. 8 (1):7-11. [QxMD MEDLINE Link].
Yaqub S, Stepenaskie SA, Farshami FJ, Sibbitt WL Jr, Fangtham M, Emil NS, et al. Kaposi Sarcoma as a Cutaneous Vasculitis Mimic: Diagnosis and Treatment. J Clin Aesthet Dermatol. 2019 Nov. 12 (11):23-26. [QxMD MEDLINE Link].
Pinos León VH, Granizo Rubio JD. Acral pseudolymphomatous angiokeratoma of children with rainbow pattern: A mimicker of Kaposi sarcoma. J Am Acad Dermatol. 2017 Feb. 76 (2S1):S25-S27. [QxMD MEDLINE Link].
Patel RM, Goldblum JR, Hsi ED. Immunohistochemical detection of human herpes virus-8 latent nuclear antigen-1 is useful in the diagnosis of Kaposi sarcoma. Mod Pathol. 2004 Apr. 17(4):456-60. [QxMD MEDLINE Link].
O'Mahony D, Gandjbakche A, Hassan M, Vogel A, Yarchoan R. Imaging techniques for Kaposi's sarcoma. J HIV Ther. 2008 Sep. 13(3):65-71. [QxMD MEDLINE Link].
Theron S, Andronikou S, Du Plessis J, et al. Pulmonary Kaposi sarcoma in six children. Pediatr Radiol. 2007 Dec. 37(12):1224-9. [QxMD MEDLINE Link].
Luzar B, Antony F, Ramdial PK, Calonje E. Intravascular Kaposi's sarcoma - a hitherto unrecognized phenomenon. J Cutan Pathol. 2007 Nov. 34(11):861-4. [QxMD MEDLINE Link].
O'donnell PJ, Pantanowitz L, Grayson W. Unique Histologic Variants of Cutaneous Kaposi Sarcoma. Am J Dermatopathol. 2010 Jan 14. [QxMD MEDLINE Link].
Yang SH, Leboit PE. Angiomatous kaposi sarcoma: a variant that mimics hemangiomas. Am J Dermatopathol. 2014 Mar. 36(3):229-37. [QxMD MEDLINE Link].
Wada DA, Perkins SL, Tripp S, Coffin CM, Florell SR. Human herpesvirus 8 and iron staining are useful in differentiating Kaposi sarcoma from interstitial granuloma annulare. Am J Clin Pathol. 2007 Feb. 127(2):263-70. [QxMD MEDLINE Link].
Bunn BK, Carvalho Mde V, Louw M, Vargas PA, van Heerden WF. Microscopic diversity in oral Kaposi sarcoma. Oral Surg Oral Med Oral Pathol Oral Radiol. 2013 Feb. 115(2):241-8. [QxMD MEDLINE Link].
Speicher DJ, Wanzala P, D'Lima M, Njiru A, Chindia M, Dimba E, et al. Diagnostic challenges of oral and cutaneous Kaposi's sarcoma in resource-constrained settings. J Oral Pathol Med. 2015 Mar 17. [QxMD MEDLINE Link].
Brambilla L, Boneschi V, Taglioni M, Ferrucci S. Staging of classic Kaposi's sarcoma: a useful tool for therapeutic choices. Eur J Dermatol. 2003 Jan-Feb. 13(1):83-6. [QxMD MEDLINE Link].
Krown SE, Metroka C, Wernz JC. Kaposi's sarcoma in the acquired immune deficiency syndrome: a proposal for uniform evaluation, response, and staging criteria. AIDS Clinical Trials Group Oncology Committee. J Clin Oncol. 1989 Sep. 7(9):1201-7. [QxMD MEDLINE Link].
Mitsuyasu RT. Clinical variants and staging of Kaposi's sarcoma. Semin Oncol. 1987 Jun. 14(2 Suppl 3):13-8. [QxMD MEDLINE Link].
Cattani P, Capuano M, Graffeo R, et al. Kaposi's sarcoma associated with previous human herpesvirus 8 infection in kidney transplant recipients. J Clin Microbiol. 2001 Feb. 39(2):506-8. [QxMD MEDLINE Link].
Frances C, Mouquet C, Marcelin AG, et al. Outcome of kidney transplant recipients with previous human herpesvirus-8 infection. Transplantation. 2000 May 15. 69(9):1776-9. [QxMD MEDLINE Link].
Khan MY, Khanam H, Koneru B, Schwartz RA. Kaposi's sarcoma in a liver transplant series. J Med. 1999. 30(3-4):185-90. [QxMD MEDLINE Link].
Khan MY, Khanman H, Koneru B. Kaposi's sarcoma in a liver transplantation series. J Med. 2000. 30:185-90.
Sabeel AI, Qunibi WY, Alfurayh OA, Al-Meshari K. Kaposi's sarcoma in Sudanese renal transplant recipients: a report from a single center. J Nephrol. 2003 May-Jun. 16(3):412-6. [QxMD MEDLINE Link].
Dollard SC, Annambhotla P, Wong P, Meneses K, Amin MM, La Hoz RM, et al. Donor-derived human herpesvirus 8 and development of Kaposi sarcoma among 6 recipients of organs from donors with high-risk sexual and substance use behavior. Am J Transplant. 2021 Feb. 21 (2):681-688. [QxMD MEDLINE Link].
Klein E, Schwartz RA, Laor Y, Milgrom H, Burgess GH, Holtermann OA. Treatment of Kaposi's sarcoma with vinblastine. Cancer. 1980 Feb. 45(3):427-31. [QxMD MEDLINE Link].
Goiriz R, Rios-Buceta L, De Arriba AG, Aragues M, Garcia-Diez A. Treatment of classic Kaposi's sarcoma with topical imiquimod. Dermatol Surg. 2009 Jan. 35(1):147-9. [QxMD MEDLINE Link].
Prinz Vavricka BM, Hofbauer GF, Dummer R, French LE, Kempf W. Topical treatment of cutaneous Kaposi sarcoma with imiquimod 5% in renal-transplant recipients: a clinicopathological observation. Clin Exp Dermatol. 2012 Feb 2. [QxMD MEDLINE Link].
Koon HB, Fingleton B, Lee JY, et al. Phase II AIDS Malignancy Consortium trial of topical halofuginone in AIDS-related Kaposi sarcoma. J Acquir Immune Defic Syndr. 2011 Jan 1. 56(1):64-8. [QxMD MEDLINE Link]. [Full Text].
Reschke R, Grunewald S, Schüürmann M, Simon JC, Ziemer M. Value of first-line surgical treatment for classic Kaposi sarcoma and potential use of checkpoint inhibitors. J Dtsch Dermatol Ges. 2020 Mar. 18 (3):256-262. [QxMD MEDLINE Link].
Rubegni P, Sbano P, De Aloe G, Flori ML, Fimiani M. Thalidomide in the treatment of Kaposi's sarcoma. Dermatology. 2007. 215(3):240-4. [QxMD MEDLINE Link].
Paghdal KV, Schwartz RA. Sirolimus (rapamycin): from the soil of Easter Island to a bright future. J Am Acad Dermatol. 2007 Dec. 57(6):1046-50. [QxMD MEDLINE Link].
Boratynska M, Watorek E, Smolska D, Patrzalek D, Klinger M. Anticancer effect of sirolimus in renal allograft recipients with de novo malignancies. Transplant Proc. 2007 Nov. 39(9):2736-9. [QxMD MEDLINE Link].
Detroyer D, Deraedt K, Schöffski P, Hauben E, Lagrou K, Naesens M, et al. Resolution of diffuse skin and systemic Kaposi's sarcoma in a renal transplant recipient after introduction of everolimus: a case report. Transpl Infect Dis. 2015 Apr. 17(2):303-7. [QxMD MEDLINE Link].
Leder HA, Galor A, Peters GB, Kedhar SR, Dunn JP, Thorne JE. Resolution of conjunctival Kaposi sarcoma after institution of highly active antiretroviral therapy alone. Br J Ophthalmol. 2008 Jan. 92(1):151. [QxMD MEDLINE Link].
Olweny CL, Borok M, Gudza I, et al. Treatment of AIDS-associated Kaposi's sarcoma in Zimbabwe: results of a randomized quality of life focused clinical trial. Int J Cancer. 2005 Feb 10. 113(4):632-9. [QxMD MEDLINE Link].
Cianfrocca M, Lee S, Von Roenn J, et al. Randomized trial of paclitaxel versus pegylated liposomal doxorubicin for advanced human immunodeficiency virus-associated Kaposi sarcoma: evidence of symptom palliation from chemotherapy. Cancer. 2010 Aug 15. 116(16):3969-77. [QxMD MEDLINE Link].
Molyneux E, Davidson A, Orem J, Hesseling P, Balagadde-Kambugu J, Githanga J, et al. The management of children with Kaposi sarcoma in resource limited settings. Pediatr Blood Cancer. 2012 Dec 19. [QxMD MEDLINE Link].
Delyon J, Biard L, Renaud M, et al. PD-1 blockade with pembrolizumab in classic or endemic Kaposi's sarcoma: a multicentre, single-arm, phase 2 study. Lancet Oncol. 2022 Apr. 23 (4):491-500. [QxMD MEDLINE Link].

Media Gallery

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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Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Coauthor(s)

W Clark Lambert, MD, PhD Clinical Professor of Pathology, Clinical Professor of Medicine (Professor of Dermatology), Rutgers New Jersey Medical School; Professor of Pathology, Rutgers Graduate School of Biomedical Sciences

W Clark Lambert, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American Dermatological Association, American Federation for Clinical Research, American Society of Dermatopathology, College of American Pathologists, Dermatological Society of New Jersey, Institute of Electrical and Electronics Engineers, International Academy of Pathology, International Federation of Pigment Cell Societies, International Society of Dermatopathology, Medical Society of New Jersey, Sigma Xi, The Scientific Research Honor Society, Society for Investigative Dermatology, Xeroderma Pigmentosum Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

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

Disclosure: Nothing to disclose.

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

Christen M Mowad, MD is a member of the following medical societies: Alpha Omega Alpha, Noah Worcester Dermatological Society, Pennsylvania Academy of Dermatology, American Academy of Dermatology, Phi Beta Kappa

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

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, Women's Dermatologic Society, National Psoriasis Foundation, American Medical Association, Phi Beta Kappa, Sigma Xi, The Scientific Research Honor Society

Disclosure: Received honoraria from Amgen for consulting; Received honoraria from Abbott for consulting; Partner received salary from Merck for management position; Received honoraria from Abbott for speaking and teaching; Received honoraria from Amgen for review panel membership; Received honoraria from Centocor for consulting; Received honoraria from Leo for consulting; Received none from Merck for other.