VI. PROGNOSIS
The prognosis of CTCL depends on the stage of disease at the time of diagnosis, age, gender, immunocompetence, and the choice and tolerance of the treatments. While there is no cure for MF and SS, the treatment should focus on controlling and limiting the skin lesions, delay the progression, and minimize the recurrence of the disease. The care for the patients should be directed at quality-of-life consideration by optimally treating pruritus and xerosis and prevention of skin infection.
PATIENT EDUCATION
mesenchymal origin). It was first described by Kaposi as idiopathic multiple pigment sarcoma.
This multifocal neoplasm is seen primarily as multiple vascular nodules in the skin and other organs. KS is associated with other neoplasms or immune disorders that occur before or after the diagnosis. KS is seen frequently in patients with HIV.
A. Definition: KS is a disease of proliferation in endothelial layer of lymph or blood vessels.
Excessive proliferation of spindle cells placed together with other cells such as endothelial cells, inflammatory cells, and fibroblasts in an epithelial layer of the vascular origin results in a formation of a new blood vessel (neoangiogenesis).
B. Incidence:
1. Incidence of KS varies according to geography.
a. KS incidence in the United States before the acquired immunodeficiency syndrome (AIDS) epidemic was only 1 for every million people, mostly classic and transplant related.
b. AIDS epidemic increased KS incidence by 20 times, about 47 cases per million in 1990s, with 1 in 2 chance of developing KS among people infected with HIV. With improving KS treatment options, average KS incidence rate decreased to 6 cases per million each year.
c. Another higher prevalence is among organ transplant recipients, with the incidence of 1 in 200 people in the United States.
d. Mostly affects people of either Jewish descent or Mediterranean descent.
e. In classic KS, two thirds of patients develop lesions only after the age of 50.
f. Disease is strikingly frequent in a region in Africa that includes Kenya, Tanzania, and Zaire with a frequency of 1.3% to 10% depending on region.
g. Among people with AIDS, KS predominates in the third and fourth decade with a mean age of 40 years.
C. Etiology (cause may be multifactorial) 1. Viral
a. New human herpesvirus (HHV-8/KS) and subsequent identification of humoral immune response to this agent in patients with KS may be causally related to KS.
b. Causative agents of KS associated with disease among young homosexual men with signs of profound immunosuppression include HIV and other specific serologic association between cytomegalovirus (CMV) and classic endemic KS.
c. Other causative agents of KS may include various microorganisms such as hepatitis B virus, human papillomavirus (HPV), and Mycoplasma penetrans as possible sources of KS.
d. With the replication of retroviral particles in KS lesions, some hypothesize a non-HIV retrovirus etiological linkage to KS.
2. Genetic predisposition 3. Geographic factors
4. Possible hormonal influences D. Pathogenesis:
1. Classic KS develops slowly and runs a benign course, although, infrequently, rapid courses with involvement of the lung, spleen, heart, and gastrointestinal tract have been
reported.
2. In addition to cutaneous lesions, it may develop on mucous membranes of the oral cavity and gastrointestinal tract.
II. CLINICAL VARIANTS
A. Classic (Mediterranean) KS (Figure 16-24)
1. Chronic skin disease affecting predominantly elderly men of Mediterranean, East European, or Jewish heritage with a peak incidence in men after the age of 60. Lesions often appear on the legs.
2. Affected individuals survive an average of 10 to 15 years from diagnosis and most often die from an unrelated cause.
3. Lesions occur most commonly as red or purple nodule or blotch on lower limbs and then upper limbs and may occur on the trunk, head, neck, genitalia, or any skin surface. May appear as blue hues in dark-skinned individuals.
4. Telangiectases may be evident on or near the tumors, ulceration may occur, or the surface may appear verrucous.
5. Lesions may be painful; burning or itching may also be present.
6. Lesions may spontaneously regress as new ones appear; spontaneous remissions have been documented.
7. Occurrence may be in the hundreds in a single patient; nodules may cluster along veins.
B. African or Endemic KS
1. Found in eastern half of African continent near the equator.
2. Described in two distinct age groups
a. Young adults with a mean age of 35 and a male/female ratio of 13:1 b. Young children with a mean age of 3 and a male/female ratio of 3:1 3. Most commonly nodular lesions that may regress spontaneously.
4. Florid variety is rapid growing and ulcerated and may bleed with tumors extending deep into the dermis and may involve underlying bone.
5. Infiltrative type is confined to the hand or foot appearing as deeply invasive, fibrotic, indurated tumors with nonpitting edema.
6. Rapidly growing enlarged lymph nodes, often confused clinically with lymphoma, typify lymphadenopathic type of KS occurring in children and young adults.
C. Iatrogenic (Transplant-Related) KS
1. Found in organ transplant patients, particularly renal transplants.
2. Seen in patients receiving chronic immunosuppressive drug therapy. Spontaneous remission after discontinuation of immunosuppressive therapy usually occurs.
D. AIDS-Associated, Epidemic KS (Figure 16-25) 1. KS is often the primary AIDS-defining illness.
2. Sexually transmitted cofactor may play a role in the development of AIDS-KS.
3. Incidence of AIDS-KS continues to rise as more HIV-infected patients progress to AIDS.
4. Lesions occur in the oral cavity, nose, postauricular, trunk, penis, legs, and feet appearing as small pink macules mimicking insect bites or small brown, tense papules.
5. Examination should include lymph node exam.
6. Macular lesions begin as salmon-colored with a pink halo.
a. Small, slightly elevated, and round.
b. Macule becomes purple or brown within a week and halo disappears.
c. Multiple lesions widely distributed; may demonstrate a mirror-image distribution.
7. Papular and nodular lesions.
a. May begin as macules.
b. Facial papules are round and less than 1 cm in size; lesions of the trunk, neck, and extremities are 1 to 2 cm and oblong.
8. Plaques and lymphatic disease.
a. Large purple plaques containing nodules with hyperkeratosis; resemble psoriasis.
b. Lymphatic involvement may produce numerous firm, red, round papules with local edema in affected nodal sites.
9. Mucocutaneous and ocular lesions.
a. Oral involvement most commonly seen as lesions on the palate.
b. Macular lesions of the conjunctiva are relatively benign.
10. Visceral lesions may affect gastrointestinal tract and lungs as well as the pharynx, heart, bone marrow, urogenital tract, brain, kidney, and adrenal glands.
FIGURE 16-24. Kaposi sarcoma. (From Werner, R. (2012). Massage therapist’s guide to pathology. Philadelphia, PA: Wolters Kluwer.)
FIGURE 16-25. Kaposi sarcoma. Multiple papules and nodules are present on this patient’s leg. (From Goodheart, H. P. (2003). Goodheart’s photoguide of common skin disorders (2nd ed.). Philadelphia, PA: Lippincott Williams & Wilkins.)
III. LABORATORY AND DIAGNOSTIC TESTS
A. Combination of clinical assessment and histological conformation by skin biopsy
B. CT scan, bronchoscopy, and endoscopy based on the symptoms and clinical suspicion on other organs’ involvement
IV. DIFFERENTIAL DIAGNOSIS
A. Bacillary angiomatosis
B. Blue rubber bleb nevus syndrome C. Melanocytic nevi
D. Pyogenic granuloma (lobular capillary hemangioma) E. Tufted angioma
V. THERAPEUTIC MODALITIES
A. There is no curative treatment for KS.
B. Local therapy
1. Excision of cutaneous lesions (often for cosmesis) 2. Radiation therapy
3. Chemotherapy either single or multiagent. Most promising results in use of liposomal encapsulated doxorubicin and daunorubicin for both AIDs-associated KS and classic KS 4. Systemic treatment of interferon alpha, alone or in combination with cytotoxic therapy or
with zidovudine for AIDS-KS
5. Highly active antiretroviral therapy (HAART) for AIDS-related KS
VI. PROGNOSIS
The prognosis is dependent on the size and location of the KS lesions, the function of the immune system, and the comorbidities of the patient. Compromised immune system and underlying comorbidities may cause challenges in making choices for treatment.