A Rare Malignant Etiology of Zosteriform Lesions: Kaposi’s Sarcoma Christina Steinmetz-Rodriguez, DO,* Leslie Mills, DO,** Robin Shecter, DO, FAOCD*** *Third-year Dermatology Resident, Palm Beach Consortium for Graduate Medical Education, JFK Medical Center North Campus, West Palm Beach, FL **Second-year Dermatology Resident, Palm Beach Consortium for Graduate Medical Education, JFK Medical Center North Campus, West Palm Beach, FL ***Program Director, Palm Beach Consortium for Graduate Medical Education, JFK Medical Center North Campus, West Palm Beach, FL Disclosures: None Correspondence: Christina Steinmetz-Rodriguez, DO; [email protected] Abstract Kaposi’s sarcoma, the most common neoplasm occurring in acquired immunodeficiency syndrome (AIDS), is a vascular tumor with often varied clinical presentation and a prevalence of 5% to 25% in the United States.1,2 We present the case of a 28-year-old man presenting with nodular, red-to-purplish macules in a zosteriform distribution, ultimately diagnosed as Kaposi’s sarcoma. Only three other cases of zosteriform Kaposi’s sarcoma have been reported in the literature. We review the classification, histology, and treatment modalities of Kaposi’s sarcomas as well as the differential diagnosis of cutaneous diseases that present in a dermatomal pattern. Introduction with intravenous acyclovir for three days and to 5 cm in diameter, were visualized above the Kaposi’s sarcoma (KS) was first described in then oral valacyclovir for a week after discharge. medial malleolus and the medial thigh. 1872 by the Hungarian dermatologist Moritz Medical history was remarkable for HIV with Shave biopsy of a representative lesion revealed Kaposi as a pigmented, idiopathic sarcoma a CD4 count of 264, hepatitis B, and a seizure a dermal tumor (Figure 3, low magnification). of the skin of elderly men. KS remains one disorder, as well as Hodgkin’s lymphoma two Increased magnification revealed atypical of the most common malignancies in AIDS 1,2 years prior, which was treated with doxorubicin pleomorphic spindle cells arranged in fascicles, patients. Before the 1980s, KS was only and paclitaxel. The patient’s medications with numerous extravasated erythrocytes and described in elderly men of Eastern European, included with efavirenz, emtricitabine, tenofovir, small capillaries between the collagen bundles Mediterranean, or Jewish descent. KS emerged levetiracetam, oxcarbazepine, and gabapentin. (Figure 4). Numerous mitotic figures were on a larger scale in the early 1980s in New York Family history was noncontributory. and San Francisco with the onset of the AIDS 2 Figure 2 epidemic. Physical examination revealed a well-appearing man who was afebrile. There were numerous red- Four variants of KS have been described in the to-purple papules, 0.5 cm to 2 cm in diameter, literature: 1) the classic form, occurring in older confined to the right hemithorax at the T4 to men of Eastern European, Mediterranean, or T6 thoracic dermatomes; they did not cross the Jewish descent; 2) the endemic form, presenting in midline (Figures 1, 2). In addition, there was a young Bantu children in Africa; 3) the transplant- single macular lesion to the left upper anterior associated form, which appears months to years thorax. Regional adenopathy was appreciated after high doses of immunosuppressive agents; 3 in the right axilla. No lesions were found on and 4) the AIDS-related form. the hard palate. Faded purplish macules, 3 cm The etiology of this vascular neoplasm remained elusive until 1994, when Cheng et al. discovered a novel type of human herpesvirus in AIDS- associated KS lesions.4 The virus was found not only in nearly all KS lesions, but also in mice injected with the virus that later developed clinically and histologically KS-like skin Figure 3 lesions.3,5 This virus was later named human herpesvirus type 8 (HHV-8) and revolutionized our understanding of this neoplasm. We describe a rare case of zosteriform KS in a 28-year-old man positive for human immunodeficiency virus (HIV) who presented with purplish papules in a dermatomal distribution on his chest that were unresponsive to herpes zoster antivirals. We also discuss the characteristics and findings of the subtypes of KS and review the differential diagnosis for papular dermatomal lesions. Case Presentation A 28-year-old, HIV-positive Caucasian man presented to the emergency room with intractable superficial thoracic pain in a zosteriform distribution associated with red-to- purple papules that had been present for three months. Four months prior to admission, the patient was hospitalized for appendicitis and underwent laparoscopic appendectomy. Post- operatively, he experienced thoracic dermatomal pain and developed eruptive, grouped vesicles on an erythematous base along the T4 to T6 thoracic dermatomes. The patient was treated Figure 1 Figure 4 STEINMETZ-RODRIGUEZ, MILLS, SHECTER Page 40 present as well. Immunohistochemical stains There are four subtypes of endemic KS: nodular, Finally, epidemic KS, which occurs in HIV- were positive for CD31 (Figure 5), CD34 florid, infiltrative and lymphadenopathic.2 The positive patients, has lesions that appear (Figure 6), factor VII-RAg, HHV-8 and nodular form presents similarly to classic KS and predominately on the face, neck and upper vimentin, while desmin was negative. Complete also demonstrates a good prognosis. The florid trunk, not the lower legs as in classical KS. blood count was within normal limits. form is more aggressive, forming fungating Furthermore, the lesions appear as reddish- Computed tomography of the chest and right and exophytic tumors.6 The infiltrative form to-pink macules and papules, as clinically shoulder showed right-sided lymphadenopathy involves invasion of the subcutaneous tissue demonstrated in our patient, not the purplish and lesions on the liver. and bone. Finally, the lymphadenopathic macules seen in classical KS.2 Lesions on the form, associated with a 100% fatality rate, is oral mucosa, such as the hard and soft palates The findings were most consistent with KS. The common in young Bantu children and presents and genitalia, are not uncommon.6 Reports patient was evaluated by Oncology with plans to with diffuse lymphadenopathy and visceral also note involvement of the lymph nodes and initiate outpatient treatment with doxorubicin; organ involvement. Treatment options include gastrointestinal tract as seen on endoscopy, however, the patient was lost to follow-up. combination chemotherapy; however, in one with lesions present from the esophagus to report looking at three-year follow-up of the rectum and on the lungs, liver and spleen, Discussion patients with the florid or infiltrative type, only as seen in our patient.2 Lesions evolve from By the spring of 1981, 21 cases of fulminant and 14% remained diseased-free, and 36% had died. faint and macular in the early patch stage to disseminated KS in young homosexual or bisexual papules and plaques in the plaque stage, finally men in California and New York were described, Iatrogenic KS presents in patients with severe 6 forming large, elevated nodules in the nodular baffling physicians across the coasts. The sudden immunosuppression resulting from organ stage.6 Systemic symptoms such as fever, chills, and rapidly increasing occurrence of KS in young transplant, autoimmune disease, or systemic weight loss, diarrhea and fatigue often appear men heralded the epidemic of a new, devastating immunosuppressive therapy for malignancy.2 with the nodular stage. Treatment includes disease now known as AIDS. KS is a multifocal Initially described in renal transplant patients, highly active antiretroviral therapy (HAART), hemorrhagic neoplastic process arising from cases have also been reported in patients with intralesional therapy with vinblastine, radiation vascular and lymphatic endothelium. KS has four pemphigus vulgaris, bullous pemphigoid, 9 7 therapy and topical alitretinoin. Systemic clinical variants (Table 1). Wegener’s granulomatosis, or systemic lupus chemotherapy for extensive cutaneous lesions 6,8 erythematosus on immunosuppressive agents. or visceral involvement includes doxorubicin or Clinical Variants of Kaposi’s Sarcoma The average time for development of lesions is daunorubicin as first-line agents. Classic KS occurs most commonly in men 16 months after starting immunosuppressive over the age of 50 of European, Jewish or therapy, and the lesions most often remain Zosteriform Kaposi’s Sarcoma Mediterranean heritage. The lesions present localized to the skin.6 About 30% of patients will Zosteriform KS, as seen in our patient, is an as red-to-purple or blue papules, patches or die due to generalized KS, and tumor progression extremely rare clinical manifestation of KS, with nodules on the distal lower extremity. As is correlated to degree of immunosuppression. only three other cases reported in literature.7,10,11 the disease course progresses, lesions may Treatment options are difficult because the risk Herpes zoster is the most common eruption become ulcerated or fungated, and venous of death from organ rejection or autoimmune to follow a dermatomal distribution, giving stasis changes and lymphedema of the lower 6 illness from discontinuing immunosuppressive rise to the name zosteriform; however, other extremities are common. Prognosis is good, therapy must be weighed against the risk of cutaneous lesions have been reported to occur with an indolent course for 10 years to 15 years death from KS. in such a pattern, including papular