Case Presentation: A 36-year-old male with no known past medical history presented with 2-month history of facial swelling and progressively worsening dysphagia. This was associated with diffuse, painless, purplish skin lesions involving multiple body parts. On admission, patient was hemodynamically stable. Examination was remarkable for oral candidiasis, diffuse purplish nodules of varying sizes involving his face, bilateral upper and lower extremities, and trunk. Facial edema was noted as well. His blood work was notable for microcytic anemia with hemoglobin of 8.3, thrombocytopenia with platelet count of 96k. He subsequently tested positive for HIV. HIV-1 RNA was significantly elevated at 144,000. CD4 count was found to be low at 6 cells/mcL. In view of his facial swelling, and dysphagia, a CT soft tissue neck with IV contrast was obtained and revealed heterogeneously enhancing multinodular involvement of multiple mucosal structures from the nasopharynx to the aryepiglottic folds, especially involving the epiglottis, as well as increased density subcutaneous fat enhancing nodules in the scalp, and a left upper lobe irregular soft tissue density; this constellation was suggestive of Kaposi sarcoma. A punch biopsy of the skin lesion was done and showed atypical vascular proliferation consistent with patch stage Kaposi’s Sarcoma. Infectious disease and oncology services were consulted. Patient was subsequently started on antiretroviral therapy and chemotherapy.
Discussion: KS is a rare angio-proliferative disease that is seen mainly in patients with AIDS. It is associated with HHV-8 infection. KS typically presents with cutaneous disease. Cutaneous lesions of KS appear most often on the lower extremities, face (especially the nose), oral mucosa, and genitalia. Laryngeal involvement of KS is rare. Most patients have had AIDS-related KS, although HIV-negative persons with laryngeal KS have also been noted. When present, it can lead to severe airway compromise. Presenting symptoms may include hoarseness, throat discomfort, urge to cough, dysphagia, stridor or complete airway obstruction. Although a presumptive diagnosis of KS can be suggested by characteristic appearance of the lesions, this should be confirmed by a biopsy. CT scan of the larynx may help delineate laryngeal mass lesions. For HIV-positive patients who develop advanced KS, treatment includes combined antiretroviral therapy (ART) plus systemic chemotherapy.
Conclusions: KS is a vascular neoplasm commonly seen in patients with AIDS, as lesions on the skin. Larynx involvement is uncommon, and in patients without AIDS or a history of immune suppression, it is infrequent. It may be suspected in patients who present with violaceous lesions of the airway.