Human Herpesvirus 8–Associated Inflammatory Cytokine Syndrome

Abstract

logic features. In 2002, Weenig et al 1 described 95 patients with skin ulcers resembling those found in PG. They described 6 disease categories that can mimic PG: vascular disorders, vasculitis, malignant diseases, primary infections, drug-induced or exogenous tissue injury, and other inflammatory disorders. 1 Cutaneous diphtheria is caused by C diphtheriae and usually affects inhabitants or travelers from endemic developing countries. Coinfection with other pathogens such as Streptococcus pyogenes or A hemolyticum is frequent. 2,3 Cutaneous diphtheria presents as painful nodules or nonhealing ulcers, and it can be highly contagious. 4 Recently, an outbreak among refugees from Africa and Syria was reported in Germany and Switzerland. 5 Early diagnosis is essential for preventing outbreaks of this disease.Treatment of CD differs from systemic diphtheria. It is based on systemic antibiotics like penicillin and erythromycin and isolation precautions. The lack of response to amoxicillin/clavulanate in our patient could be explained by the occasional resistance to penicillin that has been reported. 6 We describe a patient with CD resembling PG clinically and histologically. Strikingly, the patient also presented with a monoclonal IgG band, making the PG diagnosis even more plausible. However, the presence of conditions associated with PG such as inflammatory bowel disease, myeloproliferative disorders, or paraproteinemia has been described in almost 40% of patients misdiagnosed as having PG. 1 Clinicians must remember that PG is a diagnosis of exclusion, and it is mandatory to perform biopsies, cultures, and blood analysis to rule out neoplasms, infections, vascular, or autoimmune disorders.

A Rare Case of the Newly Recognized Kaposi Sarcoma Herpesvirus-Associated Disease

“…Overall, recent case reports demonstrate a high mortality rate (about 60%) in patients diagnosed with KICS [1,4,5]. Awareness and a high index of suspicion in patients with pre-existing HIV and KS will lead to earlier diagnosis and prompt treatment [1,4,5,9]. Here we describe a case consistent with KICS, where the patient, unfortunately, succumbed to complications of multiorgan involvement in one month.…”

“…KICS also mimics severe sepsis and acute respiratory distress syndrome (ARDS), often requiring ventilator and vasopressor support, but antibiotics do not help. Overall, recent case reports demonstrate a high mortality rate (about 60%) in patients diagnosed with KICS [1,4,5]. Awareness and a high index of suspicion in patients with pre-existing HIV and KS will lead to earlier diagnosis and prompt treatment [1,4,5,9].…”

“…Uldrick et al initially described six patients with a co-infection of HIV and HHV8, who presented with classic KSHV-MCD signs and symptoms, however, without pathologic evidence or radiographic findings associated with MCD [4,6]. All of those patients had features of leak syndrome, resulting in pulmonary infiltrates, respiratory distress, and anasarca, along with significant systemic inflammatory responses as the hallmark of this syndrome [1].…”

“…Because of its rarity, KICS is easily misdiagnosed as severe sepsis or other KS-related disorders in HIV/AIDS patients and is, therefore, probably under-recognized [1]. KICS symptoms are nonspecific and almost identical to KSHV-MCD; in addition, its laboratory abnormalities resemble KSHV-MCD by hypoalbuminemia, hyponatremia, cytopenia, increased C-reactive protein, high KSHV viral load (VL), and elevated IL-6 and IL-10 [4].…”

“…Kaposi sarcoma herpesvirus (KSHV), a human gamma herpesvirus 8 (HHV8), is associated with Kaposi sarcoma (KS), primary effusion lymphoma (PEL), hemophagocytic lymphohistiocytosis (HLH), and multicentric Castleman disease (KSHV-MCD) in patients infected with human immunodeficiency virus (HIV) [ 1 - 3 ]. As a rare and unique entity, KSHV inflammatory cytokine syndrome (KICS) was first recognized in patients infected with HIV in 2010 and is distinct from KSHV-MCD [ 2 , 4 , 5 ].…”