Oral Kaposi's Sarcoma Research Articles

Bouche et infections virales

Exclude herpes infection in the presence of acute oral ulcers of unknown origin, particularly in patients in poor general condition. Remember that asymptomatic HSV-1 shedding in saliva may result in an oral-genital transmission. Perform an anogenital examination and a screening for other sexually transmitted diseases when oral warts are diagnosed. Search for immunosuppression and monitor the patient (screening for a potential associated carcinoma) when there is rapid growth of oral warts. Consider all the clinical signs (systemic, skin, other mucosa, immunity...) when a patient has an enanthem or oral ulcerations. Ask for a HIV test when an oral Kaposi's sarcoma, a hairy leukoplakia or major aphthae are diagnosed.

Quantification of oral palatine Langerhans cells in HIV/AIDS associated oral Kaposi sarcoma with and without oral candidiasis.

Langerhans cells (LCs) are effective antigen-presenting cells that function as "custodians" of mucosa, modifying the immune system to pathogen entry, and tolerance to self-antigen and commensal microbes. A reduction in number of LCs in human immunodeficiency virus (HIV)-positive individuals may predispose to local mucosal infections. To quantitatively determine the number of oral mucosal LCs in HIV/acquired immunodeficiency syndrome HIV/acquired immunodeficiency syndrome (AIDS) associated oral Kaposi sarcoma (KS) with/without oral candidiasis (OC) and to define in situ interrelationships between the cells, OC, and HIV infection. Thirty-two periodic acid-Schiff. (PAS) stained histologic sections of palatal HIV/AIDS associated KS with intact oral epithelium were examined for Candida and divided into two groups: . (1) KS coinfected with Candida and. (2) KS noninfected with Candida. Sections were immunohistochemically stained with CD1a. The standard length of surface epithelium was measured and number of positively stained LCs counted per unit length. Control cases included non-Candida infected palatal mucosa overlying pleomorphic adenoma. (PA) and oral mucosa infected with Candida in otherwise healthy individuals. LC number per unit length of surface epithelium was statistically significantly greatest in uninfected PA mucosa and lowest in KS coinfected with Candida (P = 0.0001). A statistically significant difference was also noted between uninfected PA mucosa and non-Candida infected KS (P = 0.0014), in KS coinfected with Candida and non-infected KS (P = 0.0035), between OC and PA (P = 0.0001), and OC and KS coinfected with Candida (P = 0.0247). LC numbers are significantly reduced in oral tissues of HIV/AIDS infected patients by Candida infection when compared to oral tissues without.

Treatment of Classic Kaposi's Sarcoma Showing a Discretely Scattered Distribution with Intralesional Vinblastine Injections

Dear Editor: Classic Kaposi's sarcoma (CKS) is one of clinical types of Kaposi's sarcoma (KS)1,2. Treatment options for CKS vary depending on clinical features such as age, number and distribution of lesions, and tumor depth3. It is essential, therefore, that treatment is tailored to the individual. Although many therapeutic strategies from systemic to local treatments are available for CKS, those treatments tend not to be suitable for cases with a discretely scattered distribution. Therefore, we report on a case of discretely scattered CKS treated successfully with intralesional vinblastine injections, which have previously proved effective and well-tolerated in acquired immune deficiency syndrome-related patients with localized oral KS lesions4. A 58-year-old Korean woman presented with a 5-year history of a skin lesion on her chin. Since that time of the initial skin lesion having appeared, subsequent lesions developed slowly on the upper and lower extremities. The patient was immunocompetent. Her past medical history was unremarkable except for the use of medication for dyslipidemia. A physical examination revealed multiple non-tender, round to elongated, red-purple papules and plaques (Fig. 1A), scattered over the chin, right hand, left forearm, and left shoulder. A skin biopsy of the chin was performed with immunohistochemistry stains (Fig. 2). Laboratory test results, including a complete blood cell count, thyroid function test, and anti-human immunodeficiency virus test, were all reported within normal limits. There was no evidence of metastasis on chest or abdominal computed tomography or on total body positron emission tomography imaging. We diagnosed this case as a discretely scattered CKS without visceral involvement. Although the patient had multifocal lesions on the head and limbs, the total number and size of the lesions had not changed in the last 3-months. To minimize the complications and considerable toxicities associated with the use of systemic chemotherapy or radiotherapy, we opted to treat our patient with intralesional vinblastine administration instead. All skin lesions were treated with intralesional vinblastine injections at a dose of 0.1 mg/cm2 using a 0.1 mg/ml solution of vinblastine sulfate in sterile saline4. After 5 weeks of treatment the tumors had completely regressed with no significant side effects except for a slight postinflammatory hyperpigmentation response (Fig. 1B). The patient was kept on regular follow-up and showed no signs of recurrence of CKS 6-months after treatment. Fig. 1 Clinical presentation of a discretely scattered classic Kaposi's sarcoma. (A) Widely distributed red-purple papules and plaques evident before treatment, and (B) complete regression of the tumors 5 weeks after treatment with intralesional vinblastine ... Fig. 2 Immunohistochemical evaluation of classic Kaposi's sarcoma specimens. (A) Endothelial cells of the abnormal vessels staining positively for cluster of differentiation 31; (B) Spindle cells staining positively for Friend leukemia virus integration-1; and ... CKS is usually indolent over many years but is not life threatening5. Consequently, clinicians have often hesitated in administering systemic therapy, which decreases quality of life, in patients with discretely scattered CKS. Our patient exhibited a dramatic response without serious side effects after a single cycle of intralesional vinblastine injections and had no additional lesions under outpatient follow-up. We conclude, therefore, that intralesional vinblastine administration is one of the most effective and useful treatment alternatives for relatively stable, discretely scattered CKS. However, the 6-month follow-up may not be sufficiently long when we consider the growth pattern of CKS and the potential for occult CKS lesions caused by hyperpigmentation, which were not histologically confirmed but need to be considered in estimating patient responses following intralesional vinblastine injections.

ORAL HIV-ASSOCIATED KAPOSI SARCOMA: A COMPARISON BETWEEN IMMUNOHISTOCHEMISTRY AND qPCR TECHNIQUES FOR DETECTION OF HHV8

Objective: To compare the diagnostic accuracy of immunohistochemistry compared to real-time PCR (using a simple phenol-chloroform DNA extraction protocol) in the detection of HHV8 in small oral biopsies of Kaposi sarcoma. Also to validate the use of this DNA extraction protocol to extract HHV8 DNA.Material and methods: Seventeen cases of oral KS were examined. Data including gender, age, and anatomic location were obtained from patient´s records and histological sections stained with hematoxylin and eosin (H&E) were reviewed. Immunohistochemistry was used to detect HHV8 in lesions of interest, as well as real-time PCR.Results: All the patients were HIV positive, the mean age was 35.5 years old, and the affected oral sites were palate (47%), gingiva (29.4%), tongue (11.8%), lip (5.9%), and oral floor (5.9%). Fifteen samples showed positive staining for HHV8 and only two samples were negative. The same results were observed using real-time PCR HHV8-DNA detection.Relevance: Our findings suggest that immunohistochemistry is faster and cheaper to perform than real-time PCR and was shown to have similar levels of sensitivity and accuracy for detection of HHV8 even in small biopsies. Additionally DNA extraction using a non-commercial kit, as done in this study can further reduce the costs to a pathology service.

Kaposi sarcoma in HIV-seronegative children presenting to the paediatric oncology ward in The Queen Elizabeth Central Hospital, Blantyre, Malawi during 2002-2014.

One of the most common malignancies in HIV-endemic, resource-poor countries is Kaposi sarcoma (KS). It is an AIDS-defining disease and as Malawi's incidence and prevalence of HIV is high, KS is now the most common cancer in adult male Malawians and the second most common in women and children. Most attention has focused on HIV-seropositive adults as their number far outweighs those of children. This audit concerns the presentation and outcome of HIV-seronegative children with KS who presented in a 12-year period (2002-2014) to The Queen Elizabeth Central Hospital. Twenty (10.5%) of the 191 children with KS presenting to the paediatric oncology ward during 2002-2014 were HIV-seronegative. They were usually younger than seropositive children and 62% had severe anaemia. The main presenting complaints in the HIV-seronegative group were woody oedema, commonly of a limb, and lymphadenopathy. Woody oedema was common in children with or without HIV infection. Seronegative children with KS were less likely to have oral KS than HIV infected children. Of 11 children who completed courses of chemotherapy, seven (63%) had complete cure sustained over a 1-year follow-up period. KS is potentially curable in this group of children. Chemotherapy regimens are equally effective in HIV-seropositive and HIV-seronegative children. The presentation of HIV-seronegative children with KS differs from adults and HIV-seropositive children. Further research is necessary to determine possible triggers for developing KS in HIV-seronegative children.

Co-Infection of Cytomegalovirus in Oral Kaposi's Sarcoma: A Case Report

Co-Infection of Cytomegalovirus in Oral Kaposi's Sarcoma: A Case Report

Diagnostic challenges of oral and cutaneous Kaposi's sarcoma in resource-constrained settings.

Kaposi's sarcoma (KS), caused by HHV-8, is the most frequent HIV-associated malignancy worldwide and remains a major scourge in Sub-Saharan Africa. KS is also endemic in much of Africa. There is a risk of misdiagnosis based solely on clinical appearance and haematoxylin and eosin (H&E) staining, especially with other reactive and neoplastic vascular proliferations which occur in the mouth. This study examined oral and cutaneous biopsies from clinically diagnosed lesions of KS in Kenya, using histopathology supplemented with immunohistochemistry (IHC) and polymerase chain reaction (PCR) for HHV-8 as confirmation of diagnosis. Biopsies of 49 lesions (28 oral, 21 cutaneous) previously diagnosed as 'KS' were re-examined by H&E staining and IHC targeting HHV-8 LANA-1. Positive controls were sections from embedded BCBL-1 cell lines. Negative controls were from three different HHV-8-negative biopsies. Confirmation of HHV-8 immunohistochemistry was sought by PCR and by determining the HHV-8 ORFK1 subtype. Whilst most cases were confirmed, 12 oral and 4 cutaneous lesions displayed clinical and histological features of KS but were negative to HHV-8 IHC. These oral lesions were re-diagnosed as pyogenic granulomata (n = 6), deep mycosis (n = 1), inflamed mucosa (n = 2) or 'uncertain but not KS' (n = 3). Whilst PCR is usually helpful in differentiating HHV-8 disease, all samples were HHV-8 PCR positive, with identical sequences, suggesting cross-contamination of samples in the original pathology laboratories. HHV-8 IHC is essential for the correct diagnosis of KS, but due to the high level of contamination in resource-poor settings, PCR is inadvisable.

Phenotypic Variability of Candida Species in Patients of Oral Lichen Planus and its Therapeutic Implications

DESMOPLASTIC KAPOSI SARCOMA A NEWLY RECOGNISED ORAL MUCOSAL VARIANT Belinda Kathleen Bunn, Willie Van Heerden, Department of Oral Pathology and Oral Biology, University of Pretoria, South Africa Objective: To distinguish desmoplastic Kaposi sarcoma (DKS) from other morphological variants of oral Kaposi sarcoma (OKS) and to investigate the possibility of a pathogenetic link to IgG-related disease. Study Design: Fourteen cases of DKS were reviewed and the clinicopathological features summarised. Immunohistochemical staining for SMA, IgG and IgG4 was performed. Results: Distinctive features of DKS include:

Mucocutaneous manifestations and the relationship to CD4 lymphocyte counts among Turkish HIV/AIDS patients in Istanbul, Turkey.

Dermatologic findings differ among countries but no sufficient data about Turkish HIV-infected patients exist in the literature. Therefore, our aim in this study was to document the dermatologic manifestations and their relationships with CD4 cell counts among HIV/AIDS patients visiting our clinic for the first time in Istanbul, Turkey. A retrospective analysis of 306 HIV/AIDS patients (260 men, mean age: 38.3 years) was done in a tertiary hospital in Istanbul from January 2006 to September 2012. Information on age, sex, transmission routes, socioeconomic and educational status, CD4 counts, and dermatologic findings was collected retrospectively from medical records. Our analyses revealed at least 1 dermatologic disease in 111 of the 306 (36.2%) patients. Mean CD4 count of the patients was 393.64 cells/mm3 (range: 4-1270 cells/mm3). Oral candidiasis (12.4%), herpes zoster (5.9%), dermatophytosis (5.4%), hyperpigmentation (5.2%), and folliculitis (4.6%) were the most common skin problems. Statistically significant correlation (P < 0.05) with low CD4 cell counts was found for oral candidiasis, folliculitis, herpes zoster, hyperpigmentation, xerosis, and Kaposi's sarcoma. Dermatologic manifestations in this study were identical to those described in most studies from Asia, and there were more manifestations as the HIV infection progressed and immune functions declined.

Association between oral candidiasis and low CD4+ count among HIV positive patients in Hoima Regional Referral Hospital.

BackgroundThe aim of this study was to determine the prevalence of Human Immune Virus (HIV) related oral lesions and their association with Cluster of Differentiation 4 (CD4+) count among treatment naïve HIV positive patients.MethodsThis was a descriptive and analytical cross sectional study. Participants were 346 treatment naïve HIV positive adult patients. These were consecutively recruited from Hoima Regional Referral hospital between March and April 2012. Data collection involved interviews, oral examinations and laboratory analysis.ResultsA total of 168(48.6%) participants had oral lesions. The four commonest lesions were oral candidiasis (24.9%, CI = 20.6-29.7%), melanotic hyperpigmentation (17.3%, CI = 13.7-21.7%), kaposi sarcoma (9.3%, CI = 6.6-12.8%) and Oral Hairy Leukoplakia (OHL) (5.5%, CI = 3.5-8.4%). There was significant association between oral candidiasis and immunosuppression measured as CD4+ less than 350 cells/mm3 (OR = 2.69, CI = 1.608-4.502, p < 0.001). Oral candidiasis was the only oral lesion significantly predictive of immunosuppression (OR = 2.56, CI = 1.52-4.30, p < 0.001) with a Positive Predictive Value (PPV) of 48.2%, Negative Predictive Value (NPV) of 74.3%, 38.1% sensitivity and specificity of 81.4%.ConclusionOral candidiasis can be considered as a marker for immunesuppression, making routine oral examinations essential in the management of HIV positive patients.

OI0345 Oral squamous cell carcinoma and epithelial dysplasia in HIV-infected individuals

HIV-infected individuals are at increased risk of oral cavity Kaposi sarcoma and non-Hodgkin lymphoma. However, it is suggested this patient population may also be at increased risk of oral cavity squamous cell carcinoma (OSCC). It remains unclear whether this is secondary to known risk factors (such as smoking and alcohol) or relates to HIV-specific factors, such as coinfection with human papillomavirus or Candida species, or the use of highly active antiretroviral therapy (HAART).

British HIV Association guidelines for HIV‐associated malignancies 2014

<scp>B</scp>ritish <scp>HIV</scp> Association guidelines for <scp>HIV</scp>‐associated malignancies 2014

Short-Chain Fatty Acids from Periodontal Pathogens Suppress Histone Deacetylases, EZH2, and SUV39H1 To Promote Kaposi's Sarcoma-Associated Herpesvirus Replication

Periodontal pathogens such as Porphyromonas gingivalis and Fusobacterium nucleatum produce five different short-chain fatty acids (SCFAs) as metabolic by-products. We detect significantly higher levels of SCFAs in the saliva of patients with severe periodontal disease. The different SCFAs stimulate lytic gene expression of Kaposi's sarcoma-associated herpesvirus (KSHV) dose dependently and synergistically. SCFAs inhibit class-1/2 histone deacetylases (HDACs) and downregulate expression of silent information regulator-1 (SIRT1). SCFAs also downregulate expression of enhancer of zeste homolog2 (EZH2) and suppressor of variegation 3-9 homolog1 (SUV39H1), which are two histone N-lysine methyltransferases (HLMTs). By suppressing the different components of host epigenetic regulatory machinery, SCFAs increase histone acetylation and decrease repressive histone trimethylations to transactivate the viral chromatin. These new findings provide mechanistic support that SCFAs from periodontal pathogens stimulate KSHV replication and infection in the oral cavity and are potential risk factors for development of oral Kaposi's sarcoma (KS). About 20% of KS patients develop KS lesions first in the oral cavity, while other patients never develop oral KS. It is not known if the oral microenvironment plays a role in oral KS tumor development. In this work, we demonstrate that a group of metabolic by-products, namely, short-chain fatty acids, from bacteria that cause periodontal disease promote lytic replication of KSHV, the etiological agent associated with KS. These new findings provide mechanistic support that periodontal pathogens create a unique microenvironment in the oral cavity that contributes to KSHV replication and development of oral KS.

Oral Kaposi's Sarcoma in HIV Patients: Report of Two Cases

Oral Kaposi's Sarcoma in HIV Patients: Report of Two Cases

Http://www.omicsonline.org/open-access/doc-i-just-cant-swallow-pills-hiv-infected-patients-and-pill-phagophobia-2155-6113.1000348.php?aid=32174

Introduction: Kaposi Sarcoma (KS) is a soft tissue malignancy that has been categorized into 4 subtypes, however, in the United States, it has frequently been noted in patients with HIV/AIDS and has thus become known as an AIDS-defining illness. When KS is found in the oral cavity, it is usually seen on the hard palate. Oral KS (OKS) is most commonly seen in patients with HIV/AIDS with CD4 counts below 200, and the disease is rarely reported outside of this patient population. HIV patients may be more likely to have KS with the decline of their immune function. Report of case: We report the case of a 31 year old African American male with a 2 year history of HIV who presented to the Otolaryngology clinic with a painful tongue lesion. His CD4 count never fell below 200, which makes this case an outlier among the epidemiology of KS. This case is unique in two different ways. The dorsum of the tongue is one of the least common manifestations of OKS and secondly OKS is usually associated with CD4 counts <200 per microliter, and this patient’s CD4 count stayed above 200 cells per microliter throughout his evaluation and treatment. The tongue lesion was causing dysphagia and odynophagia that was significant enough to cause him to seek medical care. Discussion: Despite the relatively adequate resources for HAART therapy among the American indigent population, some patients still decline this highly efficacious treatment and succumb to its now rare complications. Physicians must actively investigate suspicious oral lesions in HIV patients, particularly when there is a question about the compliance of HAART therapy or other concerning features. The oral cavity may be the initial manifestation site for HIV associated KS thus any suspicious lesion in sexually active patients should lead to testing for HIV.

Knowledge and Attitudes about HIV/AIDS of Students in H.P. Government Dental College and Hospital, Shimla, India

The purpose of this cross-sectional survey was to assess the knowledge and attitudes towards patients with HIV/AIDS among dental students in H.P. Government Dental College, Shimla, India. In November 2011, a survey was conducted of all the dental students of the college using a forty-five-item, self-administered questionnaire. The total mean knowledge score was 68.3 percent (good knowledge). The mean knowledge score was statistically higher in the clinical group than in the preclinical group. A majority of the students were aware of the association between HIV and oral candidiasis (89.1 percent), major aphthous (83.2 percent), and Kaposi's sarcoma (68.9 percent). Only 4.9 percent had professional attitudes about treating patients with HIV/AIDS. Male students had significantly fewer negative attitudes and higher positive attitudes than female students. The overall attitude score was significantly higher in the clinical group than in the preclinical group. Although a majority of the students had good knowledge, there were some inadequacies in their knowledge; those were more frequently seen in the preclinical students. It is important that dental students, as future dentists, develop not only the necessary practical skills but also knowledge and attitudes that will prepare them to treat patients with HIV/AIDS.

Gender differences in oral manifestations among HIV-infected Brazilian adults

The purpose of this study was to compare gender differences in the prevalence of oral lesions in HIV-infected Brazilian adults. A retrospective study was conducted of medical records from HIV/AIDS patients from 1993 to 2004. Oral lesions were only included in this study if definitively diagnosed through microscopic analysis, therapeutic test or according to EC-Clearing house criteria. A total of 750 men and 237 women were included in the study. Statistically significant differences were observed only for oral hairy leukoplakia, Kaposi sarcoma and lymphadenopathy (P < 0.01). However, a model of logistic regression showed that only oral hairy leukoplakia presented a significant association with gender and males had a significantly likelihood (four times higher than females) of presenting with this oral manifestation [OR 4.3 (95% CI: 1.39-13.36)]. These data shows that oral manifestations are less prevalent in females than in males, particularly oral hairy leukoplakia.

Expression of PROX‐1 in oral Kaposi's sarcoma spindle cells

The histogenesis of neoplastic spindle cells of Kaposi's sarcoma is still uncertain, but some studies consider it a lymphatic vessel differentiation. Prox-1 is a nuclear transcription factor that plays a major role during embryonic lymphangiogenesis, and it has been considered a specific and sensitive lymphatic endothelial cell marker. The aim of this study was to determine the expression of Prox-1 in oral Kaposi's sarcoma comparing the results with oral benign vascular tumors including capillary hemangiomas and pyogenic granulomas. Expression of Prox-1 and HHV-8 was evaluated by immunohistochemistry in 30 oral Kaposi's sarcoma, 5 oral capillary hemangiomas, and 10 oral pyogenic granulomas. The labeling index was expressed as the percentage of positive cells for each case studied. Statistical comparison was performed using the Wilcoxon-Mann-Whitney rank sum test. Twenty-eight (93.3%) and 30 oral Kaposi's sarcoma cases were positive for Prox-1 and HHV-8, respectively, while all oral benign vascular tumors were negative for these markers. The number of Prox-1 and HHV-8 oral Kaposi's sarcoma-positive cells increased significantly from patch/plaque to nodular histological stages. The expression of Prox-1 in the neoplastic spindle cells supports the view of a lymphatic differentiation in oral Kaposi's sarcoma. Prox-1 may also be involved in the pathogenesis of oral Kaposi's sarcoma as the number of positive spindle cells increased progressively from patch to nodular stages and could be eventually useful as an additional diagnostic tool for differential diagnosis between oral Kaposi's sarcoma and benign oral vascular lesions.

Oral Kaposi's sarcoma: a review and update

Kaposi's sarcoma (KS) is an important mucocutaneous neoplasm with four well-known clinicopathologic types. Involvement of the oral cavity may be seen in all variants but is most common with AIDS-KS. The latter may signal undiagnosed HIV infection. Its common association with disseminated disease has potentially important diagnostic and therapeutic implications. Oral KS (OKS) most often affects the hard and soft palate, gingiva, and dorsal tongue with plaques or tumors of coloration ranging from non-pigmented to brownish-red or violaceous. Its involvement ranges from an incidental finding to proliferative tumor formation that interferes with mastication. OKS needs to be distinguished clinically from other entities, including pyogenic granuloma, hemangioma, bacillary angiomatosis, and gingival enlargement caused by cyclosporine, a drug frequently used in recipients of organ transplantation. KS may flare as part of the immune reconstitution inflammatory syndrome in HIV patients or develop in the context of iatrogenic immunosuppression. Management, which may depend upon a variety of factors including the clinicopathologic type of KS and results of staging, ranges from no treatment to local measures such as intralesional vinblastine or systemic administration of cytotoxic chemotherapy for disseminated disease. Modification of immunosuppressive regimens often helps control post-transplant OKS but enhances the risk of graft rejection. Screening donors and recipients of organ transplants for HHV-8, with prophylactic treatment if infected as well as institution of sirolimus early after transplantation, are proposed strategies aimed at preventing post-transplant OKS.

Successful treatment of an HIV-positive patient with unmasking Kaposi's sarcoma immune reconstitution inflammatory syndrome

BackgroundKaposi's sarcoma (KS) continues to be the most common human immunodeficiency virus (HIV)-associated neoplasm with considerable morbidity and mortality. While lesions normally resolve upon initiation of antiretroviral therapy (ART), recrudescence or unmasking of KS lesions may occur as part of immune reconstitution inflammatory syndrome (IRIS). Treatment of unmasking KS-IRIS is not yet standardised. ObjectivesTo report the successful treatment of a patient with fulminating mucocutaneous unmasking KS-IRIS by maintaining ART and using pegylated liposomal doxorubicin (PLD). Study designThe patient, a 39-year-old HIV-positive male with no previous history of KS presented with a 2-week history of cutaneous and oral KS lesions that had disseminated rapidly over the preceding 4 days. The KS lesions appeared 8 weeks after recommencing ART. At the time of this presentation, his CD4+ count was 742cells/mm3 with a HIV viral load <400copies/ml. ART was maintained and treatment with PLD commenced. ResultsDespite the rapid dissemination of KS lesions, virus was undetectable in plasma. In a late-stage vasoformative lesion, immunohistochemistry (IHC) for human herpesvirus 8 (HHV-8) antigen was light and diffuse, with stippled deposits within endothelial cell nuclei. Virus extracted from the lesion was HHV-8 subtype A. The patient responded well to PLD, relapsed a year later, but after further PLD, has remained well for the following 5 years. ConclusionDespite the absence of HHV-8 viraemia, this is clearly a case of unmasking KS-IRIS. It demonstrates that this entity can be successfully treated by maintaining ART and administering PLD.