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Facial disfigurement from Kaposi"s sarcoma in a woman living with HIV/AIDS in the HAART era
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  1. Antonio Jose Reyes1,
  2. Kanterpersad Ramcharan2,
  3. Stanley Giddings1,3 and
  4. Samuel Aboh1
  1. 1 Infectious Diseases Unit, San Fernando Teaching Hospital, San Fernando, Trinidad and Tobago
  2. 2 Medicine, SurgiMed Clinic, San Fernando, Trinidad and Tobago
  3. 3 Medicine, University of the West Indies, St Augustine, Trinidad and Tobago
  1. Correspondence to Dr Kanterpersad Ramcharan; kramcharan79{at}yahoo.com

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A 36-year-old woman presented with a progressive wasting syndrome, fatigue and disfiguring lesions on the skin of the face. There were bilateral multiple, firm, non-tender, violaceous, fungoid, nodular and plaque-type infiltrations of the periorbital regions, palate, gums, face, chest wall, upper limbs, abdomen and legs. Her body mass index was 16 kg/m2 with poor nutrition due to inability to swallow caused by oral–palatopharyngeal lesions and oral candidiasis (figure 1). The lesions infiltrated both eyelids, causing blindness due to inability to open the eyes. There was also cervical, axillary and inguinal lymphadenopathy. The systematic review was unremarkable, and the rest of the physical examination was normal.

Figure 1

Kaposi’s sarcoma causing disfigurement due to extensive fungoid, nodular and plaque types of infiltration of the face, periorbital regions bilaterally and the mouth. There is also swelling of the lips and oral candidiasis, with the sarcoma extending into the palate and gum regions with greenish purulent secretion.

The Venereal Disease Research Laboratory test was negative. HIV rapid test by ELISA and confirmatory testing for HIV were both positive. On admission, the HIV viral load was 1 200 542 RNA copies/mL and the CD4+ T-cell count was 50 cells/µL (reference values 410–1590). Laboratory studies revealed normal full blood count, renal function and liver function. Other medical investigations showed no other underlying diseases, such as infectious diseases, autoimmune, metabolic, rheumatological or endocrinological syndromes.

CT revealed normal brain and abdomen with circumferential enhancing thickening of the nasopharynx and oropharynx measuring up to 1.8 cm in the right side (figures 2 and 3). Biopsy of the skin lesions revealed spindle-cell neoplasm, which was positive for CD- 34 (figures 4 and 5). However, human herpesvirus 8 LAN-1 immunohistochemistry testing was not obtained due to unavailability. Clinical, laboratory, radiological and histopathological findings were consistent with a diagnosis of Kaposi’s sarcoma.

Figure 2

CT scan axial view image at C3 vertebral level showing circumferential thickening of the nasopharynx and oropharynx (red arrow) measuring up to 1.8 cm in the right side with narrowing of the pharyngeal space with the narrowest region measuring 0.7 cm.

Figure 3

CT scan sagittal view image showing thickening of the nasopharynx and oropharynx measuring up to 1.471 cm at C1 vertebral level, with narrowing of the pharyngeal space with the narrowest region measuring 0.7 cm. Notice the thickening of the palate extended to the uvula.

Figure 4

Tumour photomicrography: H&E staining with skin dermis infiltrated with blood vessel proliferation with elongated monomorphic spindle-shaped endothelial cells in different stages, with slit-like vascular channels containing erythrocytes (×20 magnification).

Figure 5

Tumour photomicrography: on immunohistochemistry, the tumour cells showed expression of CD34 with strong staining and diffuse positive (4+) (20X magnification).

Highly active antiretroviral therapy (HAART) (emtricitabine/tenofovir, atazanavi and ritonavir), opportunistic infections prophylaxis (trimethoprim/sulfamethoxazole, azithromycin and fluconazole) and symptom palliation were all initiated. The patient developed a fever (38.8°C), difficult breathing, cough and greenish sputum for 3 days and died suddenly on the third week of admission due to sepsis secondary to hospital acquired pneumonia. Rapid deterioration and demise following initiation of HAART in patients with Kaposi"s sarcoma, particularly those with visceral involvement, can be due to immune reconstitution inflammatory syndrome (IRIS). However, it would have been too early to demonstrate improving immunological function in her case. Our patient did not meet the criteria l for the diagnosis of IRIS.1 Vigilance for the onset of IRIS is essential when initiating HAART. The patient succumbed rapidly before we could have explored other therapeutic options. An autopsy was not performed.

Kaposi’s sarcoma, an AIDS-defining cancer that is caused by human herpesvirus 8, is characterised by cutaneous lesions that may occur alone or with visceral lesions of the oral cavity, gastrointestinal tract and respiratory system. The presence of multisystem lesions of Kaposi’s sarcoma has a poor prognosis.2 3 Differentiating Kaposi sarcoma from other vascular tumours, as well as other non-vascular spindle cell soft-tissue neoplasms, can be challenging because of the vast spectrum of vasoproliferative diseases that can mimic the disease.3 Kaposi sarcoma with extensive facial involvement as a first manifestation of HIV-AIDS is rare in the post-HAART era.

Learning points

  • Kaposi sarcoma (KS), first described by Moritz Kaposi in 1872, is a low-grade mesenchymal tumour that involves the blood and lymphatic vessels affecting primarily the skin, but it can manifest as disseminated disease.

  • Disfigurement of the face by KS is rare.

  • KS is a preventable and treatable disease with a good prognosis when diagnosed at an early stage.

  • Taking measures to avoid becoming infected with HIV or for those suffering from HIV-AIDS taking HAART could prevent most cases of KS.

  • Local therapy with alitretinoin or imiquimod creams, vinblastine, photodynamic therapy, and curettage and electrodesiccation of skin lesion can fail to halt the development of new KS lesions.

Acknowledgments

We thank Dr Ryan Rattan for his contribution with the histopathological diagnosis. We also thank Mr Simon Peters for his contribution with the technical preparation of the images.

References

Footnotes

  • Contributors AJR conceived and prepared the first draft. AJR,KR, SG and SA worked on subsequent revisions and the final manuscript. All authors assume responsibility for the final manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Next of kin consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.