# HIV-Associated Systemic Sclerosis: Literature Review and a Rare Case Report
## 2 Case Report  
A 44-year-old female patient was referred to our outpatient department (on 8 April 2021) for the complaint of mild, dull, intermittent pain, localized to the lower posterior teeth region, for the last 10-15 days. Her medical history reveals that she was diagnosed with HIV infection in 2012 (retro-positive from her husband). The patient is currently on a TLE regimen of antiretrovirals (tenofovir + lamivudine + efavirenz). Gradually, she developed tightening of the skin with reduced manual dexterity and was diagnosed with SSc in 2017. (Her laboratory investigations and treatment regimen are summarized in Table 1.)  
There was considerable weight loss with fluctuating jaundice since 2016, and her blood sugar values and blood pressure were within the normal range. Physical examination revealed that the patient was thin built and malnourished, calm, cooperative with normal intelligence, and responding to commands. Extraoral examination revealed smooth, tense, shiny, non-pinchable skin with a loss of wrinkles and wide-open eyes (mask-like facies). Lower palpebral conjunctiva elicited a pallor appearance. The skin over the forehead appeared dry and hyperpigmented with interspersed areas of hypopignmented macules (\"salt and pepper\" appearance). Atrophic nasal alae contributed to a sharp pinched appearance of the nose (mouse facies). Reduced oral aperture (microstonia) with circumoral radiating furrows (rhagades) and thinned lips resulted in a \"purse-string/fish mouth\" appearance (Figure 1A-C). The skin over the hands was hard, smooth, shiny, non-pinchable, and exhibited a \"salt and pepper\" appearance. Stiffened phalangeal joints on the fingers with reduced manual dexterity and mild resorption of the terminal phalanges were also observed (Figure 1D,E). \begin{table}
\begin{tabular}{c l l} \hline \hline
**S. No.** & **Investigation** & **Results** \\ \hline
**1.** & **HIV Status** & \\ \hline  & HIV Serology & HIV-1 + ve (retro + ve since 2012) \\  & TLE regimen of ART \\ \hline  & CD4 cell count & 440 cells/mm\({}^{3}\) \\ \hline  & HIV viral load & 2,214,173 copies/mL \\ \hline
**2.** & **Complete Blood Count (CBC)** & \\ \hline  & Hemoglobin (Hb) & 6.8 g/dL \\ \hline  & Total leucocyte count (TLC) & 4490 cells/\(\mu\)L \\ \hline  & Platelets & 1.3 lakh platelets/\(\mu\)L \\ \hline  & Erythrocyte sedimentation rate (ESR) & 85 mm/hr \\ \hline  & Bleeding time (BT) & 80 s \\ \hline  & Clotting time (CT) & 42 s \\ \hline  & International normalized ratio (INR) & 1.5 \\ \hline
**3.** & **Liver function test (LFT)** & \\ \hline  & Albumin & 2.3 g/dL \\ \hline  & Globulin & 1.0 g/dL \\ \hline  & Bilirubin & 2.6 mg/dL \\ \hline  & Alanine transaminase (ALT) & 71 units/L serum \\ \hline  & Aspartate aminotransferase (AST) & 33 units/L serum \\ \hline  & Alkaline phosphatase (ALP) & 672 IU/L \\ \hline  & Hepatitis B surface antigen (HBs Ag) & –ve \\ \hline  & Anti HCV antibody & –ve \\ \hline  & \(\alpha\)-Feto protein (AFP) & 34 ng/mL \\ \hline
**4.** & **Kidney function test (KFT)** & \\ \hline  & Na\({}^{+}\)/K\({}^{*}\) & 141/4.1 mEq/L \\ \hline  & Urea & 21 mg/dL \\ \hline  & Creatinine & 0.4 mg/dL \\ \hline  & Urine (R/M) & Normal range \\ \hline
**5.** & **Immunologic profile** & \\ \hline  & Antinuclear Antibodies (ANA) & 1:1000 (Intensity 4+) \\ \hline  & Extractable Nuclear Antigens (ENA) profile (ELISA) \\ \hline  & Anti ds-DNA autoantibody & –ve \\ \hline  & Ro (SSA) and La (SSB) autoantibody & –ve \\ \hline  & Anti Smantibodies & –ve \\ \hline  & Auto-RNP Ab & –ve \\ \hline  & Anti Scl-70- Ab & +ve (32.96 units/mL) \\ \hline  & Anti-mitrochondrial Ab (AMA) & +ve (suggestive of autoimmune hepatitis) \\ \hline  & Anti smooth muscle Ab (ASMA) & +ve (suggestive of autoimmune hepatitis) \\ \hline  & Anti cyclic citrullinated peptide & –ve \\ (Anti-CCP) & –ve \\ \hline  & Anti cerulopasmin Ab & –ve \\ \hline \hline \end{tabular}
\end{table} 
Table 1: Summary of investigations and treatment regimen.