07 March 2023
: Case report 
AIDS-Related Kaposi Sarcoma Associated with Steroid-Unresponsive Periorbital Lymphedema that Responded to Chemotherapy
Challenging differential diagnosis, Diagnostic / therapeutic accidents, Unusual setting of medical care, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis)
Yuhao Zeng1ABCDEF, Rahul Prasad1ABCDEF, Rachel D. King1BDE, Rajshri Joshi1E, Jason Lane2BDE, Sterling Shriber1E, Faris Shweikeh1E, Yani Zhang3ADEF*DOI: 10.12659/AJCR.938801
Am J Case Rep 2023; 24:e938801
Table 1. Case reports on periorbital edema in AIDS-KS.
| Source | Age | Sex | Description of KS-associated lymphoedema | CD4 (cells/µL) | Viral load (copies/mL) | TIS staging | Therapy (duration) | Diagnosis | Outcome |
|---|---|---|---|---|---|---|---|---|---|
| Frans et al (1994) []25 | 56 | M | Generalized non-pitting edema, especially in the face (periorbital region) | 538 | – | T1, I0, S0 | INF-α | Lymphangio-matous variant of KS | “Complete regression of the edema” |
| Bossuyt et al (1995) []26 | 57 | M | Pronounced non-pitting edema, initial on the feet and lower leg and later spreading gradually to the hand and face | 528 | – | T1, I0, S0 | INF-α | Lymphangioma-Like variant of AIDS-KS | “Progressive regression of edema” |
| Zidar et al (1995) []6 | 27 | M | Severe periorbital and facial edema | – | – | T1, Ix, Sx | Doxorubicin, vincristine, bleomycin (every 2 weeks ×4 months) | AIDS-KS | “Resolved rapidly” |
| Schwartz et al (1995) []27 | 32 | M | Edematous face and periorbital ecchymosis below each eye | – | – | T1, Ix, S0 | – | Ecchymotic KS | – |
| Hengge et al (2000) []28 | 29 | M | Severe lymphoedema of lower extremities, anasarca, periorbital edema, and multiple sarcomatous hyperkeratotic and erosive lesions on the trunk, both legs, and feet | 15 | – | T1, I1, S0 | Bleomycin, vincristine and doxorubicin | AIDS-related hyperkeratotic KS | Partial remission of 1 year duration |
| Bautista et al (2001) []29 | 29 | M | Facial and periorbital edema | 110 | – | T1, I1, S0 | Doxorubicin (6 weeks) | AIDS-KS | “Substantially improved 6 weeks later” |
| Connick et al (2004) []30 | 35 | M | Face (periorbital) and neck swelling, starting during the 2 week of ART | 81 | >750,000 | T1, I1, S1 | Stavudine, lamivudine, lopinavir-ritonavir + paclitaxel (Start at 2 week for 10 weeks) | AIDS-KS | “At 10 weeks of ART, facial edema had resolved” |
| Feller et al (2008) []12 | 28 | M | Development of severe edema of the face after 3-month followup of his first clinic visit | – | – | T1, Ix, Sx | Refused any Tx | AIDS-KS | Deceased (within 3 weeks of onset of rapid progressive facial lymphoedema) |
| 55 | M | Pronounced edema of the face and the lower extremities | 12 | – | T1, I1, S1 | – | AIDS-KS | Deceased (7 days after presentation) | |
| Volkow et al (2008) []31 | 19 | M | After 2 weeks of prednisone for IRIS, growing facial edema in a matter of hours | 182 | 21,000 | T1, I1, S1 | Lamivudine, stavudine, indinavir and ritonavir + vincristine and bleomycin (when facial edema developed) | AIDS-KS | Resolved |
| Feller et al (2010) []32 | 11 | M | Rapid progressive facial swelling/lymphoedema | 14 | – | T1, I1, S1 | ART | AIDS-KS | Deceased (4 days after admission, a few hours after starting ART) |
| Patel et al (2012) []33 | 37 | M | Facial puffiness with periorbital edema with minimal skin findings | 227 | 159,000 | T1, I0, S1 | – | AIDS-KS | |
| Chabria et al (2016)[]34 | 51 | M | Edema 2 weeks after initiation of prednisone (20 mg daily) for KS-IRIS | 476 | < 10 | T1, I0, S0 | Tenofovir, emtricitabine, and raltegravir + liposomal doxorubin (At onset of facial edema) | Steroid-exacerbated AIDS cutaneous KS-IRIS | Improved |
| Call et al (2022)[]35 | 21 | M | Persistent, non-tender, bilateral periorbital edema with right eye swollen shut, bilateral lower extremity non-pitting edema (received multiple corticosteroid regimens from various hospitals due to rash) | 154 | 522 | T1, I1, S0 | ART + pegylated liposomal doxorubicin | Disseminated AIDS-KS | – |
| KS – Kaposi sarcoma; AIDS-KS – acquired autoimmune deficiency syndrome-Kaposi sarcoma; ART – anti-retroviral therapy; IRIS – immune reconstitution inflammatory syndrome; TIS – Tumor, Immune System, Systemic Illness staging system. | |||||||||