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A woman with purple-brown skin lesions after immunosuppressive therapy

Published:October 11, 2022DOI:https://doi.org/10.1016/j.ejim.2022.10.007

      Keywords

      1. Case description

      An 80-year-old Greek woman presented to our clinic with fatigue and new-onset skin lesions around her neck, enlarging and multiplying over the previous 6 weeks. The lesions were asymptomatic but had been causing the patient considerable embarrassment. She had no fever, chills, night sweats or weight loss. Six months earlier, she had required treatment with corticosteroids and rituximab (375 mg/m2 of body-surface area per week for 4 weeks) for severe thrombocytopenia associated with autoimmune hemolytic anemia (Evans’ syndrome). She was physically active and her medical history was otherwise unremarkable. On examination, she had multiple violaceous, raised, and non-tender skin lesions on the neck, lower abdomen, and palms (Fig. 1). Oropharyngeal examination was unremarkable. Laboratory tests showed a normal full blood count, urea and electrolytes, liver-function tests, and C-reactive protein. Tests for HIV, hepatitis B, hepatitis C, antinuclear antibody, and anti-neutrophil cytoplasmic antibody were negative. Thoracic and abdominal CT scan did not reveal any enlargement of lymph nodes, spleen, or liver. No one in her family had a history of similar lesions.
      Fig 1
      Fig. 1Clinical photographs of skin lesions. (A) Multiple discrete, violaceous, raised lesions on the patient's neck and anterior chest that seem to follow skin-cleavage planes. The lesions were painless and non-pruritic, varying in size from several millimeters to several centimeters. (B) 1.5 × 0.5 cm non-tender, purple-brown cutaneous nodule on her right palm (arrow).

      2. What is your diagnosis?

      Immunosuppression-associated Kaposi's sarcoma.

      3. Discussion

      The most likely diagnosis is Kaposi's sarcoma (KS) as evidenced by the purple-brown skin lesions arising synchronously over widely dispersed areas, absence of fever, tempo of disease progression, history of immunosuppressive therapy, and geographic origin of the patient. A skin biopsy showed pleomorphic spindle-shaped cells which formed slit-like vascular spaces; these cells stained with anti-human herpesvirus 8 (HHV8) antibodies, consistent with KS.
      KS is a multifocal vascular tumor associated with HHV8. Four clinical variants exist: classic; endemic or African; immunosuppression-associated; and epidemic or AIDS-related. Before the HIV/AIDS epidemic, KS was chiefly seen in elderly men of Mediterranean origin, particularly in Italy and Greece, and in sub-Saharan Africa―i.e., areas with the highest frequencies of HHV8 [
      • Cesarman E
      • Damania B
      • Krown SE
      • Martin J
      • Bower M
      • Kaposi sarcoma Whitby D.
      ].
      In immunosuppression-associated KS, HHV8 is set free by a failure of immunosurveillance in patients receiving immunosuppressive therapies, particularly members of certain ethnic groups. In one study, KS developed in 12 organ-transplant recipients, 9 of whom were of Italian origin [
      • Shepherd FA
      • Maher E
      • Cardella C
      • et al.
      Treatment of Kaposi's sarcoma after solid organ transplantation.
      ]. The median interval from initiation of immunosuppression to KS is 16.5 months (range, 3-124). The clinical behavior is variable, ranging from the indolent to the fulminant. As seen here, immunosuppression KS may develop on the neck and progress rapidly; furthermore, KS should be considered in the differential diagnosis of lesions in the palm and fingers in immunosuppressed patients [
      • Naschitz JE
      • Lurie M.
      Macular palmo-plantar eruption.
      ]. This case also adds to growing evidence suggesting that rituximab can trigger or exacerbate KS [
      • Cesarman E
      • Damania B
      • Krown SE
      • Martin J
      • Bower M
      • Kaposi sarcoma Whitby D.
      ].
      The patient was treated with liposomal doxorubicin and all the lesions regressed. Physicians should maintain high clinical suspicion and seek early biopsy confirmation for KS in all immunosuppressed individuals.

      Funding Source

      None.

      CRedit authorship contribution statement

      all authors have contributed to the writing of the manuscript and review of the literature. All authors agree to the submission of this article to the European Journal of Internal Medicine.

      Patient consent

      the patient has provided consent.

      Declaration of Competing Interest

      No conflicts of interest.

      References

        • Cesarman E
        • Damania B
        • Krown SE
        • Martin J
        • Bower M
        • Kaposi sarcoma Whitby D.
        Nat Rev Dis Primers. 2019; 5: 9
        • Shepherd FA
        • Maher E
        • Cardella C
        • et al.
        Treatment of Kaposi's sarcoma after solid organ transplantation.
        J Clin Oncol. 1997; 15: 2371-2377
        • Naschitz JE
        • Lurie M.
        Macular palmo-plantar eruption.
        Eur J Intern Med. 2009; 20: e118-e119