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Violaceous papules on the legs of an elderly Mediterranean man

Published:March 08, 2018DOI:https://doi.org/10.1016/j.ejim.2018.02.025

      Keywords

      1. Case report

      A 71-year-old Italian man with a remote history of multiple myeloma presented with dark spots on his bilateral legs and feet. The lesions had been present for many months and were not painful or pruritic. The patient had not tried any treatment, and the lesions did not spontaneously resolve. He was not on any systemic immunosuppressive medications.
      Physical examination of the lower extremities revealed scattered violaceous macules, papules, and plaques on the bilateral anterior shins, dorsal and plantar feet, and toes (Fig. 1). There was no surrounding erythema, edema, scaling, or tenderness to palpation. The patient had no palpable lymphadenopathy, and his oropharynx was clear. Complete blood count and basic metabolic panel were within normal limits.
      Fig. 1
      Fig. 1Scattered violaceous macules, papules, and plaques on the bilateral anterior shins (A), dorsal and plantar feet (A, B), and toes (C).

      2. What is the diagnosis?

      2.1 Diagnosis

      Answer: Kaposi's sarcoma (KS)
      KS is a vascular neoplasm characterized by abnormal angiogenesis. It is a multifocal disease with four principal clinical variants: (1) Classic or Chronic KS, (2) Epidemic or AIDS-related KS, (3) Endemic or African KS, and (4) Iatrogenic immunosuppression-associated KS [
      • Tappero J.W.
      • Conant M.A.
      • Wolfe S.F.
      • Berger T.G.
      Kaposi's sarcoma. Epidemiology, pathogenesis, histology, clinical spectrum, staging criteria and therapy.
      ]. Classic KS, as seen in this case, occurs most frequently in elderly men of Ashkenazi or Mediterranean descent. KS is a virally-induced disease and is caused by human herpesvirus-8 (HHV-8), which is required for disease manifestation. HHV-8 can be transmitted sexually as well as by salivary shedding and vertical transmission [
      • Schwartz R.A.
      • Micali G.
      • Nasca M.R.
      • Scuderi L.
      Kaposi sarcoma: a continuing conundrum.
      ].
      Clinically, cutaneous lesions vary from small violaceous macules and papules to large plaques and ulcerated nodules depending on the clinical variant and stage of disease. Classic KS favors the lower legs and feet, whereas AIDS-related KS is generally multifocal and widespread. KS can also involve the oral mucosa, lymph nodes, and viscera [
      • Tappero J.W.
      • Conant M.A.
      • Wolfe S.F.
      • Berger T.G.
      Kaposi's sarcoma. Epidemiology, pathogenesis, histology, clinical spectrum, staging criteria and therapy.
      ]. In this case, diagnosis was suspected based upon the appearance of the characteristic violaceous lesions and their distribution on the skin and was confirmed by skin biopsy.
      Classic KS typically has an indolent clinical course, as lesions often grow slowly, and patients usually die of unrelated causes [
      • Tappero J.W.
      • Conant M.A.
      • Wolfe S.F.
      • Berger T.G.
      Kaposi's sarcoma. Epidemiology, pathogenesis, histology, clinical spectrum, staging criteria and therapy.
      ]. In older immunocompetent patients with asymptomatic disease, observation and close follow-up is preferred. Ultimately, the major goals of management include improving function, lessening lymphedema, decreasing the size of lesions, and limiting disease progression [
      • Regnier-Rosencher E.
      • Guillot B.
      • Dupin N.
      Treatments for classic Kaposi sarcoma: a systematic review of the literature.
      ].

      Disclosures

      The authors have no conflicts of interest to disclose. The authors do not have grants or additional technical support to disclose. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. This manuscript is not under consideration elsewhere and has not been previously published.

      Authorship statement

      All authors have made substantial contributions to all of the following: (1) the conception and design of the study, or acquisition of data, or analysis and interpretation of data, (2) drafting the article or revising it critically for important intellectual content, (3) final approval of the version to be submitted.

      References

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        J Am Acad Dermatol. 1993; 28: 371-395
        • Schwartz R.A.
        • Micali G.
        • Nasca M.R.
        • Scuderi L.
        Kaposi sarcoma: a continuing conundrum.
        J Am Acad Dermatol. 2008; 59 ([quiz 207-178]): 179-206
        • Regnier-Rosencher E.
        • Guillot B.
        • Dupin N.
        Treatments for classic Kaposi sarcoma: a systematic review of the literature.
        J Am Acad Dermatol. 2013; 68: 313-331