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An elderly woman with ulceronodular rash

Published:February 24, 2017DOI:https://doi.org/10.1016/j.ejim.2017.02.017

      1. Introduction

      An 86-year-old woman affected by mild cognitive impairment presented to our institution with complaints of intense headache, vomiting and fever since 2 days, complicating a 4-month history of mucosal ulcerations and progressive papulopustular skin lesions of the whole body. She had been diagnosed with scleroatrophic lichen, and a biopsy of the tongue had shown epithelial ulceration and an intense perivascular inflammatory infiltrate of the lamina propria rich in plasma cells. On examination, the patient was febrile but alert; no neck stiffness or change in mental status were present. Skin lesions consisted of multiple erythematous, keratotic papules and nodules, some of which ulcerated and covered by adherent crusts. The lesions ranged from 5 to 20 mm in size and involved the face, the trunk and the extremities. The lumbosacral region was interested by a large eczematous-like plaque, of 70 × 40 mm. Examination of soles showed faint, scaly maculopapules; palms were spared. The tongue presented coalescent ulcerated, painless lesions covered by whitish pseudomembranes; the anovulvar region was interested by keratotic and ulcerated papules and plaques (Fig. 1).
      Fig. 1
      Fig. 1Disseminated papules and nodules, some of which ulcerated and covered by crusts (A–D). Eczematous-like plaque in the lumbosacral region (D). Coalescent ulcerated lesions of the tongue (E). Maculopapules of the sole (F).
      What is the diagnosis?

      2. Diagnosis

      Treponema pallidum hemagglutination assay (TPHA) tested positive with a titer of 1:10,240, venereal disease research laboratory (VDRL) was strongly reactive. According to clinical, hysthological and serological findings, a diagnosis of malignant syphilis was posed. A concomitant neurosyphilis was excluded by cerebrospinal fluid examination. Serology for HIV tested negative. The patient was treated with a 14-day course of ceftriaxone, achieving rapid recovery and a complete healing of skin and mucosal lesions within 30 days. Anamnestic investigation revealed a marriage relationship with a much younger man, aged 51 years, who was recognized as the probable source of infection.
      Malignant syphilis (ulceronodular syphilis) is a rare and severe form of secondary syphilis mainly characterized by the presence of exuberant ulceronecrotic lesions [
      • Requena C.B.
      • et al.
      Malignant syphilis in an immunocompetent female patient.
      ,
      • Fisher D.A.
      • Chang L.W.
      • Tuffanelli D.L.
      Lues maligna. Presentation of a case and a review of the literature.
      ]. It is more frequently encountered in HIV-infected persons, even though it has been observed in other immunocompromised patients, as well as in otherwise healthy subjects [
      • Requena C.B.
      • et al.
      Malignant syphilis in an immunocompetent female patient.
      ]. Affected individuals present a highly reactive syphilis serology and an optimal response to antibiotic treatment [
      • Requena C.B.
      • et al.
      Malignant syphilis in an immunocompetent female patient.
      ,
      • Fisher D.A.
      • Chang L.W.
      • Tuffanelli D.L.
      Lues maligna. Presentation of a case and a review of the literature.
      ]. Original diagnostic criteria include a severe Jarisch-Herxheimer reaction [
      • Fisher D.A.
      • Chang L.W.
      • Tuffanelli D.L.
      Lues maligna. Presentation of a case and a review of the literature.
      ].
      In European countries the overall rates of syphilis have increased in the past decade, warranting renewed attention to this infection [
      • European Centre for Disease Prevention and Control
      Annual Epidemiological Report 2016 – Syphilis.
      ]. The present report confirms the need to keep awareness of this protean disease, and to consider it in differential diagnosis whenever a patient presents with ulceronodular rash, irrespective of age at onset and presence of clear risk factors.

      Conflict of interests

      The authors state that they have no conflicts of interest.

      References

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        • et al.
        Malignant syphilis in an immunocompetent female patient.
        An Bras Dermatol. 2014; 89: 970-972
        • Fisher D.A.
        • Chang L.W.
        • Tuffanelli D.L.
        Lues maligna. Presentation of a case and a review of the literature.
        Arch Dermatol. 1969; 99: 70-73
        • European Centre for Disease Prevention and Control
        Annual Epidemiological Report 2016 – Syphilis.
        ECDC, Stockholm2016