Spider bite presenting as fever, macrophage activation syndrome and a skin ulcer
  • Marco Fedele
    Institute of Internal Medicine, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
  • Mariangela Antonelli
    Internal Medicine and Alcohol Related Disease Unit, Department of Medical and Surgical Sciences, Columbus-Gemelli Hospital, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
  • Egidio Carbone
    Institute of Internal Medicine, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
  • Marco Di Stefano
    Institute of Internal Medicine, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
  • Raffaele Manna
    Institute of Internal Medicine, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy; Periodic Fever and Rare Diseases Research Centre, Università Cattolica del Sacro Cuore, Rome, Italy
  • Giovanni Addolorato
    Internal Medicine and Alcohol Related Disease Unit, Department of Medical and Surgical Sciences, Columbus-Gemelli Hospital, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy; CEMAD Digestive Disease Centre, Department of Medical and Surgical Sciences, Hepatology and Gastroenterology Unit, Università Cattolica del Sacro Cuore, Rome, Italy

Keywords

Cutaneous ulcer, fever of unknown origin (FUO), loxoscelism, macrophage activation syndrome (MAS), primary cutaneous anaplastic large cell lymphoma (PC-ALCL)

Abstract

Introduction: Fever of unknown origin (FUO) refers to a condition of prolonged increased body temperature, without identified causes. The most common cause of FUO worldwide are infections; arthropod bites (loxoscelism) should be considered in view of the spread of the fiddleback spider. Loxoscelism can present in a cutaneous form (a necrotic cutaneous ulcer) or in a systemic form with fever, haemolytic anaemia, rhabdomyolysis and, rarely, macrophage activation syndrome (MAS). For this suspicion, it is important to have actually seen the spider.
Case description: A 71-year-old man was admitted to our department because of intermittent fever, arthralgia and a necrotic skin lesion on his right forearm that appeared after gardening. Laboratory tests were negative for infectious diseases, and several courses of antibiotics were administered empirically without clinical benefit. Whole-body computed tomography showed multiple colliquative lymphadenomegalies, the largest one in the right axilla, presumably of reactive significance. A shave biopsy of the necrotic lesion was performed: culture tests were negative and histological examination showed non-specific necrotic material, so a second skin and lymph node biopsy was performed. The patient developed MAS for which he received corticosteroid therapy with clinical/laboratory benefit. Cutaneous and systemic loxoscelism complicated by MAS was diagnosed. Subsequently, the second biopsy revealed morphological and immunophenotypic findings consistent with primary cutaneous anaplastic large cell lymphoma (PC-ALCL).
Conclusions: Skin lesions and lymphadenomegalies of unknown origin should always be biopsied. It is very common to get indeterminate results, but this does not justify not repeating the procedure to avoid misdiagnosis.

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References

  • Bryan CS. Fever of unknown origin: the evolving definition. Arch Intern Med 2003;163:1003–1004.
  • Wright WF, Wang J, Auwaerter PG. Investigator-determined categories for fever of unknown origin (FUO) compared with international classification of diseases-10 classification of illness: a systematic review and meta-analysis with a proposal for revised FUO classification. Open Forum Infect Dis 2023;10:ofad104.
  • Fusto G, Bennardo L, Duca ED, Mazzuca D, Tamburi F, Patruno C, et al. Spider bites of medical significance in the Mediterranean area: misdiagnosis, clinical features and management. J Venom Anim Toxins Incl Trop Dis 2020;26:e20190100.
  • Erat T, Yahsi A, Canakci C, Korkmaz A, Karahan C, Ileri T, et al. A rare cause of secondary hemophagocytic lymphohistiocytosis: systemic loxoscelism. Turk J Pediatr 2020;62:641–646.
  • Bennett RG, Vetter RS. An approach to spider bites. Erroneous attribution of dermonecrotic lesions to brown recluse or hobo spider bites in Canada. Can Fam Physician 2004;50:1098–1101.
  • Fardet L, Galicier L, Lambotte O, Marzac C, Aumont C, Chahwan D, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol 2014;66:2613–2620.
  • Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues, revised 4th edition, Vol 2. Lyon: International Agency for Research on Cancer (IARC); 2017.
  • Liu HL, Hoppe RT, Kohler S, Harvell JD, Reddy S, Kim YH. CD30+ cutaneous lymphoproliferative disorders: the Stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma. J Am Acad Dermatol 2003;49:1049–1058.
  • Yu JB, McNiff JM, Lund MW, Wilson LD. Treatment of primary cutaneous CD30+ anaplastic large-cell lymphoma with radiation therapy. Int J Radiat Oncol Biol Phys 2008;70:1542–1545.
  • Ardigò M, Marulli GC, Cota C, Mastroianni A, Berardesca E. Bexarotene and interferon-alpha combination therapy in a patient affected by relapsing anaplastic large cell lymphoma with cutaneous involvement. J Drugs Dermatol 2007;6:216–219.

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    Published: 2024-05-20
    Issue: 2024: Vol 11 No 6 (view)

    How to cite:
    1.
    Fedele M, Antonelli M, Carbone E, Di Stefano M, Manna R, Addolorato G. Spider bite presenting as fever, macrophage activation syndrome and a skin ulcer. EJCRIM 2024;11 doi:10.12890/2024_004440.

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