Advertisement
Mayo Clinic Proceedings Home
MCP Digital Health Home

Scalp Necrosis in Giant Cell Arteritis

      A 67-year-old woman presented with a 2-month history of headache; jaw claudication, weight loss of 6 kg; right-hand ischemia; and scalp necrosis (Figure 1A and B ), which developed at the same time as her other symptoms. She had no visual symptoms and ophthalmic examination results were normal, with no occult visual arterial abnormalities suggestive of subclinical ischemia. Both temporal arteries were indurated on physical examination. Her C-reactive protein (CRP) level was at 92 mg/L.
      Figure thumbnail gr1
      Figure 1Scalp necrosis related to giant-cell arteritis.
      Duplex ultrasonography showed halo sign (dark halo around arterial lumen) on temporal arteries (Figure 2A) and inflammatory stenosis of right axillary artery (Figure 2B). Moreover, there was aortitis on computed tomography scan.
      Figure thumbnail gr2
      Figure 2Duplex ultrasonography showed halo sign (dark halo around arterial lumen) on a cross section of temporal artery (A) and inflammatory stenosis on longituinal section of right axillary artery (B).
      The patient refused temporal artery biopsy. The diagnosis of scalp necrosis as a complication of giant-cell arteritis (GCA) was made on high clinical suspicion of GCA (age above 50, headache, weight loss, biological inflammatory syndrome, and scalp necrosis) and 2 positive imaging tests; in this context, the diagnosis of GCA may be made without a biopsy.
      • Dejaco C.
      • Ramiro S.
      • Duftner C.
      • et al.
      EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice.
      Prednisone 1 mg/kg per day was started; symptoms improved quickly, with normalization of CRP and a complete healing of the scalp necrosis at 1 month.
      The differential diagnoses of scalp ulceration and crusting includes basal cell carcinoma, squamous cell carcinoma, herpes zoster, pustular dermatosis of the scalp, irritant contact dermatitis, pyoderma gangrenosum, and postradiation changes.
      Scalp necrosis related to GCA is rare. It is related to occlusive arterial vasculitis, causing tissue ischemia. Lesions respond to systemic corticosteroids, usually at higher doses (prednisone >40 mg/day), and recurrence may occur during steroid taper.
      • Currey J.
      Scalp necrosis in giant cell arteritis and review of the literature.
      Scalp necrosis could be associated to higher incidence of visual loss (32%)
      • Currey J.
      Scalp necrosis in giant cell arteritis and review of the literature.
      ,
      • Soderstrom C.W.
      • Seehafer J.R.
      Bilateral scalp necrosis in temporal arteritis: a rare complication of Horton’s disease.
      or tongue necrosis
      • Soderstrom C.W.
      • Seehafer J.R.
      Bilateral scalp necrosis in temporal arteritis: a rare complication of Horton’s disease.
      ,
      • Tsianakas A.
      • Ehrchen J.M.
      • Presser D.
      • et al.
      Scalp necrosis in giant cell arteritis: case report and review of the relevance of this cutaneous sign of large-vessel vasculitis.
      and is usually considered as a poor prognostic indicator in GCA, requiring urgent treatment. Therefore, we believe that tocilizumab, given its recent approval for the treatment of GCA, should be specifically evaluated in this situation.

      References

        • Dejaco C.
        • Ramiro S.
        • Duftner C.
        • et al.
        EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice.
        Ann Rheum Dis. 2018; 77: 636-643
        • Currey J.
        Scalp necrosis in giant cell arteritis and review of the literature.
        Br J Rheumatol. 1997; 36: 814-816
        • Soderstrom C.W.
        • Seehafer J.R.
        Bilateral scalp necrosis in temporal arteritis: a rare complication of Horton’s disease.
        Am J Med. 1976; 61: 541-546
        • Tsianakas A.
        • Ehrchen J.M.
        • Presser D.
        • et al.
        Scalp necrosis in giant cell arteritis: case report and review of the relevance of this cutaneous sign of large-vessel vasculitis.
        J Am Acad Dermatol. 2009; 61: 701-706