Giant Cell Arteritis (GCA) presenting as scalp necrosis
Griffin Laoise, NG Wan Lin, Ryan Sheila, Leonard Niamh, Doran John-Paul, Ramsay Bart
Rheumatology/ Dermatology Univeristy Hospital Limerick
GCA is a systemic inflammatory vasculitis affecting medium to large vessels. Prompt diagnosis can be challenging yet important to prevent complications such as visual loss by treatment with systemic steroids. Introduction of steroid sparing agents can be considered to reduce side effects of corticosteroids. Here we describe a challenging presentation of GCA treated with steroids and tocilizumab.
A 79-year-old man presented with a one month history of painful scalp ulceration. This was preceded by a low-grade headache and neck pain for 3 weeks for which he applied a non-steroidal anti-inflammatory gel. He denied other symptoms. He had a history of coronary artery disease and chronic obstructive airway disease. On physical examination, he had a large area of ulceration of the vertex of his scalp measuring 13.5 x 9.5cm with necrotic eschar (figure 1). The remainder of clinical examination was unremarkable. His erythrocyte sedimentation rate was 59 mm/hour.Skin biopsy of his scalp showed ulceration and acute inflammation of muscular blood vessels indicating vasculitis. Giant cells were not seen.
Despite topical therapy with clobetasol and oral minocycline and dapsone his ulceration failed to improve. A temporal artery biopsy was then preformed, as histopathology was non-specific on scalp biopsy. Granulomatous inflammation with mononuclear infiltration of medium sized vessels was seen and confirmed the diagnosis of Giant Cell Arteritis (GCA). He commenced on oral corticosteroids.
His ulceration improved slowly and some weeks into treatment, he developed severe bilateral leg pain which was confirmed as ruptured Achilles tendons on ultrasound scanning. He has been commenced on fortnightly tocilizumab with a plan to gradually reduce steroid therapy.
Scalp ulceration and necrosis are rare cutaneous complications of GCA and present a diagnostic challenge for the physician in the absence of typical symptoms. It suggests multi-vessel involvement as the scalp is a well vascularised area with 4 supplying arteries1 and is associated with a higher incidence of irreversible visual loss and tongue gangrene, than those with GCA and no scalp necrosis2. Adequate and prompt immunosuppression is therefore important. Our case demonstrates benefit of temporal artery biopsy in cases of diagnostic uncertainly and the potential musculoskeletal complications of systemic steroid therapy.
1.Drummer W, Zillikens D, Schulz A et al. Scalp necrosis in the temporal (giant cell) arteritis: implications for the dermatological surgeon. Clin Exp Dermatol 1996; 21: 154-8
2. Currey J. Scalp Necrosis in giant cell arteritis and review of the literature. Br J Rheumatol 1997; 36: 814-16.
Figure 1: Large are of scalp ulceration with necrotic eschar