D
CP
CED
CPD • Clinicopathological case
Clinical and Experimental Dermatology
Painful scalp ulceration M. Tsakok,1 O. Espinosa de los Monteros,2 R. Turner1 and B. Esdaile1 Departments of 1Dermatology and
2
Dermatopathology, Churchill Hospital, Oxford, UK
doi: 10.1111/ced.12283
Clinical findings A 79-year-old woman presented with a 1-month history of rapidly progressing, tender scalp ulceration. Three months before the onset of ulceration, she had presented to her general practitioner with unsteadiness, vomiting, and headache that was mainly focused around her left temporal and occipital regions. She also described feeling generally unwell, with anorexia and weight loss. Computed tomography of her head performed at that point did not reveal any abnormality. She was referred to our skin cancer screening clinic to exclude a malignancy. On physical examination, there were serpiginous necrotic areas of ulceration across the occipital region of the patient’s scalp (Fig. 1). The rest of her skin examination was unremarkable, with no splinter haemorrhages or digital nail infarcts seen. Visual examination was normal. Laboratory investigations showed a raised erythrocyte sedimentation rate (ESR) of 98 mm/h (normal range for a female aged > 50 years: < 30 mm/h).
Figure 1 Area of ulceration on occipital scalp.
Histopathological findings On histological examination of a biopsy taken from the edge of an area of ulceration, a single vessel was seen in the subcutis, with a lymphocytic infiltrate in its wall (Fig. 2). This was seen only in one of the sections examined. A biopsy of the patient’s left temporal artery showed transmural chronic inflammation with fragmentation of the internal elastic lamina and hypertrophy of the tunica intima. Giant cells were identified in the vicinity of the internal elastic lamina, Correspondence: Dr Ben Esdaile, Department of Dermatology, Churchill Hospital, Oxford, OX3 7LE, UK E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 27 October 2013
418
Clinical and Experimental Dermatology (2014) 39, pp418–419
Figure 2 A subcutaneous vessel with mural lymphocytic infiltra-
tion (haematoxylin and eosin, original magnification 920).
highlighted with immunohistochemical staining for CD68 (PGM-1) antigen. What is your diagnosis?
ª 2014 British Association of Dermatologists
D CP Clinicopathological case
Diagnosis Giant cell arteritis (GCA) with the rare feature of scalp ulceration.
Discussion GCA, also known as temporal arteritis, is a wellknown systemic granulomatous vasculitis of the medium and large arteries, with a predilection for the extracranial branches of the carotid artery. The inflammatory cascade, with thrombosis formation and resultant arterial occlusion, gives rise to the typical ischaemic symptoms of headache, scalp tenderness and jaw claudication. These findings prompt urgent investigation and treatment to prevent the muchfeared complication of visual loss, caused by occlusion of the posterior ciliary arteries that supply the optic nerve head (anterior ischaemic optic neuropathy). The non specific symptoms and signs of malaise, fever, sweating, arthralgia, weight loss and anaemia often accompany this presentation. Scalp necrosis is an extremely rare but dramatic complication of GCA.1–6 It is associated with a much higher incidence of visual loss (67%)5,6 and with other severe complications such as claudication, stroke, myocardial infarction and tongue gangrene. Indeed, a high mortality rate of 38%4 has been reported to occur in patients with GCA accompanied by scalp ulceration. Necrosis occurs when the four arteries (arteriae temporalis, frontalis, etroauricularis and occipitalis) irrigating the temporal region of the scalp become occluded. The probability of necrosis is likely to be determined by the degree of anastomosis between these vessels and the number of occluded vessels. The average time between symptom onset and scalp ulceration is 2.9 months.6 Furthermore, no case of scalp ulceration appearing after initiation of treatment has been documented, suggesting that earlier initiation of treatment may serve to prevent this complication. Our patient responded well to the standard treatment with prednisolone 60 mg daily for 1 month,
ª 2014 British Association of Dermatologists
which improved her headaches and has allowed for slow healing of the areas of ulceration. The plan is to gradually taper her steroids with the addition of a steroid-sparing agent to try to prevent relapses. This case highlights that although skin features occur rarely in the clinical course of GCA, it may fall to the dermatologist to make the diagnosis when earlier recognition has failed or when the classic signs and symptoms of GCA are absent. If the level of suspicion is high, treatment should be instigated without delay, as it can be sight- and even life-saving.
Learning points
•
Scalp necrosis and ulceration is a rare feature of GCA. • It is associated with a high incidence of visual loss. • Treatment with oral steroids should be instigated promptly, with a gradual tapering dose over 1–2 years to avoid relapses.
References 1 Monteiro C, Fernandes B, Reis J et al. Temporal arteritis presenting with scalp ulceration. J Eur Acad Dermatol Venereol 2002; 16: 615–17. 2 Campbell FA, Clark C, Holmes S. Scalp necrosis in temporal arteritis. Clin Exp Dermatol 2003; 28: 488–90. 3 Abdullah AN, Keczkes K, Wyatt EH. Skin necrosis in giant cell (temporal) arteritis: report of three cases. Br J Dermatol 1989; 120: 843–6. 4 Soderstrom CW, Seehafer JR. Bilateral scalp necrosis in temporal arteritis: a rare complication of Horton’s disease. Am J Med 1976; 61: 541–6. 5 Currey J. Scalp necrosis in giant cell arteritis and review of the literature. Br J Rheumatol 1997; 36: 814–16. 6 Tsianakas A, Ehrchen J, 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: 701–6.
Clinical and Experimental Dermatology (2014) 39, pp418–419
419
CP
CED
CPD • Clinicopathological case
Clinical and Experimental Dermatology
Painful scalp ulceration M. Tsakok,1 O. Espinosa de los Monteros,2 R. Turner1 and B. Esdaile1 Departments of 1Dermatology and
2
Dermatopathology, Churchill Hospital, Oxford, UK
doi: 10.1111/ced.12283
Clinical findings A 79-year-old woman presented with a 1-month history of rapidly progressing, tender scalp ulceration. Three months before the onset of ulceration, she had presented to her general practitioner with unsteadiness, vomiting, and headache that was mainly focused around her left temporal and occipital regions. She also described feeling generally unwell, with anorexia and weight loss. Computed tomography of her head performed at that point did not reveal any abnormality. She was referred to our skin cancer screening clinic to exclude a malignancy. On physical examination, there were serpiginous necrotic areas of ulceration across the occipital region of the patient’s scalp (Fig. 1). The rest of her skin examination was unremarkable, with no splinter haemorrhages or digital nail infarcts seen. Visual examination was normal. Laboratory investigations showed a raised erythrocyte sedimentation rate (ESR) of 98 mm/h (normal range for a female aged > 50 years: < 30 mm/h).
Figure 1 Area of ulceration on occipital scalp.
Histopathological findings On histological examination of a biopsy taken from the edge of an area of ulceration, a single vessel was seen in the subcutis, with a lymphocytic infiltrate in its wall (Fig. 2). This was seen only in one of the sections examined. A biopsy of the patient’s left temporal artery showed transmural chronic inflammation with fragmentation of the internal elastic lamina and hypertrophy of the tunica intima. Giant cells were identified in the vicinity of the internal elastic lamina, Correspondence: Dr Ben Esdaile, Department of Dermatology, Churchill Hospital, Oxford, OX3 7LE, UK E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 27 October 2013
418
Clinical and Experimental Dermatology (2014) 39, pp418–419
Figure 2 A subcutaneous vessel with mural lymphocytic infiltra-
tion (haematoxylin and eosin, original magnification 920).
highlighted with immunohistochemical staining for CD68 (PGM-1) antigen. What is your diagnosis?
ª 2014 British Association of Dermatologists
D CP Clinicopathological case
Diagnosis Giant cell arteritis (GCA) with the rare feature of scalp ulceration.
Discussion GCA, also known as temporal arteritis, is a wellknown systemic granulomatous vasculitis of the medium and large arteries, with a predilection for the extracranial branches of the carotid artery. The inflammatory cascade, with thrombosis formation and resultant arterial occlusion, gives rise to the typical ischaemic symptoms of headache, scalp tenderness and jaw claudication. These findings prompt urgent investigation and treatment to prevent the muchfeared complication of visual loss, caused by occlusion of the posterior ciliary arteries that supply the optic nerve head (anterior ischaemic optic neuropathy). The non specific symptoms and signs of malaise, fever, sweating, arthralgia, weight loss and anaemia often accompany this presentation. Scalp necrosis is an extremely rare but dramatic complication of GCA.1–6 It is associated with a much higher incidence of visual loss (67%)5,6 and with other severe complications such as claudication, stroke, myocardial infarction and tongue gangrene. Indeed, a high mortality rate of 38%4 has been reported to occur in patients with GCA accompanied by scalp ulceration. Necrosis occurs when the four arteries (arteriae temporalis, frontalis, etroauricularis and occipitalis) irrigating the temporal region of the scalp become occluded. The probability of necrosis is likely to be determined by the degree of anastomosis between these vessels and the number of occluded vessels. The average time between symptom onset and scalp ulceration is 2.9 months.6 Furthermore, no case of scalp ulceration appearing after initiation of treatment has been documented, suggesting that earlier initiation of treatment may serve to prevent this complication. Our patient responded well to the standard treatment with prednisolone 60 mg daily for 1 month,
ª 2014 British Association of Dermatologists
which improved her headaches and has allowed for slow healing of the areas of ulceration. The plan is to gradually taper her steroids with the addition of a steroid-sparing agent to try to prevent relapses. This case highlights that although skin features occur rarely in the clinical course of GCA, it may fall to the dermatologist to make the diagnosis when earlier recognition has failed or when the classic signs and symptoms of GCA are absent. If the level of suspicion is high, treatment should be instigated without delay, as it can be sight- and even life-saving.
Learning points
•
Scalp necrosis and ulceration is a rare feature of GCA. • It is associated with a high incidence of visual loss. • Treatment with oral steroids should be instigated promptly, with a gradual tapering dose over 1–2 years to avoid relapses.
References 1 Monteiro C, Fernandes B, Reis J et al. Temporal arteritis presenting with scalp ulceration. J Eur Acad Dermatol Venereol 2002; 16: 615–17. 2 Campbell FA, Clark C, Holmes S. Scalp necrosis in temporal arteritis. Clin Exp Dermatol 2003; 28: 488–90. 3 Abdullah AN, Keczkes K, Wyatt EH. Skin necrosis in giant cell (temporal) arteritis: report of three cases. Br J Dermatol 1989; 120: 843–6. 4 Soderstrom CW, Seehafer JR. Bilateral scalp necrosis in temporal arteritis: a rare complication of Horton’s disease. Am J Med 1976; 61: 541–6. 5 Currey J. Scalp necrosis in giant cell arteritis and review of the literature. Br J Rheumatol 1997; 36: 814–16. 6 Tsianakas A, Ehrchen J, 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: 701–6.
Clinical and Experimental Dermatology (2014) 39, pp418–419
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