Temporal arteritis with scalp ulceration and blindness

Temporal arteritis or giant cell arteritis is a systemic granulomatous vasculitis of medium and large‐sized arteries, most frequently involving the temporal artery. It presents with headache, fever, elevated erythrocyte sedimentation rate and anemia. If untreated, complications like ischemic optic neuritis may even lead to blindness. Although very rare, scalp necrosis and ulcerations are the most common dermatologic findings. An 85‐year‐old woman presented with a two‐month history of bilateral scalp ulcerations, followed by permanent loss of vision in the left eye due to the delay in diagnosing temporal arteritis.     

巨细胞动脉炎1例

报告1例巨细胞动脉炎。患者男，80岁。因头面部皮下结节伴疼痛1个月余入院。皮肤科检查；前额，双侧额部有条索状皮下结节，有轻微触痛，双眼视物模糊。皮损组织病理检查符合巨细胞动脉炎改变，给予糖皮质激素治疗后病情好转。     

A case of temporal arteritis successfully treated with recombinant interleukin-2

An 80-year-old Japanese woman with temporal arteritis was treated with systemic recombinant human interleukin-2 (IL-2) (1 x 10(6) unit/day for six weeks). The presenting symptoms of headache and skin necrosis and abnormal laboratory findings, such as an elevated erythrocyte sedimentation rate and CRP, promptly improved without any serious side effects. Although the pathogenesis of temporal arteritis and the mechanism(s) of the beneficial effect of IL-2 on it still remain unknown, this preliminary study highly encourages further investigations.     

Giant cell (temporal) arteritis involving both external and internal carotid arteries.

A 76-year-old woman with giant cell (temporal) arteritis was described; she presented with a one year history of headache and tinnitus. Histopathological findings from a superficial temporal artery showed arteritis with granulomatous changes. Bilateral carotid arteriograms demonstrated the stenoses of both internal carotid arteries as well as the narrowing of the superficial temporal arteries. Although we dermatologists rarely encounter the disease in daily clinical practice, it is of clinical importance to perform cerebral angiography in patients suspected of temporal arteritis.     

Temporal arteritis presenting with scalp necrosis and a normal erythrocyte sedimentation rate

A diagnosis of temporal arteritis is important to establish in order to reduce the incidence of blindness due to optic nerve ischaemia and other serious complications.¹ We describe a case in which the diagnosis was delayed as a result of a rare presentation and a normal erythrocyte sedimentation rate (ESR).     

Giant microcystic adnexal carcinoma of the scalp

Microcystic adnexal carcinoma (MAC) is an uncommon, locally aggressive tumor. It typically involves the upper lip of middle‐aged adults, and in rare instances the scalp. We report a Japanese woman with a giant MAC on the scalp. Physical examination revealed a 110 mm × 120 mm induration on her parietal region. Microscopically, the tumor showed both pilar and sweat gland differentiation. Resection included the cranium; for reconstruction we used a titan mesh allograft and covered it with a free latissimus dorsi muscle flap and a mesh skin graft. Ours is the first case of a MAC measuring more than 100 cm² arising on the scalp of an individual in the third decade of life.     

Bilateral scalp necrosis with giant cell arteritis

We present a patient with bilateral scalp necrosis caused by giant cell arteritis (temporal arteritis). A 67-year-old woman, who had been treated with 5 mg of oral prednisolone every other day for polymyalgia rheumatica, developed painful egg-sized regions of necrosis on both of her temples. Doppler pulsemetory revealed bilateral obstruction of the temporal arteries. Biopsy revealed ischemic necrosis of the skin and necrotic angiitis of the temporal arteries with giant cell infiltration. Bilateral stenosis of the internal carotid arteries and moderate retinal bleeding were revealed by angiography. Daily administration of prednisolone (20 mg/day) with intravenous and topical limaprost alphadex markedly improved her condition. The ulcers reepithelized without surgical treatment. There are few reports of bilateral scalp necrosis. Rapid and complete obstruction of the temporal artery may result in this condition. Simultaneous development of two ulcerative lesions in the ventro-parietal cranial regions is thought to correspond to systemic arterial involvement, including involvement of the internal carotid arteries.     

Macular lymphocytic arteritis: first clinical presentation with ulcers

Macular lymphocytic arteritis describes a recently reported entity, clinically characterized by asymptomatic hyperpigmented macules on the lower limbs, without association of systemic diseases. Histopathologically it is characterized by a lymphocytic arteritis with a hyalinized fibrin ring. We report a new case presenting with ulceration, a finding not previously described. A 25‐year‐old Hispanic woman was evaluated for a 1‐year history of a gradually progressive, asymptomatic eruption that begins at level of both knees and progressively affects both legs and feet. She also referred recently appeared ulcers on inner right ankle without previous traumatism. Physical examination revealed multiple fairly well‐defined light brown and faint pink patches with petechiae on as well as retiform crusts and livedoid lesions on inner right ankle. Both types of lesions were biopsied showing lymphocytic arteritis with fibrinoid necrosis and thrombus. There were no relevant laboratory alterations. The clinical peculiarity of our case is the clinical image of the lesions mimicking a pigmented purpuric dermatosis and the presence of a non‐traumatic ulcer which could be explained because chronic lymphocytic damage may cause ischemic damage. Ulceration in our case supports consideration of macular arteritis as a latent form of cutaneous polyarteritis nodosa.     

Dermoscopic follow‐up of therapeutic response in mantle cell lymphoma with secondary involvement of the scalp

The scalp is a potential location for both benign and malignant tumors. Lymphoproliferative diseases can involve the skin as a primary or secondary manifestation. Dermoscopy is a noninvasive diagnostic tool for rapid diagnosis, screening, and follow‐up of the majority of skin tumors. Mantle cell lymphoma (MCL), a rare type of aggressive systemic lymphoma, usually occurs as a generalized lymphadenopathy, commonly with infiltration of the bone marrow, spleen, gastrointestinal tract, and Waldeyer's ring. In rare cases, it can also involve other structures, such as the lungs, central nervous system, liver, or skin. We report the case of a 74‐year‐old male patient suffering from MCL since 2015. Complete remission was obtained after R‐CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) treatment. During maintenance therapy with rituximab, a solitary tumor occurred on the scalp. Dermoscopy of the lesion suggested relapse because of the presence of multiple chaotically distributed short linear vessels with multiple red dots within the hair follicles. Histological examination confirmed the diagnosis of MCL. After second‐line therapy with rituximab and bendamustine (R‐B), the tumor of the scalp completely disappeared and dermoscopy showed no abnormalities.     

Type 1 interferon signature in the scalp lesions of alopecia areata

Background Autoimmune attack of the bulbar region of anagen phase hair follicles by CD8+ T cells and Th1 cytokines has been proposed to result in hair loss in alopecia areata (AA). The initiating stimuli are unknown. As interferon‐α therapy may trigger AA, we propose that type 1 interferons are involved in the induction of disease. Objectives To compare lesional scalp from patients with AA with scalp lesions of cutaneous diseases associated with local type 1 interferon‐related protein expression. Methods Lesional scalp of patients with AA, discoid lupus erythematosus, lichen planopilaris and androgenetic alopecia was examined by immunohistochemistry for expression of the type 1 interferon‐inducible myxovirus protein A (MxA), the chemokine receptor CXCR3, and the cytotoxic proteins granzyme B (GrB) and T‐cell intracytoplasmic antigen 1 (TiA‐1). Results MxA was expressed in the intradermal and subcutaneous compartments of the hair follicle including sebaceous glands in inflammatory AA similar to lesions of cicatricial alopecia (discoid lupus erythematosus, lichen planopilaris) but not in the epidermal compartment of AA, and not at all in noninflammatory AA or androgenetic alopecia. The location of CXCR3‐expressing cells correlated with MxA expression. The inflammatory cells around the hair follicle in AA included a lower number of GrB+ and TiA‐1+ cells compared with cicatricial alopecia and demonstrated predominant TiA‐1+ expression. Conclusions We demonstrate the expression of type 1 interferon‐related proteins in the inflammatory lesions of AA. The distribution pattern of the interferon signature and cytotoxicity‐associated proteins in AA differs from cicatricial alopecia.     

Pyoderma gangrenosum associated with Takayasu's arteritis responding to cyclosporin

A 33-year-old caucasian woman with pyoderma gangrenosum associated with Takayasu's arteritis responded to treatment with cyclosporin. This patient is unusual in that both ulcerative and vesiculopustular forms of pyoderma gangrenosum were present. This has not previously been reported with Takayasu's arteritis.     

类风湿关节炎合并皮肤结核伴破溃一例

患者,女,64岁。双侧前臂、手背红肿破溃流脓8个月。既往有类风湿关节炎病史。皮损组织病理见抗酸染色阳性菌,考虑结核。综上诊断为类风湿关节炎并双侧前臂皮肤结核伴感染。给予利福平,阿莫西林克拉维酸钾,异烟肼,乙胺丁醇,吡嗪酰胺,双环醇及中药化腐清创术换药,病情逐渐好转。     

头皮局限性神经纤维瘤伴脱发一例

患者,男,22岁。脱发1年余,头皮棕褐色肿物6个月。病理可见毛囊微小化,真皮下部大量细长梭形瘤细胞,波浪状排列。S-100多克隆(+),SOX-10(+),MelanA(+),Vimentin-10(+)。诊断为头皮局限性神经纤维瘤。后行手术治疗,随访中。     

头皮小汗腺痣合并乳头状汗管囊腺瘤1例

报告1例罕见头皮小汗腺痣合并乳头状汗管囊腺瘤。患者男,78岁,出生时即发现头皮黄豆大小肿物。皮肤科检查：头皮顶部可见一肤色及玫瑰红色斑块。皮损组织病理检查示：部分表皮呈疣状增生,真皮深层可见大量结构正常的小汗腺腺体及导管;另一部分表皮向真皮凹陷形成囊状腔,囊状腔下部可见乳头状突起,乳头状突起由两排细胞组成,乳头状突起基质内可见较多浆细胞。诊断：头皮小汗腺痣合并乳头状汗管囊腺瘤。予手术切除皮损及皮瓣修复术。术后18个月电话随访无复发。     

伴结节性红斑的大动脉炎2例临床及组织病理分析并文献复习

报告2例伴结节性红斑的大动脉炎。例1.患者女,43岁。例2.患者女,21岁。二例患者均因双下肢反复出现结节性红斑就诊,其中例2有间断发热。皮肤科检查：二例患者双下肢均可见散在结节性红斑。二例患者皮损组织病理检查均提示血管周围少许单核细胞或淋巴细胞浸润,皮下脂肪间隔增宽,胶原增生,部分脂肪细胞变性坏死,呈混合性脂膜炎改变。计算机断层摄影血管造影术（CTA）提示2例患者血管管腔均有不同程度的狭窄,例1患者狭窄部位位于头臂干、双侧颈动脉及锁骨下动脉,例2患者位于左锁骨下动脉。诊断：大动脉炎。