Williams Ramone Faith, Nazarian Rosalynn M, Smith Gideon P
Division of Dermatology, Albert Einstein School of Medicine, Montefiore Medical Center.
Dermatol Online J. 2017 Oct 15;23(10):13030/qt6r0508bc.
We present the case of woman in her 50s who developed numerous red-brown telangiectatic macules on her trunk and extremities, as well as persistent dry eyes and dry mouth. Skin biopsy was consistent with telangiectasia macularis eruptiva perstans (TMEP). Serum tryptase was elevated suggesting systemic involvement. Anti-Ro and La were negative. ANA was positive. Salivary gland biopsy revealed a focus score of 3 and immunostains revealed infiltrates of aberrant CD117 positive mast cells. This case suggests a mechanistic role of mastocytosis in salivary compromise.
我们报告了一例50多岁的女性病例，该患者躯干和四肢出现大量红棕色毛细血管扩张性斑疹，同时伴有持续性干眼和口干。皮肤活检结果与持久性发疹性斑状毛细血管扩张症（TMEP）相符。血清类胰蛋白酶升高提示存在全身受累。抗Ro和La抗体为阴性。抗核抗体呈阳性。唾液腺活检显示灶性评分3分，免疫染色显示有异常CD117阳性肥大细胞浸润。该病例提示肥大细胞增多症在唾液腺损害中起机制性作用。
