King Anne L, Montagnon Carmen M, Todd Austin, Agrawal Shruti, Wieland Carilyn N, Lehman Julia S, Johnson Emma F
Department of Dermatology, Mayo Clinic, Rochester, MN.
Department of Dermatology, Vanderbilt Health, Nashville, TN.
Am J Dermatopathol. 2025 Feb 1;47(2):105-109. doi: 10.1097/DAD.0000000000002878. Epub 2024 Nov 5.
Maculopapular cutaneous mastocytosis (MPCM) is a rare disorder characterized by a pathologic accumulation of mast cells in the skin, which may or may not be accompanied by systemic mastocytosis. Diagnosis of MPCM on skin biopsy can be challenging because the findings may be subtle. Although mast cell density in MPCM has been reported, data informing a proposed cutoff for diagnosis and diagnostic criteria are limited.
We identified adult patients diagnosed with MPCM and urticarial tissue reaction/chronic urticaria on skin biopsy and compared the mast cell and eosinophil counts per 1 mm 2 in 10 cases each of MPCM, chronic urticaria, and normal skin from routine biopsies. All slides were stained with CD117, and CD117-positive mast cells were counted per 1 mm 2 using digital microscopy. Eosinophils were counted on hematoxylin and eosin-stained slides per 1 mm 2 using digital microscopy.
The median number of mast cells per 1 mm 2 was significantly higher in MPCM than in cases of urticaria and normal skin/control tissue (177.3 vs. 26.8 vs. 47.8 mast cell per mm 2 , respectively; P ≤ 0.001). The calculated "cut point" for mastocytosis versus chronic urticaria and normal skin was 66 mast cells per 1 mm 2 , whereas the value for controls versus urticaria was 37 mast cells per 1 mm 2 . Eosinophils had similar density in MPCM and urticaria, and their presence was significant in the differentiation of MPCM and urticaria from normal tissue.
This study adds to the literature by providing objective mast cell density data to distinguish challenging cases of cutaneous mastocytosis from urticarial reactions and normal skin. Future studies could explore the development of computer-aided estimations of cellular density with more extensive comparison with other inflammatory conditions to translate our findings more readily into clinical practice.
斑丘疹性皮肤肥大细胞增多症(MPCM)是一种罕见疾病,其特征为肥大细胞在皮肤中病理性积聚,可能伴有或不伴有系统性肥大细胞增多症。皮肤活检诊断MPCM具有挑战性,因为其表现可能不明显。尽管已有关于MPCM中肥大细胞密度的报道,但用于诊断的建议临界值和诊断标准的数据有限。
我们确定了经皮肤活检诊断为MPCM以及荨麻疹组织反应/慢性荨麻疹的成年患者,并比较了MPCM、慢性荨麻疹和常规活检的正常皮肤各10例中每1平方毫米的肥大细胞和嗜酸性粒细胞计数。所有玻片均用CD117染色,使用数字显微镜每1平方毫米计数CD117阳性肥大细胞。使用数字显微镜在苏木精和伊红染色的玻片上每1平方毫米计数嗜酸性粒细胞。
MPCM中每1平方毫米肥大细胞的中位数显著高于荨麻疹病例和正常皮肤/对照组织(分别为每平方毫米177.3个、26.8个和47.8个肥大细胞;P≤0.001)。肥大细胞增多症与慢性荨麻疹和正常皮肤的计算“切点”为每1平方毫米66个肥大细胞,而对照与荨麻疹的该值为每1平方毫米37个肥大细胞。MPCM和荨麻疹中嗜酸性粒细胞密度相似,其存在对于区分MPCM和荨麻疹与正常组织具有重要意义。
本研究通过提供客观的肥大细胞密度数据,将具有挑战性的皮肤肥大细胞增多症病例与荨麻疹反应和正常皮肤区分开来,从而丰富了文献资料。未来的研究可以探索计算机辅助细胞密度估计的发展,并与其他炎症性疾病进行更广泛的比较,以便更轻松地将我们的研究结果转化为临床实践。