Department of Pathology, Department of Laboratory Medicine, Iida Municipal Hospital, Shinshu University Hospital, Iida, Matsumoto, Japan.
Department of Laboratory Medicine, Shinshu University Hospital, Matsumoto, Japan.
J Eur Acad Dermatol Venereol. 2023 Oct;37(10):2124-2132. doi: 10.1111/jdv.19284. Epub 2023 Jul 4.
Acquired idiopathic generalized anhidrosis (AIGA) leads to heat intolerance due to the loss or reduction in thermoregulatory sweating over an extensive area of the body. The pathomechanism of AIGA is still unclear but is believed to be autoimmune.
We investigated the clinical and pathological features of inflammatory AIGA (InfAIGA) and noninflammatory AIGA (non-InfAIGA) within the skin.
We compared anhidrotic and normohidrotic skin samples from 30 patients with InfAIGA and non-InfAIGA, as well as skin samples of melanocytic nevus as a negative control. We conducted morphometric analysis and immunohistochemical analysis of cell types and expression of inflammatory molecules (TIA1, CXCR3 and MxA). MxA expression was used as a proxy for type 1 interferon activity.
We found that tissue samples from patients with InfAIGA exhibited inflammation within the sweat duct and atrophy of the sweat coil, whereas patients with non-InfAIGA exhibited only atrophy of the sweat coil. Cytotoxic T lymphocyte infiltration and MxA expression were only observed in the sweat ducts of patients with InfAIGA.
InfAIGA is associated with increased sweat duct inflammation and sweat coil atrophy, whereas non-InfAIGA is only associated with sweat coil atrophy. These data suggest that inflammation leads to epithelial destruction of sweat ducts associated with the sweat coil atrophy and subsequent loss of function. Non-InfAIGA may be regarded as a postinflammatory state of InfAIGA. These observations indicate the contribution of both type 1 and type 2 interferons to sweat gland injury. The mechanism involved is similar to the pathomechanism of alopecia areata (AA).
获得性特发性全身性无汗症（AIGA）导致广泛身体区域的体温调节性出汗丧失或减少，从而导致耐热性下降。AIGA 的发病机制尚不清楚，但被认为是自身免疫性的。
我们研究了皮肤内炎症性 AIGA（InfAIGA）和非炎症性 AIGA（non-InfAIGA）的临床和病理特征。
我们比较了 30 例 InfAIGA 和 non-InfAIGA 患者的无汗和正常汗皮肤样本，以及作为阴性对照的黑素细胞痣皮肤样本。我们进行了细胞类型和炎症分子（TIA1、CXCR3 和 MxA）表达的形态计量分析和免疫组织化学分析。MxA 表达被用作 1 型干扰素活性的替代物。
我们发现，来自 InfAIGA 患者的组织样本表现出汗腺内炎症和汗盘管萎缩，而来自 non-InfAIGA 患者的组织样本仅表现出汗盘管萎缩。细胞毒性 T 淋巴细胞浸润和 MxA 表达仅在 InfAIGA 患者的汗腺中观察到。
InfAIGA 与汗腺炎症增加和汗盘管萎缩有关，而非 InfAIGA 仅与汗盘管萎缩有关。这些数据表明，炎症导致与汗盘管萎缩相关的汗腺上皮破坏，随后功能丧失。非 InfAIGA 可能被视为 InfAIGA 的炎症后状态。这些观察结果表明 1 型和 2 型干扰素均对汗腺损伤有贡献。所涉及的机制类似于斑秃（AA）的发病机制。
