DiPreta E A, Smith K J, Williams J, Skelton A H
National Naval Medical Center, Bethesda, Maryland 20889-5600, USA.
J Cutan Med Surg. 2000 Jul;4(3):156-60.
Vogt-Koyanagi-Harada (VKH) disease is a rare autoimmune disease primarily involving pigmented structures. The commonly associated alopecia usually precedes leukodermic changes seen in the hair and skin.
We present a 46-year-old oriental woman with VKH disease who developed diffuse alopecia followed by the regrowth of nonpigmented hairs. The biopsy specimens showed a peribulbar mononuclear infiltrate with increased telogen/catagen:anagen follicles. The most prominent additional histologic finding was melanin pigment release from the matrix into the dermal papillae, fibrous tracks, and surrounding perifollicular sheaths. The peribulbar mononuclear cells showed diffuse immunohistochemical staining of approximately 80 to 90% of the cells for CD3, CD45RO. More than 50% of the mononuclear cells showed positive staining for CD4, whereas approximately 10 to 20% showed staining for TIA with TIA+ cells within the follicular epithelium.
Although the histologic features seen in the alopecia associated with VKH are consistent with alopecia areata, the prominent pigment release does suggest that the prime target are the melanocytes and that keratinocytes may be secondarily involved.
伏格特-小柳-原田(VKH)病是一种罕见的自身免疫性疾病,主要累及色素结构。常见的脱发通常先于毛发和皮肤出现的白斑变化。
我们报告一名46岁患有VKH病的东方女性,其出现弥漫性脱发,随后长出无色素毛发。活检标本显示毛囊周围单核细胞浸润,休止期/退行期与生长期毛囊比例增加。最显著的额外组织学发现是黑色素从基质释放到真皮乳头、纤维束及周围毛囊鞘中。毛囊周围单核细胞约80%至90%的细胞对CD3、CD45RO呈弥漫性免疫组化染色。超过50%的单核细胞对CD4呈阳性染色,而约10%至20%的细胞对颗粒酶A呈阳性染色,且在毛囊上皮内可见颗粒酶A阳性(TIA+)细胞。
尽管VKH相关脱发的组织学特征与斑秃一致,但显著的色素释放确实表明主要靶标是黑素细胞,角质形成细胞可能是继发受累。