種痘様水疱症の1例

HYDROA VACCINIFORME

I．緒言

　幼少時より日光照射部位に，再発性に，紅斑，中心臍窩を有する水疱及び痂皮を生じ，瘢痕を以つて治癒する一見痘瘡に類似せる特徴を持つ疾患に，1862年BazinはHydroa vacciniformeと名付け，一独立疾患とした。しかしその後，皮膚症状が本症のそれに著しく類似せるものの中で，尿中にPorphyrinを排泄する患者が発見され，Porphyrin尿症として区別された。そして更に，Hydroa vacciniformeは逆にPorphyrin尿症の不全型で，Porphyrin尿がみられないものに過ぎないと云う考えすらも出現し，昨今いささか両症の関係が混乱しているかに見受けられる。我々が最近検索した限りに於いては，Porphyrin代謝には異常を認めず，Bazinの古典的記述に一致すると思われる3才男児の症例を経験したので，ここに報告する。

A 3-year-old boy has had episodes of exacerbation of a photosensitive eruption in spring. At his first visit, vesicles with a red areola, some of which were umbilicated, blood crusts and slightly depressed scars were present on the forehead, cheeks, ears and back of the hands disseminatedly or in groups. His past and family history were not contributive.

The results of laboratory tests including quantitative and qualitative analyses of porphyrin in the blood and urin were within normal limits.

Histologic specimen from the vesicle showed intraepidermal multilocular nature with a tendency of retiform degeneration. The papillary and subpapillary layers of the dermis were edematous and had a dense infiltrate composed mainly of lymphoid cells. There was a patchy infiltrate of lymphoid cells around the vessels and skin appendages throughout the dermis and subcutaneous tissue. The dilatation of vessels, swelling of the endothelial cells and thickening of the vessel walls were noted.

Irradiation test with xenon arc lamp revealed normal MED when filtered with color glass filter UV29 (Toshiba), higher MED by UV31. Higher MED was also noted with irradiation of inter-mittent mercury vapor lamp. Only irradiation with natural sunlight (1.4 viton/cm²) for 45 minutes could produce papules and vesicles, the appearance of which was interfered with sunscreen.