Brocq氏先天性魚鱗癬樣紅皮症ニ就テ

The patient, a boy aged 3 years, is the 5th child of the family. There is no family history of heredity. His parents are not consanguineous. His mother was quite healthy during her pregnancy. Shortly after the child was born, it was noticed that many linear intensive erythemata were widely distributed over the cutaneous surface of the child in a rather symmetrical fashion. After a month or so, these erythemata were mostly covered with horny scales of rather coarse nature. The condition improved in warm weather, but became worse in cold weather. On account of the condition of the skin the patient was brought to our clinic with his mother. The patient is normal-sized and normally developed physically for his age. He is rather bright and intelligent. The affected parts-the head, the face, the neck, the thorax except the right abdomen, the extremities including hands and feet-are covered with horny plates varying in degree, associated with slight itching only when warm. In places the plates are so thick that they resemble a horny armour, but still maintaining their linear arrangement. These thick scales are hard to remove, and when removed there occurs slight bleeding. Even such a flexor surfaces as the axilla and the palms and soles are covered with thick scales. On the face, on the buttocks and on the femoral region linear erythemata besides scales are very noticeable. The development of hair and nails is remarkable. The mucous membranes are not involved. No abnormality in the internal organs is traceable. On examination it was found that the Wassermann test of the bloodserum of both the parents and the the patient was negative; Pirquet's cuti-reaction was also negative. No abnormality in the urine and the faeces. The blood was O type. The heamoglobin-index (Sahli) 75. The number of the erythrocytes was 8620000, that of the leucocytes was 10300 (among them neutrophilous 51,8%, small lymphocytes 32,9%, large lymphocytes 4,9%, eosinophiles 8,7%, basophiles 1,7%, transitional forms 0,9%.) The blood sugar content (Hagedorn and Jensen) was 0,092gm.%. The cholesterin content in the total blood (Bloor) was 0,132gm.%. The cholesterin content in the plasma (Bloor) was 0,103gm.%. The cholesterin content in the blood-corpuscles (Bloor) was 0,175gm.%. The salt content in the blood (Rusziniak) was 0,4979gm.%. The glutathione content in the blood (Tannicliffe) was 0,0184gm.%. Examination of the vegetative nervous system with pilocarpin, atropin and adrenalin showed no abnormality. Both Aschner's reaction and Tschermack's reaction were negative, Histological examination showed remarkable thickening of the horny layer with partial parakeratosis, decrese of the granular layer, acanthosis of the pricklecell layer. In the corium there was a moderate degree of round-celled infiltration and vascular dilation. The elastic fibres were mostly diminished, degenerated at least, in the parts infiltrated with cells. The author, accordingly, is of opinion that the aetiological factors mentioned by former investigators of this disease are not detectable in this patient. The remarkable decrease of the gluiathione content in the blood of this patient must have some connection with the manifestation of this disease.