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Five Cases of Porphyria Cutanea Tarda with Mild Cutaneous Changes: Evaluation of the Efficacy of Phlebotomy by the Pattern Analysis of Urinary Porphyrins
Author(s) -
aka Shigeo,
Ohgami Taro,
Murayama Fumio,
Yamashita Kazunori,
Yoshida Hikotaro
Publication year - 1986
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.1986.tb02926.x
Subject(s) - porphyria cutanea tarda , porphyria , phlebotomy , porphyrin , urine , urinary system , excretion , medicine , photosensitivity , chemistry , pathology , biochemistry , physics , quantum mechanics
Five cases of porphyria cutanea tarda (PCT) with mild cutaneous changes are reported. Acute episodes of photosensitivity were not seen in all patients. Laboratory examinations showed a predominance of uroporphyrin (UP) excretion in the urine in 4 out of 5 cases. One case showed a relatively low urinary porphyrin level even though abnormality of the porphyrin pattern was present. Four cases showing a predominance of UP excretion in the urine revealed a high level of serum coproporphyrin (CP) as well. Histopathologically, all cases showed a deposition of PAS positive materials surrounding the small blood vessels in the upper dermis, in addition to the deposition of IgG materials visualized by the immunofluorescent technique. Phlebotomy was performed fifteen times in one case. The urinary porphyrin level was gradually reduced to normal levels and the urinary porphyrin pattern shifted from a predominance of UP and hepta‐carboxyl porphyrin to a predominance of CP excretion. Analysis of the urinary porphyrin profile is useful as a laboratory tool for diagnosis or observation of the course of PCT, as well as for the quantitative analysis of urinary porphyrins.