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Phacomatosis Pigmentovascularis Type IIb Associated with Sturge‐Weber Syndrome and Pyogenic Granuloma
Author(s) -
Hagiwara Keisuke,
Uezato Hiroshi,
aka Shigeo
Publication year - 1998
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.1346-8138.1998.tb02491.x
Subject(s) - pyogenic granuloma , medicine , dermatology , sturge–weber syndrome , pathology , lesion
A case of phacomatosis pigmentovascularis (PPV) in a 6‐year‐old girl with Sturge‐Weber syndrome, pyogenic granuloma, and other complications is described. It is relatively rare that a complete form of Sturge‐Weber syndrome was associated with PPV. A review of the literature on PPV, focusing on total number of reported cases and etiological speculations, is presented. To our knowledge, a total of 118 cases of PPV, including the present one, have been reported to date. Regardless of many speculations, the true etiology remains unknown. The average “density” of mast cells (MCs) per mm 2 appearing in the central region of the pyogenic granuloma was calculated to be 86.3/mm 2 and that in the adjacent nevus flammeus was 37.9/mm 2 . The “density” of mast cells in pyogenic granuloma separately calculated from ten other cases was 105.5 ± 28.6/mm 2 (mean ± SD), compared with that in normal skin, 6.85 ± 4.9/mm 2 (n=20). There was a significant difference between the two, indicating that MCs are closely associated with angiogenesis in pyogenic granuloma.