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A case of fibromuscular dysplasia and aortic dissection
HUMAN PATHOLOGY
Volume 23, No. 12 (December
CORRESPONDENCE
Guidelines
for Letters
Letters to the Editor will be published at the discretion of the editor as space permits and are subject to editing and abridgement. They should be typewritten, double-spaced, and submitted in triplicate. They should be limited to 500 words or less and to no more than five pertinent references.
Are There Hyperplastic Capillaries in Sneddon’s Syndrome? To the Editor:-1 would like to congratulate Zelger et al’ for their outstanding description of the arterial lesions in Sneddon’s syndrome. As is the case with any innovative work, their article presents more questions than answers. I would like to ask the authors two questions: first, were they always able to distinguish the dilated capillaries in the periarterial space from sweat glands (situated supposedly in the lower right corner of their Fig ?a) and, second, was there restitutio ad integrum in the periarterial space or did the extensive angiogenesis lead to fibrosis? As far as I am aware, hyperplastic capillaries similar to sweat glands were first described by Gans and Steigleder’ in the capillary hemangioma. I have reported them in the hemangiopericytoma, the hemangioleiomyoma, and the cutis marmorata congenita.’ Zelger et al’ have accumulated an enormous amount of material concerning Sneddon’s syndrome. Perhaps they would be willing to give their authoritative opinion about the hyperplastic capillaries in this syndrome and, eventually, in other diseases.” JIKI T. &XANEK, MD UMC-School of Medicine Columbia, MO
The above lrttrr was referred to the authors of the ctrticle in question, who offer thu following reply: To thr E&&:--Increased numbers of dilated capillaries in the periarterial space, which Dr Beranek calls “hyperplastic capillaries,” are indeed striking vascular phenomena in Sneddon’s syndrome, as recently described.’ We never had problems in distinguishing these capillaries from sweat glands/ducts on morphologic grounds in paraffin specimens. Morphologic distinction was confirmed by immunohistochemical analyses performed on serial paraffin sections: endothelial cells labeled with endothelial markers JC/70A (CD3 1; Dako; diluted 1: 10) and Q BEnd 10 (CD34; Oxoid; 1:50) and sweat glands labeled with polyclonal CEA (Dako; 1: 100) and monoclonal EMA (Dako; 1:50). Yet, when performing studies on snap-frozen material we had enormous difficulties in distinbwishing vascular structures with collapsed lumina from other structures of the dermosubcutaneous area (eg, hair follicles, nerves) (manuscript in preparation).
1438
1992)
Despite extensive angiogenesis in early inflammatory stages, 110 or only insi@icantly increased fibrosis was observed in the periarterial space in later stages, which was in contrast to the often-marked fibrosis within the original lumen side (see Fig 5a. b, and e in our report’). The time frame of these events best fits that of a reactive process due to angiogenic factors (platelet-derived growth factor, basic fibroblast growth factor) released by altered endothelial cells and/or smooth muscle cells/myofibroblasts.‘” Alternatively, these vessels could be interpreted as a compensatory circuit, which would still not explain its later involution. We have 110 particular experience with hyperplastic capillaries in disorders other than Sneddon’s syndrome; thus, we are not able to provide an authoritative opinion regarding their origin. Immunohistochemical results with antibodies for adhesion molecules, cytokines, or growth factors might help to further elucidate this question. BEKNHAW ZILGEK, MD NOKBWT SEW, MD I’ETEK FRIISCH, MD llniversity of Innsbruck Innsbruck. Austria
A Case of Fibromuscular and Aortic Dissection
Dysplasia
To the Uilor:-Gatalica et al’ have not provided convincing evidence for generalizecl fibromuscular dysplasia complicated by dissecting aortic aneurysm. Their Fig 2, top simply shows fibromuscular intimal thickening in a coronary artery with the possibilit): of a focal mecliolytic lesion in the upper part of the field. Figure 2. bottom illustrates what appears to be a healed dissection of the aorta with prolapse of a torn intimomedial flap into the lumen. The defect is partially filled by myofibroblastic reparative tissue. The terminal acute aortic dissection is situated in the outer media peripheral to the chronic tear. Fibromuscular dysplasia of the aorta is a rare condition in comparison to aortic dissection.’ This patient may well have had recurrent aortic dissection 011 a background of systemic hypertension with secondary changes mimicking fibromuscular dysplasia. H. AI.EXANIIE:RHE~:GT~~I~I,MD McMaster LJniversity Medical Centre Hamilton, Ontario, Canada
The about letter was rcferrrd to the ccuthon of the article in p&ion. who oJer the followzng reply:
our
To the Editor:-We report of a case
appreciate Dr Heggtveit’s of fibromuscular dysplasia
interest in with aortic
Volume 23, No. 12 (December
CORRESPONDENCE
Guidelines
for Letters
Letters to the Editor will be published at the discretion of the editor as space permits and are subject to editing and abridgement. They should be typewritten, double-spaced, and submitted in triplicate. They should be limited to 500 words or less and to no more than five pertinent references.
Are There Hyperplastic Capillaries in Sneddon’s Syndrome? To the Editor:-1 would like to congratulate Zelger et al’ for their outstanding description of the arterial lesions in Sneddon’s syndrome. As is the case with any innovative work, their article presents more questions than answers. I would like to ask the authors two questions: first, were they always able to distinguish the dilated capillaries in the periarterial space from sweat glands (situated supposedly in the lower right corner of their Fig ?a) and, second, was there restitutio ad integrum in the periarterial space or did the extensive angiogenesis lead to fibrosis? As far as I am aware, hyperplastic capillaries similar to sweat glands were first described by Gans and Steigleder’ in the capillary hemangioma. I have reported them in the hemangiopericytoma, the hemangioleiomyoma, and the cutis marmorata congenita.’ Zelger et al’ have accumulated an enormous amount of material concerning Sneddon’s syndrome. Perhaps they would be willing to give their authoritative opinion about the hyperplastic capillaries in this syndrome and, eventually, in other diseases.” JIKI T. &XANEK, MD UMC-School of Medicine Columbia, MO
The above lrttrr was referred to the authors of the ctrticle in question, who offer thu following reply: To thr E&&:--Increased numbers of dilated capillaries in the periarterial space, which Dr Beranek calls “hyperplastic capillaries,” are indeed striking vascular phenomena in Sneddon’s syndrome, as recently described.’ We never had problems in distinguishing these capillaries from sweat glands/ducts on morphologic grounds in paraffin specimens. Morphologic distinction was confirmed by immunohistochemical analyses performed on serial paraffin sections: endothelial cells labeled with endothelial markers JC/70A (CD3 1; Dako; diluted 1: 10) and Q BEnd 10 (CD34; Oxoid; 1:50) and sweat glands labeled with polyclonal CEA (Dako; 1: 100) and monoclonal EMA (Dako; 1:50). Yet, when performing studies on snap-frozen material we had enormous difficulties in distinbwishing vascular structures with collapsed lumina from other structures of the dermosubcutaneous area (eg, hair follicles, nerves) (manuscript in preparation).
1438
1992)
Despite extensive angiogenesis in early inflammatory stages, 110 or only insi@icantly increased fibrosis was observed in the periarterial space in later stages, which was in contrast to the often-marked fibrosis within the original lumen side (see Fig 5a. b, and e in our report’). The time frame of these events best fits that of a reactive process due to angiogenic factors (platelet-derived growth factor, basic fibroblast growth factor) released by altered endothelial cells and/or smooth muscle cells/myofibroblasts.‘” Alternatively, these vessels could be interpreted as a compensatory circuit, which would still not explain its later involution. We have 110 particular experience with hyperplastic capillaries in disorders other than Sneddon’s syndrome; thus, we are not able to provide an authoritative opinion regarding their origin. Immunohistochemical results with antibodies for adhesion molecules, cytokines, or growth factors might help to further elucidate this question. BEKNHAW ZILGEK, MD NOKBWT SEW, MD I’ETEK FRIISCH, MD llniversity of Innsbruck Innsbruck. Austria
A Case of Fibromuscular and Aortic Dissection
Dysplasia
To the Uilor:-Gatalica et al’ have not provided convincing evidence for generalizecl fibromuscular dysplasia complicated by dissecting aortic aneurysm. Their Fig 2, top simply shows fibromuscular intimal thickening in a coronary artery with the possibilit): of a focal mecliolytic lesion in the upper part of the field. Figure 2. bottom illustrates what appears to be a healed dissection of the aorta with prolapse of a torn intimomedial flap into the lumen. The defect is partially filled by myofibroblastic reparative tissue. The terminal acute aortic dissection is situated in the outer media peripheral to the chronic tear. Fibromuscular dysplasia of the aorta is a rare condition in comparison to aortic dissection.’ This patient may well have had recurrent aortic dissection 011 a background of systemic hypertension with secondary changes mimicking fibromuscular dysplasia. H. AI.EXANIIE:RHE~:GT~~I~I,MD McMaster LJniversity Medical Centre Hamilton, Ontario, Canada
The about letter was rcferrrd to the ccuthon of the article in p&ion. who oJer the followzng reply:
our
To the Editor:-We report of a case
appreciate Dr Heggtveit’s of fibromuscular dysplasia
interest in with aortic