Quiz 47: What is your diagnosis?

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Diagnosis: Quiz 47

Quiz 47

Answer: Vascular malformation with scar and foreign body reaction

Criteria for diagnosis histopathologically: In the lower half of the dermis and in the subcutis, even at the level of skeletal muscle, is a proliferation of fibrocytes in company with coarse but fibrillar bundles of collagen in array haphazard, those being findings of a scar. In a locus nearby are epithelioid histiocytes, some of them multinucleate, that house in their cytoplasm material extraneous, i.e., particles of gauze, those being changes of a foreign body granulomatous reaction. Above and below the zones of scar and granulomatous inflammation is a marked increased in the number of endothelium-lined spaces dilated extraordinarily, some of them having an outline bizarre, all situated at some distance from one another, those being attributes of a vascular malformation.

Differential diagnosis histopathologically: There is none.

Criteria for diagnosis clinically: A smooth-surfaced reddish tumor crisscrossed by telangiectases is a vascular malformation whose delled surface is testimony to biopsies having been performed previously at the site by punch technique.

Differential diagnosis clinically: The tumor could be a benign or malignant neoplasm, as well as a vascular malformation, and biopsy is requisite to clarify the conundrum.

Clinicopathologic correlation: The tumor is comprised largely of the proliferation of blood vessels expanded inordinately and that extend throughout the dermis into the subcutis. The redness is a consequence of those vascular spaces dilated remarkably having been filled in vivo with red blood cells. The telangiectases on the surface are the result of prominently dilated end vessels in the upper part of the dermis, enlarged prominently in vivo and there filled with blood. The dells represent sites from which punched-out biopsy specimens had been obtained. The surface of the lesion is smooth because the stratum corneum is normal. The findings histopathologic of granulomatous inflammation do not add to the clinical appearance of this tumor, and the retraction of the scar is a factor in the formation of dells.

Options for therapy predicated on knowledge of histopathologic findings: This vascular malformation extends from the upper part of the reticular dermis into the subcutis to the base of the section and, doubtless, even deeper. That being so, and should the patient wish to have the lesion removed for purposes of cosmesis, a surgical excision, must be carried to normal skin around the tumor and beyond the base of it to near fascia if the abnormality vascular is to be extirpated in its entirety.

1) The scar evident in this section of tissue is secondary to trauma inflicted by punch biopsies carried out before. The dells on the surface of the tumor are a consequence both of loss of 4.0 mm pieces of tissue from the sites and of retraction by virtue of fibroplasia that developed secondary to the trauma visited by the procedure. The foreign body in the cytoplasm of epithelioid histiocytes is gauze implanted during the biopsies. 

The use of the word "tumor" in this context applies to the size (2.0 cm) and the shape (domed) of the lesion, not as a synonym for "neoplasm"; the process pathologic is a malformation, not a neoplasm.

2) The fibrosis shown in the photomicrographs fulfills criteria for a scar because the markedly increased number of fibrocytes arranged randomly is joined by coarse but fibrillar bundles of collagen also arrayed haphazardly. Although, as a rule, a scar is complemented by an increase in number of dilated venules, in this instance there actually are fewer blood vessels in it than in the region above and below it where the vasculature of the malformation is eye-catching.

3) A scar has certain repeatable characteristic features, clinically and histopathologically, the process commencing usually as granulation tissue. After that, yet still early in the course of a scar, there is a florid increase in the number of plump fibrocytes associated with delicate fibrillary bundles of collagen, that combination being joined at times by mucin discernible readily. Often the fibrocytes and the bundles of collagen are organized parallel to surface epidermis and are accompanied by what seems to be an increase the in number of dilated venules, they tending to be more or less perpendicular to the surface of the skin. In time, the number of fibrocytes in a scar decreases progressively and the bundles of collagen, although continuing to be fibrillar, become ever more coarse. It is that stage of a scar which is illustrated in these photomicrographs. Clinically, a scar can be hypertrophic, atrophic, or flush with the surrounding skin.

4) Fibrosis and fibroplasia are synonyms. Sclerosis is a very late stage of fibrosis at which the number of fibrocytes is few and the bundles of collagen no longer are identifiable as discrete units, the appearance of the tissue so affected then being designated conventionally, if not accurately, "homogenized." Fibrosis is not synonymous with "scar," it being encountered in a host of circumstances ranging from processes inflammatory to ones neoplastic (where it is known as desmoplasia). The three most common examples of fibrosis as a manifestation of an inflammatory process are scar, keloid, and dermatofibroma.

5) A vascular malformation may present itself in ways protean, one of which is termed nevus flammeus, another angiolymphoid hyperplasia with eosinophilia, and yet another arteriovenous shunt, to mention but three of them. The malformation pictured here has not yet been given a name specifically identifying. Note well that unlike the situation histopathologic in a benign neoplasm of blood vessels, i.e., a hemangioma, in which the abnormal vascular spaces are in very close proximity to one another, in a vascular malformation just the reverse is the case, namely, the abnormal vessels dilated extravagantly are separated widely from one another.

6) As a rule, a venous ulcer tends to appear above a medial malleolus or lateral to it and the ulcer itself often is vaguely rectangular with a ragged border and a shallow base that sports "proud flesh", i.e., granulation tissue. By contrast, an arterial ulcer develops more distally over bony prominences and the ulcer itself inclines to be round with a sharply demarcated border and a deep base that sometimes is covered by an eschar.

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