S9C8P1-Scleromyxedema

S9C8P1-1: In a most general manner, the designation, collagenosis, might be accepted as a characterization of a variety of alterations in the fibrous components of the dermis, including both collagen and elastica. Conceptually, it also can include mucinous alterations of connective tissue. The collagenous components and the mucinous components are interdependent. In practice, a characterization of the fibrous components, as structures that are independent of the mucinous matrix of the dermis, is a morphologic convenience. In morphologic terms, the elastic fibers of the reticular dermis float in a mucinous matrix. Almost certainly, the collagen bundles, themselves, include a mucinous interstitial component, a component that normally is masked by the close packing of collagen fibrils.

Collagenosis, as defined herein, gives recognition to generalizations as defined above. In addition, the designation, with qualifications, gives recognition to sclerotic disorders. Thus, the general category, collagenosis, includes the sclerotic collagenoses, the mucinous collagenoses (mucinoses), and the elastotic collagenoses (the elastoses). In general usage, the suffix, -osis, is sometimes characterized as signifying “full of.” As employed in this section, it might be thought of as signifying “abnormal or diseased condition.”  In the above field, there is a band of altered fibrous tissue in the upper portion of the reticular dermis. Collagen bundles in the area are thin and straight (parallel to the skin surface). In a central focus, there is an increased number of spindle cells among the altered collagen bundles. Inflammation is not a prominent feature (sclerotic phase of scleromyxedema).

S9C8P1-2: In this field, the collagen bundles, in a localized area in the upper portion of the reticular dermis, are thin; they are loosely spaced in a mucinous matrix (mucinous phase of scleromyxedema). In the spaces among the collagen bundles, spindle cells are increased in number per unit area. There is also an increase in the number of small vessels. There are minimal perivascular infiltrates. The epidermis shows hyperkeratosis, parakeratosis, and accentuation of the basal layer. On the right, the basal unit of the epidermis is hyperplastic. On the left, the superficial unit of the epidermis is hyperplastic, a feature contrasting with  a relative atrophy of the basal epidermal unit (mucinous phase of scleromyxedema).

S9C8P1-3: With a colloidal iron stain, mucinous changes are prominent in the upper 1/4 of the reticular dermis. The changes are band-like from side to side. The focality, which characterizes a lesion of granuloma annulare, is lacking (but on several earlier biopsies from this same patient, the changes had been interpreted as those of granuloma annulare).

S9C8P1-4: At this magnification and without regard for the changes on the H&E sections, the patterns might be compared to some of the features seen in a lesion of granuloma annulare (collagen is red, mucin is blue, and elastica and cells are yellow).

S9C8P1-5: In this area of sclerosis, the collagen bundles are small and interwoven; they are relatively closely spaced in comparison with the patterns in mucinous zones. Fibroblastic cells are spindle and stellate in outline; delicate cell processes tend to embrace the small collagen bundles.

S9C8P1-6: An area showing minor mucinous changes is hypercellular. Fibroblastic cells have elongated nuclei  and elongated, delicate cell processes. Histiocytes are rounded or polygonal.

Histiocytes are often a prominent feature of the mucinoses; they may have a role in both the fibrillation (unbundling of collagen bundles) and the unmasking of the background mucinous matrix. Many of the nuclei have central nucleoli; they are activated.

Kim YJ, Kim YT, Kim  J-H: Self-healing juvenile cutaneous mucinosis. J Am Acad Dermatol 199430:815-16.

S9C8P1-7: The dermis shows widening of spaces among collagen bundles of the reticular dermis; the spaces contain mucinous material. The altered dermis shows hyperplasia of mesenchymal cells. There is not a significant degree of sclerosis, but the changes are in keeping with nephrogenic fibrosing dermopathy.

S9C8P1-8: The dermis is hypercellular. The cells mostly are spindle shaped; they are fibroblastic cells. Histiocytes, as might be seen in the interstitial infiltrates of granuloma annulare, are not a prominent feature. Mucinous deposits are prominent in the spaces among collagen bundles (nephrogenic fibrosing dermopathy).

 

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