S9C10P3-Sclerosing Dermatomyositis

S9C10P3-1: In this perifollicular area of a lesion of dermatomyositis, lichenoid patterns, that are cell-poor, are present at the epithelial-stromal interface; there are liquefactive changes at the interface. A thickened basement membrane is convoluted. The perifollicular sheath is fibrotic. The patterns are compatible with an incipient phase of a scarring alopecia, mediated at the level of a cell-poor lichenoid reaction.

S9C10P3-2: A mucinosis can be characterized as the lytic phase of a collagenosis, or as the lytic phase of connective tissue activation. In contrast, some disorders of the skin are represented in sclerotic patterns; they might be characterized as the sclerotic phase of connective tissue activation. Connective tissue activation often tends to be manifested first at connective tissue interfaces. Early on, they may be manifested at the interface between the papillary dermis and the reticular dermis, or between the reticular dermis and the subcutaneous fat. In this field, there is a pattern of sclerosis affecting the papillary dermis and the upper portion of the reticular dermis (blue arrows). In addition, there is a vertically oriented, linear zone of sclerosis to the right of the center of the field in the reticular dermis (just to the left of the sweat gland in this same area) (green arrows). This area of fibrosis probably represents sclerosing atrophy of a hair follicle. The epidermis shows acanthosis (basal unit hyperplasia) and hyperpigmentation of the basal layer (the epidermal pattern is somewhat psoriasiform). The papillary dermis is widened and fibrotic; the fibrosing process extends into the upper 1/4 of the reticular dermis. The pattern resembles that of superficial morphea (the pattern in the papillary dermis is not that of classic lichen sclerosis et atrophicus), but the clinical setting was dermatomyositis. By custom, this is a morphea-like process but, conceptually, the lesion might also be characterized as dermatomyositis in a sclerotic phase. Perhaps, most observers would take the combination as an indication of a mixed connective tissue disease.

S9C10P3-3: In this field of a lesion, in a patient with a diagnosis of dermatomyositis, the epidermis shows the pattern of a cell-poor lichenoid reaction (liquefaction degeneration, basal unit atrophy, superficial unit hypertrophy, and compact hyperkeratosis). There is a zone of superficial dermal fibrosis, involving the papillary dermis and the upper portion of the reticular dermis. Vessels are ectatic ( dermatomyositis, sclerotic phase).

 

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