S15C5P1-Sclerodermoid Porphyria

S15C5P1-1:This a sclerotic lesion in the setting of porphyria. The epidermis shows hyperkeratosis, mild acanthosis, and effacement of rete ridges. There are spotty areas showing liquefaction degeneration at the dermal-epidermal interface. A zone of sclerosis extends from the dermal-epidermal interface into the upper portion of the reticular dermis. Vessels are ectatic. The dermis shows mild solar elastosis. There are minimal perivascular infiltrates of lymphoid cells. The patterns, in distribution, and in alterations at the dermal-epidermal interface as well as in the fibrous character of the upper portion of the dermis, might be compared to changes seen, in the same anatomic compartments, in lesions of LS&A.
BuiltWithNOF

S15C5P1-2: In the area of liquefaction degeneration, the basement membrane shows hyalinosis (blue arrows). The dermis, near the dermal-epidermal interface, is hypocellular. A blue arrow points to a rounded zone of hyalinosis adjacent to a small vessel with a thickened wall. The structure at the extremity of the arrow is interpreted as a sclerotic, hyalinized vessel. The patterns somewhat resemble those of superficial morphea.

S15C5P1-3: The structure between the blue arrows is interpreted as an obliterated, hyalinized vessel; as such, the vascular changes qualify as evidence of an obliterative hyaline angiopathy. The pattern at the dermal-epidermal interface is a cell-poor lichenoid reaction.

S15C5P1-4: The features include: hypertrophy of cells of the superficial unit of the epidermis with hypergranulosis. The changes also include: atrophy of the basal unit of the epidermis; basement membrane hyalinosis; a cell-poor lichenoid reaction; superficial dermal sclerosis; and a hyalinizing angiopathy (see S15C7P1-1-6 for comparison with superficial morphea and LS&A).

 

NEXT PAGE in spatial sequence along horizontal axis of tier

Beige BARS to right provide access to CHAPTERS of this SECTION. Mauve bars provide access to other SECTIONS on this SITE.

BACK a PAGE in spatial sequence along horizontal axis of tier

GREEN BARS above provide access to 2 web sites

UP ONE TIER; if at tier 3, up to respective parent PAGE at tier 2; if at tier 2, UP to HOME

MAUVE BARS to right provides access to other SECTIONS on this SITE