S6C38P32-Porphyria

S6C38P32-1: This is material from the same case of variegate porphyria seen in S6C37P3-1; the epidermal margin of the vesicle is represented on the right. The papillary dermis is widened and hyalinized. Vessels of the reticular dermis and a sweat duct also show adventitial hyalinization.

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S6C38P32-2: In this field, the papillary dermis is at the top of the field, it is hyalinized. The sheath of a sweat duct is widened and hyalinized; the sheath is laminated. A vessel near the center of the field shows prominent perivascular hyalinization in laminated patterns.

S6C38P32-3: Vessels in the deeper portion of the dermis near the deep margin of the specimen show similar patterns of perivascular hyalinization in laminated patterns. On the right, hyalin extends irregularly into the neighboring reticular dermis.

S6C38P32-4: This is a lesion of lupus erythematosus in a patient with porphyria (LE-porphyria).

S6C38P32-5: At higher magnification, the epidermis shows a poorly developed  basal unit with liquefaction degeneration at the dermal-epidermal interface. The basement membrane is thickened and hyalinized.

S6C38P32-6: This is another example of porphyria cutanea tarda; this PAS-hematoxylin stain shows positive staining in the walls of dermal vessels, particularly the vessels extending into the dermal papillae. This PAS positivity is the result of the deposition of immunoglobulins and complement in the walls of the vessels. The deposits apparently lend rigidity to the involved vessels; the involved vessels act as a strut which supports the naked papillae. The festooning owes its prominence to the rigid vessels.

S6C38P32-7: In this same patient, milia were a feature of a lesion at another site. Milia of this type probably represent obstructed (disrupted) sweat ducts (porphyria cutanea tarda).

 

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