Pathology
Images
Atlas of dermatopathology
Josef Feit, Hana Jedličková, Günter Burg, Luděk Matyska, Spasoje Radovanovic et al.
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+ Introduction
+ Literature
+ Support
+ Skin biopsy
+ Non-tumorous skin diseases
+ Skin tumors
+ Cysts of the skin and subcutis
+ Soft tissue lesions
+ Some lesions of the oral cavity
+ Dermatovenerology
+ Collection of histological slides by prof. Günter Burg
+ Collection of histological slides by prof. Werner Kempf
+ Collection of histological slides of dr. Ram Chandra Adhikari
Porphyria cutanea tarda
Introduction

A group of defects in heme production. Porphyrins and their precursors are present in tissues and are present in urine or feces. Most of porphyrias are accompanied vy skin problems, some are accompanied by liver disorders, anemia and other symptoms. Porphyria cutanea tarda is the most common group of porphyrias. Its signs are usually limited to skin.

Etiology, pathogenesis
  • autosomal dominant
  • uroporphyrinogen decarboxylase defect
  • increased concentration of uroporphyrin in urine
Clinical signs
  • the sun exposed skin: the face, backs of the hands, nape of the neck, ears
  • fragile skin with blisters, erosions, crusts, sclerodermoid changes
  • hyperpigmentation, hypertrichosis, actinic changes
  • healing with scars and milia
  • photosensitivity (photoactivated porphyrins in the skin generate toxic oxygen species)
  • prolonged course, patients aged 30 – 50 years, in younger women using contraceptives
  • iron, drugs, estrogens, alcohol may provoke porphyria
  • porphyrinuria with orange fluorescence in UV light
  • hepatopathy, raised serum iron level
Pictures

Porphyria cutanea tarda: Porphyria cutanea tarda, Clinical picture (883)

Porphyria cutanea tarda, forearm: Porphyria cutanea tarda, Clinical picture (885)

Further examples of porphyria cutanea tarda:

Histology

the appearance of skin is similar in all types of porphyria: tense subepidermal hemorrhagic bulla in photosensitive areas (dorsa of hands), PAS positive subepidermal deposits, thickened, PAS positive vascular walls