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Cutaneous Porphyrias by Mind Map: Cutaneous Porphyrias
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Cutaneous Porphyrias

Because of the presence of phototoxic porphyrins, all cutaneous porphyrias are associated with photosensitivity. The liver accounts for 15% of the heme synthesis, utilizing the synthesis of cytochromes and catalases. The remaining 85% of the synthesis occurs in the bone marrow. Partial enzymatic activity of any of the 8 enzymes of the heme synthesis pathway results in excessive accumulation and excretion of porphyrins and their precursors, porphyrinogens.  

Types

Child

Manifests in Infancy to Childhood Acute photosensitivity shortly after UVR Exposure

Adult

Manifests after 2nd Decade Chronic Symptoms Appears several days after sun exposure Increased fragility of the skin, scarring, especially on dorsal hands

Labs

Algorithmic Testing

By Source

By Disease