VBDs Porphyria Cutanea Tarda

Question 1

Porphyria Cutanea Tarda

Clinical Feature. Lesion

Answer 1

• skin fragility followed by formation of tense vesicles/bullae and erosions on photoexposed skin
• gradual healing to scars, milia

Question 2

Porphyria Cutanea Tarda. Sites

Answer 2

common sites: light-exposed areas subjected to trauma, dorsum of hands and feet, nose, and upper
trunk

Question 3

Porphyria Cutanea Tarda on FACE

Answer 3

periorbital violaceous discolouration, diffuse hypermelanosis, facial hypertrichosis

Question 4

Porphyria Cutanea Tarda

Pathophysiology +Asoc Patholo

Answer 4

• uroporphyrinogen decarboxylase deficiency leads to excess heme precursors
• can be associated with hemochromatosis, alcohol abuse, DM, drugs (estrogen therapy, NSAIDs), HIV,
hepatitis C, increased Fe indices

Question 5

Porphyria Cutanea Tarda Epidemiology

Answer 5

30-40 yr old, M>F

Question 6

Porphyria Cutanea Tarda Investigations

Answer 6

• urine and 5% HCl shows orange-red fluorescence under Wood’s lamp (UV rays)
• 24 h urine has elevated uroporphyrins
• stool contains elevated coproporphyrins
• immunofluorescence shows IgE at dermal-epidermal junctions

Question 7

Porphyria Cutanea Tarda Management. avoid. procedures and PO

Answer 7

• discontinue aggravating substances (alcohol, estrogen therapy)
• phlebotomy to decrease body iron load
• low dose hydroxychloroquine