ac intermittent porphyria/Porphyria cutanea tarda - de_novo

[ArchivalUser]

Who can explain??

[ArchivalUser]

For example.

PCT patients typically present with blistering in a sun exposed area, skin fragility, scarring with milia formation (presents as 1-2 mm white papules) and hypertrichosis. Other cutaneous changes include hyperpigmentation and sclerodermoid lesions. In sporadic PCT or acquired PCT, association with exposure to hepatitis B or C or HIV as well as alcohol intake, estrogen therapy, and dialysis treatment is increasingly recognized.

Manifestation of PCT is related to deficiency of the activity of the enzyme uroporphyrinogen decarboxylase. Therefore, confirmation of PCT is by demonstration of the excess porphyrin by-products resulting from the enzyme defect: chiefly uroporphyrin and hepatocarboxylic porphyrins in urine and plasma or serum and isocoproporphyrin in feces.





A 47-year-old man comes in to see you for a full physical exam and complains of new blisters forming on his hands for the last few months. He is a construction worker and has been busy for the summer season. While working, he "pulled a muscle" and has been taking naproxen daily to relieve the pain for the past 2-3 months. He noted shortly after initiation of naproxen that new blisters developed on the back of his hands. He appears healthy and tanned. Cutaneous examination shows no sclerodermoid changes on his face or hands and no hypertrichosis on his face. There are numerous hyperpigmented scars mixed with 2-3 tense bullae overlying normal skin on the dorsum of his hands. Around the scars, there are numerous 1-2 mm white papules. He denies any history of hepatitis. Your next step should be to

A. ask about gluten sensitivity and diarrhea because this is most likely Dermatitis herpetiformis


B. check antinuclear antibody level and look for dry eyes and mouth


C. check antinuclear antibody level and renal panel to rule out lupus erythematosus


D. search for an occult internal neoplasm


E. stop naproxen and check urine porphyrin level and a viral hepatitis panel

[ArchivalUser]

Thanks, What about Intermittent porphyria shuun??

[ArchivalUser]

Acute intermittent porphyria occurs in people of all ethnic groups but may be more common in people from Northern .
Acute intermittent porphyria (AIP) is one of the porphyrias, a group of diseases involving defects in heme metabolism and that results in excessive secretion of porphyrins and porphyrin precursors. AIP manifests itself by abdomen pain, neuropathies, and constipation, but, unlike most types of porphyria, patients with AIP do not have a rash.

The sequence of events in attacks usually is (1) abdominal pain, (2) psychiatric symptoms, such as hysteria, and (3) peripheral neuropathies, mainly motor neuropathies.

Skin rash.....DD between..AIP(No skin rash) and PCT(Has skin rash)

[ArchivalUser]

Great shuun

[ArchivalUser]

In PCT HBV OR HVC associated ?
in explanation in kaplan q bank they mention both but i one q they give both choices and ans is only hcv?
plz clearify this
Thanks

[ArchivalUser]

PCT is mostly HCV associated.
If they give picture of PCT and ask the next step ?
its hepatitis C testing