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Simple cases
Case 47
Adult man complains of recurrent sores on hands.
History
How long have you had these lesions?
For about 9 months. Could it be my nerves. My wife and I are having lots of trouble. In addition, to relax a bit, I have started to drink again.
This skin disease is most common in middle-aged men, often alcoholics. It can also be associated with liver disease in the form of hepatitis B or C.
Do you have similar lesions elsewhere on your body?
No; just my hands. Sometimes I even get blisters.
This disease is usually limited to sun-exposed areas. The skin is fragile and easily bruised, forming erosions and blisters. The lesions heal with scars and milia.
What kind of work did you used to do?
Doc, I'm not retired; I am only 50. I am a gardener.
Affected patients often have marked actinic damage and appear older than their true age.
Do you have any other major illnesses? Have you had any major operations?
I had my tonsils out when I was 5. I have never had surgery since. About 5 years ago, the docs told me I had a little bit of sugar and some liver problems.
Good question. This disease is often associated with liver disease or diabetes mellitus.
Do you take any medications?
No. I used to take Antabuse, but I stopped a while back.
Essential question!! Several medications can make this disease worse. The most common trigger is however alcohol. There is also a familial form of the disease, but it is rare.
Have you noticed any other changes?
I noticed two things. Funny, but my urine got darker, like a amber beer. In addition, I started getting hair on my temples and cheeks, even though I'm loosing it on my head.
The urine in this disease is often dark and fluoresces orange with Wood light examination. Hypertrichosis of the cheeks and temples is another common finding.
Have you tried any treatment?
My family doctor gave me some cortisone cream but it didn't help.
Not surprising; corticosteroid creams play no role in managing this disease and might worsen the skin fragility.
Lesion
Choose the right efflorescences:
Serous blisters on the backs of the hands are absolutely classic for this disease.
Poikiloderma features atrophy, telangiectases and pigmentary changes. This is not seen here.
An erosion is a superficial skin defect; minor trauma produces erosions in this patient's fragile skin.
Hives are transient swellings, not seen here.
Diagnosis
Choose the right diagnosis:
The hands are a common site, but not with fragility and blisters. Acute dermatitis has erythema and sero papules; chronic dermatitis has lichenification.
Goodthis is a classic case. Skin fragility with blisters on the backs of the hands is porphyria cutanea tarda until proven otherwise. This is the only common form of porphyria and should be familiar to general physicians as well as dermatologists.
Pemphigus presents with fragile blisters, often in mouth, not limited to hands.
Once again, hands commonly involved but with violaceous papules over the knuckles (Gottron papules). No blisters.
Therapy
Choose the right therapy(ies):
As peculiar as it sounds, taking blood to reduce iron stores is the safest and best treatment for porphyria cutanea tarda.
Very low-dose chloroquine is also effective. CAVE: do not prescribe the doses of chloroquine used for malaria; they could be life-threatening.
Both are important, although neither is likely to bring relief without additional measures. In addition, all hepatotoxic medications should be avoided.
CAVE: the oral antidiabetic agents are mostly hepatotoxic and must be avoided in porphyria cutanea tarda.
Porphyria cutanea tarda is not a bullous autoimmune disease and should not be treated as such. This would be a major error.
Test
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