Tuesday, April 06, 2010

Nail Dystrophy in an Eight Year-Old Girl

Introduction:  In the past, we published a case of localized acrodermatitis continua.  The father of a child with this diagnosis in the U.S. came across our post on VGRD and asked our advice for his daughter.  Your opinions may help with the diagnosis and management of this child.  One can only imagine how this disorder impacts on a young child. Perhaps, one of us has had a favorable outcome with a similar patient.


History:  Please help with an opinion on our eight year-old daughter who has had an acral dermatitis for the past 5 years.   The swelling started at the cuticle and slowly moved back towards the first knuckle over the years and was associated with itching. Initially it was diagnosed as insect bites.  About a year ago her fingers became more swollen and a doctor made a clinical diagnosis of fungus (no tests were done).  She was treated first with vinegar soaks, then triamcinalone cream then Grifulvin 125mg/tsp.  None was effective and we then saw a new dermatologist who referred us to a pediatric dermatologist who she made a diagnosis: Acrodermatitis Continua of Hallopeau.  She did a fungal culture which grew out a soil contaminant that was not felt to be significant.   Our daughter is presently on clobetasol ointment.  The nail looks a bit better but not the skin.  Treatment discussions so far have included Thalidomide, Psoralen plus UVA or UVB, Acitretin, Methotrexate and others.  We know that these medications can have serious side-effects and that this disease can be resistant to treatment.  Our daughter has a lot of finger pain and can't pick up thing with her fingers.  She is only a child and we'd appreciate your thoughts.

Clinical Photos:






































Questions:
1) Are there alternative diagnoses?
2) What therapies have you had success within similar cases?
3)  Any further work-up?

11 comments:

  1. Ashok K. SharmaApril 07, 2010

    I can well understand the concern of the parents.
    The clinical pictures do suggest Acrodermatitis continua(although I would like to see a picture of the palm also).
    I have noted the tried therapies. I have used Protopic oint (tacrolimus) and Daivobet oint (calcipotriol and betamethasone) for a couple of my cases with appreciable result (skin shows improvement over about 6-8 weeks with nails showing some improvement over about 4-6 months). It is a difficult condition to manage in all age groups.Severely dystrophic nails in my cases have never shown a complete recovery to normal-like nail plates.
    For cases with multiple nail involvement (as in the present case), biologics like etanercept may hold some promise. I am contemplating trying etanercept for some of the cases I see in future.

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  2. I find Dr Ashok Sharma's comments useful. This is a difficult case to manage. There is no long term effective treatment. Of all the topical treatments, I favour tacrolimus ointment 0.1%. If it is troublesome, I would consider oral acetretin but there is long term adverse effects that need to be considered especially to skeletal and bone changes. Cyclosporin or methotrexate may be a better option. Courses of oral antibiotics may be helpful. When the clinical trials of biologics eg usetekinumab and infliximab are available, then perhaps we may consider them too.

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  3. I would consider xrays to rule out bone changes or arthritis. This may also guide tx. Consider low dose mtx (2.5mg/wk) or anti-TNF drugs. Continuing topical clobetasol and perhaps considering Cordran tape are other thoughts.

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  4. Why don't you try topical Tazarotene 0.1% in this child.

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  5. A colleague with a special interest in nail disorders sent this comment: "Thank you for for the pics. They really look like Acrodermatitis continua of Hallopeau. However, before embarking on any potentially harmful therapies I would recommend a biopsy. A 3 or 4 mm punch is easily tolerated even by a child and is, in the long run, much better than a long-term, potentially hazardous treatment.

    I would also hesitate to give MTX to a child and have no own experience with thalidomide. A TNF-alpha blocker might be an option is abd when the comination of a vitamin D derivative with a potent setroid fails.

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  6. Dear All
    My approach would be:
    1. Do a 3 mm punch biopsy, to confirm the diagnosis.
    2. After confirming it as Acrodermatitis continua,
    3. I have experience with Tab. Dapsone 1- 2 mg/kg body weight per day, in children. Start only after getting G6PD levels done. Monitor Hemogram, liver function tests.
    4. Apply topical Halobetasol cream at night.
    There after one can think of Methotrexate and biologics.
    All the best
    DR Manish Pahwa
    New Delhi

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  7. I have not treated this condition in a child. In adults most will respond to a strong topical steroid such as Diprosone OV or Clobetasol but there are intermittent flares and most patients require continuous use to keep the condition at bay. Tacrolimus topically BD can be used to prevent flares. In a recalcitrant case at this age Methotrexate would be my next choice. Some of my colleagues recommend methotrexate soaks but I would use it orally in small weekly doses to try to control the condition. Pediatric rheumatologists use it a lot in children with rheumatoid and psoriatic arthritis and the children usually cope very well with fewer issues than adults.

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  8. I agree with Dr Pahwa completely - confirm with biopsy, then dapsone, failing that, methotrexate.

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  9. Julianne MannApril 11, 2010

    I'm a dermatology resident in Oregon and have shared this case with one of my pediatric dermatology attendings, who agrees that this looks like Acrodermatitis continua of Hallopeau. He has had success with topical steroids, Dovonex, and Protopic - if no response to these would consider narrow band UVB or Excimer laser before going on to a systemic medication like MTX.

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  10. This comment has been removed by a blog administrator.

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  11. Oh !! I have the same problem , I have visited may Doctors and they said me just lies, one of them said me said m you have Onycholysis , it is a loosening of the exposed portion of the nail from the nail bed, usually beginning at the free edge and continuing to the lunula.!!

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