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In this section we have spot diagnoses posted on a daily basis since June 2010, now over 1700! You can review the archived cases and read the suggested diagnoses by users and the final comment by Dr Uma Sundram, the Editor-in-Chief and main spot diagnosis host. Case are uploaded each week day by 10 a.m. UK time with the correct diagnosis will generally be posted at 8 p.m. UK time. Why not view the most recent spot diagnosis and proffer a diagnosis?

Case Number : Case 2931 - 30 September 2021 Posted By: Saleem Taibjee

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11 year old girl. Persistent and progressive hyperkeratotic rash on flexures and feet.


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Saman Fatah

Posted

There is alternating parakeraosis/orthokeratosis with irregular acanthosis and few other changes in epidermis. I wondered about PRP juvenile type but the flexural involvement is some what unusual clinically.

Would be interesting to see other thoughts?

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Anil Patki

Posted (edited)

Alternating parakeratosis and hyperkeratosis are seen only in horizontal direction and not in checkerboard pattern. Acanthosis and papillomatosis are a bit excessive for the diagnosis of PRP. I think that this could be an inflammatory linear verrucous epidermal nevus (ILVEN) if it clinically fits.

Edited by Anil Patki
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Richard Logan

Posted

Actually I agree with Saman that there are histological features of PRP.  The parakeratosis and orthokertosis do alternate both vertically and horizontally, and there is some fusion of adjacent rete ridges.  The papillomatosis is rather more than is normally seen in biopsies of PRP, but that may be a feature of the site of the biopsy, which I presume was taken from the foot.  As Saman says, flexural involvement is not usual in the various forms of juvenile PRP.  I think is is unlikely that this is ILVEN as the history suggests that there is symmetrical involvement.

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vincenzo

Posted

Too papillomatous for PRP. ILVEN is histologically a better option, for me. But I’m thinking about some other...

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Saleem Taibjee

Posted

Interesting dialogue...

We discussed this case at MDT, and the favoured clinical diagnosis was eventually Progressive Symmetrical Erythrokeratoderma. The literature indicates that some patients have an identified mutation in loricrin, genetic testing is awaited on our patient. The limited descriptions of the histology in the literature indicate hyperkeratosis with papillomatosis and acanthosis. Parakeratosis is not really mentioned, although it does seem to be present in our case. Clinically this doesn't really fit for ILVEN which is a mosaic disorder, often in a Blaschkoid distribution. PRP doesn't usually show this degree of papillomatosis, and also didn't fit particularly well with the clinical features.

BW Saleem

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vincenzo

Posted

I was thinking about some variant of ichtiosis, but this is really an unknown chapter for me....

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