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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 2620 - 22 July 2020 Posted By: Dr. Hafeez Diwan

Please read the clinical history and view the images by clicking on them before you proffer your diagnosis.
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14 year old female with PMH of asthma, eczema, and intermittent/relapsing predominantly unilateral vulvar swelling. There was a prior biopsy in 2008. It showed “lymphatic proliferation compatible with lymphangioma circumscriptum”. No significant history of GI symptoms, or sarcoidosis.
Physical exam: multiple pearly vesicular appearing lesions in a cluster predominantly on the right side of the vulva (biopsy part B), also at the same side, a pedunculated flesh colored lesion with a firm consistency (part A). Clinically it has been attributed to CALME syndrome.


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Richard Logan

Posted

I had never heard of CALME syndrome before, so I guess it is extremely rare.  The reported associated histology is really of normal constituents of vulvar tissue.  Here we have lymphangiectiasiae, fibrosis and rather sarcoidal granolomata.  I think this might be cutaneous Crohn's disease.

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John Zhang

Posted

Interesting case. Those small aggregates of epithelioid cells can be intravascular Langerhan cells (to be confirmed by IHC). Is there a consideration for Langerhan cell histiocytosis?

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Dr. Hafeez Diwan

Posted

There were two lesions biopsied.  The top left and bottom right panels are from the same lesion.  The other images are of the other lesion, which was pedunculated and fleshy.  There is no history of Crohn's disease.  These were interpreted as lymphangioma.  The fleshy lesion in addition shows intravascular histiocytosis, which is probably reactive in this setting.

CALME stands for "Childhood asymmetry labium majus enlargement."

I wanted to thank Dr. Sarah Al-Awami (who provided the photomicrographs and the history).

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Dr. Richard Carr

Posted

Interesting case. Certainly I've seen some very lymphangioma-like lesions in patient's with Crohn's or other reasons for lymphatic obstruction e.g. radiotherapy, obesity, prior surgery and pressure related etc.

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