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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 1525 - 28 April Posted By: Guest

Please read the clinical history and view the images by clicking on them before you proffer your diagnosis.
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10-year-old girl undergoing long-term work-up by rheumatology for an autoinflammatory disorder presents for evaluation of recurrent, tender papules and plaques involving the face, trunk, and extremities since 3 weeks of age. Cutaneous lesions have flared over the last 5 days following a decrease in her daily dose of prednisone. She has a past medical history of recurrent fever, panniculitis, elevated acute phase reactants, transient arthritis, and aseptic meningitis. There is no family history of autoimmune disease or immunodeficiency. She is on methotrexate 17.5 mg PO weekly and prednisone 17.5 mg PO daily. Her labs show intermittent elevations in ESR, CRP, and AST/ALT.

Dr Hafeez Diwan.


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Raul Perret

Posted

Still's disease in first place, consider Kawasaki as a differential for cpc (any mucous involvement, adenopathy? asian patient?)

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Raul Perret

Posted

Clinically is more consistent with still's. I dont know if you agree with me but considering only the histology i though of sweet's syndrome or even arthropod bite. It was after Reading the clinical picture that Still came to my mind and I gave more importance to the diskeratotic cell's seen high on the epidermis as well as the important inflammatory infiltrate

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Dr. Mona Abdel-Halim

Posted

Actually, I thought of Sweet syndrome. Clinically fits. Also it can occur in association with rheumatological diseases. Still's I think would be different clinically with the main presentation being a macular rash faintly erythematous looks like an exanthem more than being tender papules and plaques. Also the subepidermal papillary dermal edema is characteristic of sweet's

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Raul Perret

Posted

I still think the patient has still's disease maybe with superimposed  Sweets syndrome, but histologically I see upper epidermal diskeratocytes, a feature not seen in Sweet's so would state all the findings in the final diagnosis and consider overlapping for clinical correlation

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Arti Bakshi

Posted

Agree with the basic pattern of neutrophilic dermatosis.

Mona's point is valid that the rash should be more exanthematous for Still's. On the other hand, It is quite an early onset for Sweets (from 3 weeks). Neutrophilic dermatosis can be associated with a number of auto-inflammatory syndromes. FMF can have tender papules and plaques, however neutrophilic infiltrate is usually milder. The cryoprin associated syndromes have a urticarial presentation. There are some rarer subtypes such as CANDLE syndrome and HIDS, which can mimic Sweets. Wonder if this is one of them. Interesting case!!

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Nitin Khirwadkar

Posted

Agree with above. Favour Sweet's/periodic fever syndrome/cryopyrin aasociated.

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vincenzo polizzi

Posted

I just knowed the Sweet's syndrome, so i thought of it...But i'm learning a lot about CANDLE and CAPS etc, thanks to all of you! 

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Dr. Hafeez Diwan

Posted

This is CANDLE, as has been so astutely pointed out by some.  Essentially a neutrophilic dermatosis (the histological features resemble those of histiocytoid Sweet's).

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Arti Bakshi

Posted

Wow, so this was really CANDLE!  Never seen a case before, so thanks Dr Diwan for sharing a great case.

Looking at the images again, there is a mixture of neutrophils and some less mature forms too, which would be consistent. Have added a link to an article discussing the histology of this entity.

 

http://journals.lww.com/amjdermatopathology/Abstract/2015/07000/Histologic_and_Immunohistochemical_Features_of_the.2.aspx

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vincenzo polizzi

Posted

Dr Hafeez, were the mononuclear atypical cell in dermis CD163+ ?

Thank very much fro this interesting case and Good Week End!

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Guest parasa gayatri devi

Posted

thank you all  for the interesting case

 

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