Diagnostic Challenge

presented by

Amanda Oakley FRACP

Hamilton, New Zealand

June 9, 2007

(1) Clinical Director and Consultant Dermatologist, Department of Dermatology Health Waikato, Hamilton, New Zealand

Abstract A 14-year-old girl presented to the paediatricians with a fever, arthralgia and small purpuric and possibly target-like spots on her legs. Biopsy of the plaque on her neck shows full thickness necrosis histologically with little inflammation. EM-like. Has anyone seen anything like this? Is it erythema multiforme? Other possible diagnosis? Treatment?
14-year-old girl
One month
Face, trunk and legs
Clinical History

A month ago, a 14-year-old girl presented to the paediatricians with a fever, arthralgia and small purpuric and possibly target-like spots on her legs. She had been previously well and had taken no medications. After extensive negative investigation she was commenced on penicillin in case she had bacterial endocarditis, and was sent home.
She was readmitted yesterday with on-going fever and arthralgia, and crops of extremely painful plaques mainly affecting her face. Earlier lesions on her abdomen and limbs have resolved leaving marked hypo-hyperpigmentation or scarring. Biopsy of the plaque on her neck shows full thickness necrosis histologically with little inflammation. EM-like. Several new plaques have been observed to arise overnight despite an initial dose of prednisone 40mg. There is no mucosal involvement to date.
All tests so far negative - we are thinking up some more tests but we don't know the correct diagnosis. No other drugs as far as we can ascertain.


Diagnosis Erythema multiforme?
Questions and Teaching Points

Has anyone seen anything like this? Is it erythema multiforme? Other possible diagnosis? Treatment?

Comments from Faculty and Members

Rick Sontheimer MD, Professor of Dermatology, Department of Dermatology, University of Oklahoma Health Science Centre, Oklahoma City, OK, USA on June 10, 2007

Consider Rowell's syndrome and check for Ro/SS-A and La/SS-B antibodies. Would also not exclude a vasculitis (eg, Henoch-Schonlein purpura). Biopsy of the early, inflammatory, pre-necrotic phase of a new lesion could help address both possibilities.

David Elpern MD, Dermatologist, Williamstown, MA, USA on June 10, 2007

Are the lesions placed symmetrically? What is this child like? It seems that infection has been ruled out. Collagen vascular disease would be unlikely but would be considered.

I keep coming back to atypical drug eruption (var. FDE with serum-sickness like features) and after all else exhausted, factitial from an ingestant. For instance, she's been on acetoaminophen (Paracetamol) as in and out-patient. I think this will turn out to be an entity, however.

Steve Higgins MD, Resident in Dermatology, Duke University School of Medicine, Durham, NC, USA on June 10, 2007

I have never seen a case like this. Could it be erythema elevatum diutinum?

Ian McColl FRACP, Consultant Dermatologist, John Flynn Medical Centre, Tugun, Queensland, Australia on June 10, 2007

When a 14 years old girl presents with atypical skin lesions and full thickness skin necrosis without inflammation, my first thought is self induced dermatitis. The problem is the fever and arthralgia!

Full thickness skin necrosis could be seen with a drug reaction or viral induced erythema multiforme but you could also consider gonococcaemia, syphilis or hepatitis C, Kawasaki's disease or polyarteritis nodosa, Sneddon's syndrome,Henoch Schonlein syndrome, cryoglobulinaemia or other autoimmune collagen disease.

Phytophotodermatitis, a variant of Pityriasis lichenoides and glucagonoma syndrome would be even less likely than some of these other suggestions! Kikuchi's disease can give facial lesions like these but there is lymphadenopathy and the histology is not epidermal necrosis. Another biopsy please!

Henry Foong FRCP, Consultant Dermatologist, Ipoh, Malaysia on June 10, 2007

Thanks for presenting this interesting case. When I look at the clinical presentation, the lesions look like some form of lupus or vasculitis. However, the histology was unusual with full epidermal necrosis with little inflammation. This is more consistent with erythema multiforme - epidermal type.

Consider drug eruption, SLE, herpes simplex infection and mycoplasma infection too. Do blood tests to consider these possibilities. Perhaps, withhold prednisolone if it is not helping much.

Jayakar Thomas MD, Professor of Dermatology, Chennai, India on June 11, 2007

I strongly will go with a diagnosis of Dermatitis artefacta. Would certainly be cautious to rule out SLE/Rowell's syndrome.

Steven Chow FRCPI, Senior Consultant Dermatologist, Pantai Medical Centre, Kuala Lumpur, Malaysia on June 11, 2007

I am of the opinion that the child has atypical fixed drug eruption. The clinical pictures on the top left hand panel seems diagnostic.

Ian McColl FRACP, Consultant Dermatologist, John Flynn Medical Centre, Tugun, Queensland, Australia on June 11, 2007

I spent some time looking through Shelley last night. Ignoring the histopathology consider histoplasmosis as a cause of erythema multiforme and exclude Yersinia in the bowel and Mycoplasma in the lungs as causes. Rowell's syndrome also came up as an EM look alike presentation of subacute lupus as Rick has already commented. A biopsy of a fresh lesion would indeed help.

Khalifa Sharquie MD, Professor of Dermatology, College of Medicine, University of Baghdad, Baghdad, Iraq on June 11, 2007

It is interesting case. The skin features and the result of biopsy are suggestive of either FDE or dermatitis artefacta. Look at the history again and assess the psychology of the patients. If these are excluded, please remember SLE.

Omid Zalgari MD, Rasht, Iran on June 11, 2007

I think the diagnosis of erythema multiforme is still on the table! (as these days politicians say). Generalized fixed drug eruption, serum sickness and "urticaria multiforme" (a new entity?) are among my differentials. Thanks for sharing this interesting case.

Muhsin Aldhalimi MD, Head, Department of Dermatology, College of Medicine, Kufa, Iraq on June 11, 2007

I think that tumid type of localised cutaneous lupus erythematosus is the most probable diagnosis. Healing on systemic steroids and the presence of dyspigmentation - hypo and hyperpigmentation- and early scarring greatly support this diagnosis. Further investigations to confirm it is needed. Fixed drug eruption was the first diagnosis that one think of just by looking on the photos without reading the presentation

Khalid Al-Alboud MD, Consultant Dermatologist, Makkah, Saudi Arabia on June 11, 2007

I have similar feeling of Dr David that , this might be dermatitis artifacta.

Nurul Amin MD FRCP, Professor of Dermatology, Dhaka, Bangladesh on June 11, 2007

In my opinion we should look into the previous drug history and close observation for dermatitis artefacta.

Choon Siew Eng FRCP, Consultant Dermatologist, Johor Bahru, Malaysia on June 11, 2007

This looks like recurrent FDE. Hence, it is very important to look for the culprit drug. Had similar patients whose lesions become more and more with repeated exposure. One eventually developed EM major secondary to repeated exposure to mefenamic acid.

Amanda Oakley FRACP, Hamilton, New Zealand on June 11, 2007

Update on the case. Thank you for your contributions.

The lesions are peeling off her face and are much less painful, but she has developed a new plaque on her abdomen today. She has not been febrile in hospital and now appears quite well (on prednisone however).

Again I asked about medications and this time she has remembered taking paracetamol for headaches! I have crossed it off her drug chart and hope that fixed drug eruption is the correct explanation.

Jag Bhawan MD, Professor of Dermatology & Pathology, Boston Univ. Sch of Medicine, Boston, MA, USA on June 12, 2007

Interesting and challenging case! I would think it represents some form of drug eruption presenting as erythema multiform/toxic epidermal necrolysis. Biopsy of an early lesion may be of help. Does the patient have any lymphadenopathy, hepato or splenomegaly? I do not think it is factitial!

Khaled el-hoshy MD, Troy, Michigan, USA on June 12, 2007

Dermatitis Artefacta

Azad Kassim MD, Consultant Dermatologist, Hasa, Saudi Arabia on June 12, 2007

Really it is very interesting case. If there is no documented fever (reported in hospital), still possibility of Dermatitis Artifacta is very high, based on the configuration of lesions, their characteristic shapes, sites and color. Also some lesions are leaving scars behind. And the most important issue is that the histopothology of these lesions is not specific to any dermatosis, but it favours facticial dermatitis (Epidermal necrosis and damage with little or sparse dermal inflammation).

Ibrahim Misk MD, Dermatologist, Jordan on June 14, 2007

Interesting case. Many differentials: fixed drug eruption, dermatitis actefacta?

Update on the Case:

Amanda Oakley FRACP, Hamilton, New Zealand on June 16, 2007

Further update; A biopsy of a new lesion showed the same EM-like changes with some eosinophils - possible drug eruption. However new lesions developed 2 days after stopping paracetamol and while on high dose prednisone. We do not think they are artefactual but she is well and no fever has been documented while in hospital. I believe she has now been discharged (she is primarily under the care of the paediatricians).

Henry Foong FRCP, Ipoh, Malaysia on June 16, 2007

If the repeated biopsy of the new lesion showed similar EM-like changes, then the actual diagnosis of this case would be erythema multiforme and not fixed drug eruption. Apart from drug eruptions, erythema multiforme-like lesions can occur in SLE (Rowell's Syndrome) so it is important to check for ANA, anti-Ro, anti-La and rheumatoid factor as well.

Update on the case by Amanda Oakley FRACP on June 29, 2007

Yes, it turned out to be a chemical burn! She had been puffing salbutamol on the skin. The fever and arthralgia were imaginary - anxious mother! Apparently this injury is well described in teenagers' online literature. Thanks for your help with this case.


R Patel and S J Potter. Ten puffs too many. Archives of Disease in Childhood 2004;89:1129 (Full text)

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