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