| Abstract |
A 52-year-old man presented with 2-week history of fever,
malaise and painful erythematous plaques over the face, chest
and upper limbs. A diagnosis of sweet's syndrome was made. The
management of this condistion was discussed. |
Patient |
C.G., 52-year-old man |
Duration |
5 months |
Distribution |
Face, Chest and Upper limbs |
| History |
52-year-old Indian presented with fever, malaise and painful
erythematous plaques over the face, chest and upper limbs
of 2 weeks duration. He had an URTI 3 weeks prior to the onset
of skin lesions. There was no history of bleeding disorder,urinary
or bowel symptoms. There was no history of drug intake prior
to onset of lesions.
Past history: he had similar lesions 5 months ago which had
cleared spontaneously after 3 weeks with post inflammatory
pigmentation.
Family history: His father died of leukemia (type not known)
at the age of 50 years.
|
| Physical Examination |
Patient was febrile-temp 38.2 degC. He
had multiple erythematous tender plaques over the malar region,
upper limbs and presternal region of varying sizes and shapes.
Some of them were arciform. There was no sensory impairment
or nerve thickening. There was no mucosal lesions. No generalised
lymphadenopathy or organomegaly. |
| Images |
|
| Laboratory Data |
Total WBC count - Differential count - P75%, L23%, E2%
Hb/RBC Count - within normal limits
ESR - 34mm/hr
Peripheral blood film showed leukocytosis with neutrophilia
|
| Histopathology |
There was a dense dermal neutrophilic infiltrate involving
the upper and mid dermis with leukocytoclasis, extravasation
of RBC's.There was no frank vasculitis,or blister formation.There
were no atypical cells in the inflammatory infiltrate
|
| Diagnosis |
Sweet's Syndrome |
|
Reasons Presented |
Probably an under-diagnosed condition since fever and neutrophilia
is not seen in all cases (though called acute febrile neutrophilic
dermatosis).
Sweet's syndrome can herald the onset of malignancy.
|
| Questions |
1) Should the patient receive episodic therapy with steroids
or does he require maintenance treatment with drugs like Dapsone
2) Is he at a risk of malignancy especially since his father
died of leukemia
3) How long should he be followed up?
|
| References |
1)Sweet RD: An acute febrile neutrophilic dermatosis. Br
J Dermatology 76:349,1964
2)Cohen PR et al: Malignancy associated sweet's syndrome: Review
of world literature. J Clin Oncol 6:1887, 1988
|
| Keywords |
sweet's syndrome, febrile neutrophilic dermatoses |
| Comments from Faculty and Members |
Jeffrey Callen MD, Professor of Medicine (Dermatology),
Chief, Division of Dermatology, Department of Medicine, University
of Louisville School of Medicine, Louisville, KY, USA
on July 27, 2006
1) Should the patient receive episodic therapy with steroids
or does he require maintenance treatment with drugs like Dapsone?
It sounds to me like he has had two episodes of Sweet's. With
only two such instances, I would not place the patient on chronic
therapy, but rather treat as you have with a short course of
corticosteroids. Should he develop more episodes that are frequent,
then I would consider suppressive therapy. Although dapsone
has been used, in my experience it is of limited value. There
are multiple therapies, but one of the more reliable is thalidomide.
2) Is he at a risk of malignancy especially since his father
died of leukemia? If the father's leukemia was genetically
based (Philadelphia chromosomal abnormality) then perhaps the
patient is at increased risk. However, I do not perform evaluation
beyond a CBC with a peripheral smear and perhaps a serum protein
electorphoresis.
3) How long should he be followed up?
If the disease is in remission, then you might consider allowing
the patient to contact you only when additional problems arise.
Davy Chan MD, Hong Kong, SAR China, on August
30, 2006
I don't think this man needs long term maintaince with dapsone.
However, given the family hostory of leukaemia and the ? relapse
nature of this episode, I think I will follow up the patients
regularly.
|
