This document describes the case of a 48-year-old woman who presented with joint pain and was found to have an elevated ASO level and was diagnosed with rheumatic fever. She later developed fever, breathlessness, and a skin rash. A skin biopsy showed features of Sweet's syndrome. Her condition improved with treatment but she later had recurrent fever and joint swelling. She was ultimately diagnosed with Sweet's syndrome.

1. Sweet syndrome to ?
Dr. Chit Soe
Associate Professor
TSGH

2. Pain joints + ASO
• Daw MM Myint,
• 48 year, married
• 75, 51st
street, Yangon
• On 12.1.01
• Pain joints off and on for 3 weeks
• Consulted to a cardiologist, at private clinic
• ASO reflexly checked and as usual found to be
raised (300 IU)

3. PE
• Apart from arthralgia of ankles and wrists,
no joint swelling was noted.
• No skin rash
• No heart murmur
• Other systems were normal
• Diagnosed- ? Rheumatic fever
• Received 3 weekly inj Pen.

4. Fever + Dyspnoea
• On 15.9.01
• She suffered high fever and breathlessness
• Admitted to cardiac medical unit, YGH
• CP showed neutrophil leucocytosis
(13,000/cumm with 80% neutrophil)
• MP, Urine RE NAD
• ECHO- NAD

5. • CXR show
opacity in left
middle zone

6. ? Pneumonia
• IV ceftriaxone 1 G BD started for fever and
lung shadows

7. Skin Rash
• On 19.9.01
• Erythematous skin rash appears
• Dermatologist was called for consultation

8. Fever, Pneumonitis, skin rash ?SLE

9. ANA negative

10. Skin Biopsy

11. Microscropic
• Section of the skin show markedly
acanthotic epidermis with parakaratosis.
Epidermis show features of
pseudoepitheiomatous hyperplasia with
spongiosis in many areas.
• The parakeratotic cells are intermingled
with neutrophils.
• Characteristic spongioform pustule of
Reiter’s diseases is not found.

12. Patient Recovered
• Sputum C & S was sterile
• Fever comes down
• DC on 25.9.01

13. Recurrent of fever + Joint
swelling
• On 27.9.01
• Very high fever at home
• Increasing rash
• Joint swellings
• Admitted to Asia Royal under
Rheumatologist

14. • CT chest

15. • CT chest
• Opacity in left middle
zone

16. Collapse consolidation, ? TB

17. Sweet syndrome
• Sputum AFB (-) for 3 days
• ICT TB (-)
• Blood culture- sterile
• Repeat ANF (-)

18. Sommer S, Wilkinson SM, Merchant WJ, Goulden V.
Sweet's syndrome presenting as palmoplantar pustulosis.
: J Am Acad Dermatol. 2000 Feb; 42(2 Pt 2): 332-4.
• Sweet's syndrome was initially described
as a reactive dermatosis characterized by
sudden onset of fever, leucocytosis, and
raised erythematous plaques infiltrated
with neutrophils, pneumonitis and
therefore called acute febrile neutrophilic
dermatosis.

19. Slow resolved pneumonia
• Unasyn
• (Ampicillin + salbactam)
• And
• Clarithromycin

20. Psoriasis- Autoimmune process
• Methylprednisolone pulse therapy
• 1G infusion OD for 3 days
• Methotrexate 10mg stat orally and weekly
• Folic acid 5 mg orally weekly

21. Calcipotriol ointment

26. Onycholysis

27. Gradual improvement

28. Much improved

29. Scherak O, Kolarz G, Popp W, Wottawa A, Ritschka L, Braun O.
Lung involvement in rheumatoid factor-negative arthritis.
Scand J Rheumatol. 1993; 22(5): 225-8.
• Transbronchial biopsy was performed on 9
patients with SNA and on 59 patients with RA.
• Abnormal histologic features of lung tissue were
observed in 4 out of 9 patients with SNA (2 with
fibrosis, 1 with follicular lymphoid hyperplasia, 1
with desquamative interstitial pneumonitis).
• The abnormal lung histology in RA patients was
more pronounced, however, the differences
between SNA and RA were not significant.

30. Cuhadaroglu C, Korular D, Erelel M, Kiyan E, Kilicaslan Z.
Respiratory distress with acitretin, reversal by corticosteroid.
Dermatol Online J. 2001 Dec; 7(2): 5.
• The retinoic acid derivatives are used for
disorders of keratinization such as
psoriasis. Acitretin, which is a synthetic,
aromatic derivative of retinoic acid, is
frequently used to treat psoriasis. The
retinoic acid syndrome (RAS), described
with all-trans-retinoic acid (ATRA) in
patients and characterized by fever and
respiratory distress.

31. • .
• Soriatane (Acitretin) - Wow and this is one
of the newer "wonder drugs." Usually the
skin condition returns and you may need
to begin taking Acitretin again.
• Acitretin must not be used to treat women
who are able to bear children.

32. Salaffi F, Manganelli P, Carotti M, Subiaco S, Lamanna G, Cervini C.
Methotrexate-induced pneumonitis in patients with rheumatoid
arthritis and psoriatic arthritis: report of five cases and review of the
literature.
Clin Rheumatol. 1997 May; 16(3): 296-304.
• Pneumonitis is emerging as one of the most
unpredictable and potentially serious, adverse effects of
treatment with MTX.
• Its prevalence in rheumatoid arthritis (RA) has been
estimated from several retrospective and prospective
studies to range from 0.3% to 18%. On the other hand,
MTX-induced pneumonitis seems to be very rare in
psoriatic arthritis (PsA).
• Our review of 194 RA patients and 38 PsA patients
receiving MTX has identified four RA patients and one
PsA patient with MTX-induced pneumonitis, giving a
prevalence of 2.1% and 0.03%,

33. Safer version
• UVB treatment was first used in 1925 by
Dr Goeckerman, and
• Coal Tar treatment has existed for more
than 100 years.

34. Pipitone N, Kingsley GH, Manzo A, Scott DL, Pitzalis C.
Current concepts and new developments in the treatment of psoriatic
arthritis.
Rheumatology (Oxford). 2003 Oct;42(10):1138-48. Epub 2003 Jun 16.
• Mild forms can usually be controlled by non-steroidal
anti-inflammatory drugs (NSAIDs).
• Intra-articular glucocorticoid injections are indicated in
patients with persistent mono- or oligoarthritis.
• Patients with severe and progressive articular disease
not responsive to NSAIDs should be treated with
disease-modifying anti-rheumatic drugs (DMARDs) to
prevent joint damage and disability.
• Currently, methotrexate and sulphasalazine are
considered the DMARDs of choice,
• Recently, tumour necrosis factor alpha inhibitors have
proved effective in many PsA patients with
pelvispondylitis or recalcitrant peripheral synovitis

35. Wozel G, Pfeiffer C.
Leflunomide--a new drug for pharmacological immunomodulation Hautarzt.
2002 May;53(5):309-15.
• The novel immunomodulatory agent leflunomide exhibits
a strong anti-inflammatory action.
• This isoxazole derivative is chemically unrelated to any
hitherto applied immunosuppressants now leflunomide
has just been approved for therapy of rheumatoid
arthritis, its mechanism of action affects multiple
inflammatory pathways, thereby suggesting it to be a
potent therapeutic agent in autoimmune diseases, graft
rejection, and tumour therapy.
• First dermatological experience has been gained in
psoriasis and bullous pemphigoid. The role of
leflunomide in the dermatologist's therapeutic
armamentarium will evolve during the next years.

36. Latest
• dermalight products solve the problems of over-
exposure to ultraviolet light by maximizing the
delivery of narrow-band UVB radiation (in the
311-312nm range, the most beneficial
component of natural sunlight) while minimizing
exposure to superfluous UV.
• UVB narrow-band also avoids the adverse side
effects of the psoralen drugs used in
conventional PUVA therapy, since UVB
treatment requires no supplemental drugs.)
• UVB treatment is around 83% successful and
narrow band UVB treatment is around 87%
successful.

37. Narrow band UVB 311

38. False drugs
• Skin-Cap is a product from Spain that contains a
very potent and highly dangerous steroid that is
illegal in most countries, it also DOES NOT list
the steroid in the ingredients and claims to have
FDA approval, when the FDA have given no
such approval.
• various telemarketing campaigns of internet and
newspaper and radio ads for topical zinc
pyrithione products

39. Lessons
• Joint pain + ASO = Rheumatic fever
• CT Granuloma = TB
• Foreign skin prep = Good
• Rash + multisystem= SLE