Sweet syndrome to ?

Dr. Chit Soe

Associate Professor

TSGH

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)

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.

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

• CXR show opacity in left middle zone

? Pneumonia

• IV ceftriaxone 1 G BD started for fever and lung shadows

Skin Rash

• On 19.9.01

• Erythematous skin rash appears

• Dermatologist was called for consultation

Fever, Pneumonitis, skin rash ?SLE

ANA negative

Skin Biopsy

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.

Patient Recovered

• Sputum C & S was sterile

• Fever comes down

• DC on 25.9.01

Recurrent of fever + Joint swelling

• On 27.9.01

• Very high fever at home• Increasing rash• Joint swellings

• Admitted to Asia Royal under Rheumatologist

• CT chest

• CT chest• Opacity in left middle

zone

Collapse consolidation, ? TB

Sweet syndrome

• Sputum AFB (-) for 3 days

• ICT TB (-)

• Blood culture- sterile

• Repeat ANF (-)

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.

Slow resolved pneumonia

• Unasyn

• (Ampicillin + salbactam)

• And

• Clarithromycin

Psoriasis- Autoimmune process

• Methylprednisolone pulse therapy

• 1G infusion OD for 3 days

• Methotrexate 10mg stat orally and weekly

• Folic acid 5 mg orally weekly

Calcipotriol ointment

Onycholysis

Gradual improvement

Much improved

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.

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.

• .

• 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.

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%,

Safer version

• UVB treatment was first used in 1925 by Dr Goeckerman, and

• Coal Tar treatment has existed for more than 100 years.

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

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.

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. 

Narrow band UVB 311

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

Lessons

• Joint pain + ASO = Rheumatic fever

• CT Granuloma = TB

• Foreign skin prep = Good

• Rash + multisystem= SLE