Skin , STD & Leprosy

Prajakta Bhisey, Vidya Kharkar, Sunanda Mahajan, Uday Khopkar


A 57 year old housewife came with red raised painful lesions over the dorsa of hands associated with high grade fever and joint pains since 15 days. Patient had h/o upper respiratory tract infection following which she had developed these lesions within a week. Patient had h/o similar episode 1 month back, which also was preceded by upper respiratory tract infection. The episode had responded dramatically to systemic steroids, which were tapered and stopped over 1 month. There was no history of mucosal involvement or history suggestive of malignancies. Past/ personal/ family history was not significant.

On Examination -

The vital parameters and general examination was normal. Cutaneous examination revealed multiple erythematous, tender, plaques, nodules over the dorsum of fingers and lateral aspect of palms, upper back, with few nodules showing evidence of pseudovesiculation and pustules. Systemic examination was normal.

Fig. 1: Bilateral erythematous edematous papules	Fig. 2: Erythematous tender plaques and nodules with pseudovesiculation.
Fig. 3: Side of palm-edematous papules and plaques with pseudovesiculation and pustules.

Investigations revealed leucocytosis (16,000/cmm), with neutrophilia (80%), raised ESR (108 mm/hr), ASLO titre was high (400 IU/ml) (normal - <250 IU/ml). Pus for smear culture and antibiotic sensitivity from the pustules grew Staphylococcus aureus and Klebsiella. Throat swab grew Streptococcus pyogenes. Skin biopsy was suggestive of dense neutrophilic infiltration with neutrophilic dust in the dermis with marked upper dermal edema.

H&E stained section (20 X) from papules showing upper dermal edema with neutrophilic perivascular infiltrate.

Fig HP1

What is your diagnosis?

Click here for Answer

 

 

 

 

 

 

 

Answer :

This is a case of Sweet's syndrome ( classical type) also called as acute febrile neutrophilic dermatosis.

Patient was treated with Tab Erythromycin for URTI and 40 mg of prednisolone. Patient responded dramatically with subsiding of lesions within a week.

Discussion

Sweet's syndrome, also called as acute febrile neutrophillic dermatosis, is characterized by fever, peripheral neutrophilia, acute onset of painful erythematous papules, plaques, nodules and histological findings of dense neutrophilic infiltrate without evidence of primary vasculitis 1.

There are three types of Sweets syndrome -

Firstly, Classical/ Idiopathic type which is seen in middle aged females, usually is associated with streptococcal URTI, irritable bowel syndrome2, pregnancy and has all the above features.

Second is the Malignancy associated which is usually associated with hematological malignancies like AML, Genitourinary, Breast, GI tumors in 15% of patients3.

Third is the drug induced type which is seen in patients receiving granulocyte- colony stimulating factor therapy (G-CSF). Additionally it can also be associated certain drugs like minocycline, trimethoprim - sulphamethoxazole, carbamazepine, hydrallazine and OCP

Pathogenesis - Various theories have been proposed, the exact cause is not known. Few of the theories suggest that it may be due to hypersensitivity to bacterial, viral, drug, tumor antigens, or due to presence of cytokine dysregulation leading to nonspecific leakage of IgG, IgM,C3 and fibrin from damaged vessels.

Clinical Features -

Patient has persistent high grade fever, neutrophilia, elevated ESR associated with erythematous to violaceous tender papules, nodules coalescing to form plaques over face, arms, neck4.

Pseudovesiculation may be present and may be studded with tiny pustules due to migration of neutrophils. Oral lesions are uncommon.Fever may/may not be present. Arthralgia / arthritis / myalgia may be present.

Diagnosis - Diagnostic criteria by Su and Liu5

Major Criteria-

1) Acute onset of typical skin lesions.
2) Histopathological findings typical of sweets syndrome.

Minor Criteria -

1) Fever > 38oC.
2) Associated malignancy, inflammatory disorder, antecedent respiratory and GI infection.
3) Excellent response to KI/steroids.
4) Investigations - ESR> 20mm/hr, leucocytes >8000 cells/cmm, neutrophils >70%, C-reactive protein - positive.

Drug induced sweets syndrome - 2 major criteria + 1 minor criteria + temporal relationship with drug.

Histopathological findings -

Dense, predominantly neutrophillic infiltrate in upper dermis with prominent papillary dermal edema, which may lead to subepidermal vesiculation neutrophilic karyorrhexis (neutrophilic dust) may be present.

Treatment -

1) Systemic steroids. 30 - 60 mg /day tapered to 10 mg within 4-6 wks.

2) KI 900mg daily for 2 weeks.

3) Topical potent steroids like clobetasol propionate or intralesional steroids ( 3mg/ml).

4) Oral colchicines 1.5 mg od for 7 days then taper to 0.5 mg over 3 weeks.

5) Others like clofazmine, dapsone, indomethacin can be used.

Course and prognosis -

It is a benign condition, the eruption may persist for a weeks/ months and then involute without leaving scars.

In this case, the Sweets syndrome was precipitated by URTI, which responded to a course of antibiotics and systemic steroids. Till today, patient has not developed any recurrences.

References -

1) Sweet RD :An acute febrile neutrophilic dermatosis Br.J.Dermatol 76,349,1964.

2) Felt DL et al :sweets syndrome:systemic signs and symptoms and associated disorders, Mayo clinic Proc 70:234,1995.

3) CohenPR, Kurzrock R:sweet syndrome and cancer lin Dermatol 11:149, 1993.

4) CohenPR, Kurzrock R:sweet syndrome: a neutrophillic dermatosis classically associated with acute onset and fever Clin Dermatol 2000 ,18:265-82.

5) Von den Driesch :Sweets syndrome (acute febrile neutrophilic dermatosis),J.Am Acad Dermatol 31:535, 1994.