Kavita D. Mogale
From the Departments of Pediatrics J.N. Medical College, Belgaum, Karnataka,
Reprint requests; Dr. P. V. Havaldar, Professor, A-5, Staff Quarters, J.N.
Belgaum 590 010, Karnataka, India.
Manuscript Received: November 19, 1997; Initial review completed: November 3D,
1997; Revision Accepted: April 19, 1998
Acute febrile neutrophilic dermatosis was first reported by Sweet in 1964(1). So
far more than 100 cases have been reported and most of them in adults(2).
Till 1996, 20 children with this Syndrome were reported in the literature(2-6),
none of them are from the Indian Subcontinent. Here we report one such case with
classical features of Sweet's syndrome.
An ll-year-girl presented with history of fever and skin lesions of six months
duration. There was no history of contact with tuberculosis. She did not have
any com- plaints of joint pains, cough, loss of weight, loss of appetite or
bleeding manifestations. On examination she was well nourished, pale with high
grade fever, but not toxic. There were no purpuric spots or lymphadenopathy. The
showed painful erythematous oval, crusted plaques ranging from the size of 1 x 1
cm to 3 x 3 cm situated over all four extremities
1). The crusts were yellowish brown firmly adherent and bled on removal. The
margins of the ulcers were not under- mined. They were 20-30 in number with
fresh small, waxing and waning pustular eruptions. Abdominal examination showed
hepatomegaly of 3 cm while the other systems were normal. The laboratory
investigations revealed hemoglobin of 6.3 g/ dl, total leukocyte count of 15,200
cells/cu mm, with neutrophilia of 69%. ESR was 100 m/l h by Westergren's method.
Serum proteins, serum bilirubin and creatinine were within normal limits. Of the
immunoglobulins IgG (2800 mg/ dl; normal 779- 1456 mg/dl) and IgA (550 mg/dl;
12-208 mg/ dl) were elevated and IgM (150 mg/dl; normal 35-132 mg/dl) was
normal. LE cell phenomenon and HBs Ag were non contributory.
Typical lesions of
syndrome over the extremities.
Serum complement C3 and C4 levels and the leukocyte function tests like
Nitroblue tetrozolium test, candidacidal assay, phagocytosis of killed C.
albicans and neutrophil chemotaxis assay were within normal limits. X-ray
chest was also normal. The cultures from the skin lesions taken thrice from
depth of the ulcers after removal of cursts and puncturing of fresh lesions, did
not yield any bacteria or fungus. The Mauntoux test was negative. Bone marrow
which was normoblastic, normocellular and normal liver biopsy did not help to
find out the cause of the anemia or any disease associated with this syndrome.
Skin biopsy showed hyperkeratosis with crust formation and subepidermal
inflammatory cell infiltrates mainly neutrophils with few lymphocytes and
eosinophils with no evidence of vasculitis (Fig. 2). She was treated with
multiple antibiotics like cefotaxime (100 mg/kg! day), cloxacillin (50 mg/kg/
day), gentamicin (5 mg/kg/ day) and ciprofloxacin (20 mg/kg/ day), each for a
period of two weeks with no response. She was then put on steroids (prednisone 2
mg/kg/day in 3 divided doses) with no supplementary antibiotics and the lesions
started to heal with falling off the crusts', leaving healthy non-granulomatous
epidermis over a period of 6-8 days. There was no residual scar formation in any
lesions. At the end of 8 weeks all lesions disappeared and she was discharged on
low maintenance dose of corticosteroids (prednisone 5 mg/ day). Currently the
patient is under regular follow-up with no skin lesions for 2 months.
Sweet syndrome is characterized by fever, leukocytosis, neutrophilia, multiple
sharply demarcated painful erythematous plaques mainly on face and extremities with dermal infiltration of mature neutrophils without vasculitis(7). It usually lasts for 3-9 weeks and resolves spontaneously. However, recurrent lesions lasting over 12 years have been observed(6). A rapid
response to cortkosteroids has also been reported(6).
Fig. 2. Photograph to show edema beneath the epidermis. Dense infiltrate of neutrophils, eosinophils surround blood vessels and appendages (H&E x35).
The characteristic skin histology consists of infiltrates- in upper and mid dermis which are most intense around blood vessels. Neutrophils
predominate in early stages whereas mononuclear cells and histiocytes may be present in later stages. There is no evidence or leucocytoclastic vasculitis. Immune complex deposits are not always seen but one patient with linear homogenous immunoglobulin M has been reported(6).
Sweet syndrome has been associated
with malignancies, inflammatory bowel disease, rheumatological disorders, glycogen storage disorders, congenital dys-erythropoetic anemia, Fanconi's anemia, multifocal sterile osteomyelitis, aspetic
meningitis, respiratory tract infection, Behcet's disease, erythema multiforme and granulocyte colony stimulating factor therapy( 6,7).
Two major and six minor criteria for diagnosis of Sweet syndrome have been proposed. A definitive diagnosis requires presence of both major and atleast two of the minor criteria. The major criteria are: (i) abrupt onset of tender erythematous or violaceous plaques, (ii) predominantly neutrophilic infiltrates in the dermis without leucocytoclastic vasculitis. The minor criteria include: (i) fever, (ii) arthralgia, (iii) conjunctivitis, (iv) underlying conditions as malignancy, (v) leucocytosis, (vi)
lack of response to antibiotic treatment and response to corticosteroid therapy(6).
The present case described typically fits in the diagnostic criteria having both
the major and three of the six minor criteria being present. We Were unable to diagnose any underlying disease like malignancy OF hematological disorders. It has been noted
that whenever underlying malignancy is not found,' it predominantly affects females, thus the suspicion for malignancy in Sweet's syndrome should be higher in male patients(7). Sweet syndrome is extremely rare in children. The exact etiology is not known. It is assumed to occur as a hyper- sensitivity reaction in bacterial, viral or tumor antigen in response to inappropriate secretion of endogenous cytokines as interleukin I or GCSF(6). The present case described though not having any under- lying disease is under surveillance for the same. The important differential diagnosis for Sweet syndrome is pyoderma gangrenosum.
Pyoderma gangrenosum and Sweet's syndrome have many features in common like, neutrophilic infiltration, altered immunological
reactivity, association with malignancy, particularly hematological, development after GCSF therapy, response to immunosuppression treatment and reports of co-existence in a single patient. Hence, many authors believe that pyoderma gangrenosum and Sweet's Syndrome represent a continuum of spectrum of disease resulting from a single pathophysiological phenomenon, the common denominator being an inflammatory process mediated by neutrophils. Unlike pyoderma gangrenosum the lesions are not ulcerative and tend to heal without scarring(7).
Sweet's syndrome shows rapid response to corticosteroids, but recurrence is common. Antibiotics are found to be inef- fective.Potassium iodine, aspirin, colchicine and indomethacin have been tried but found to be in no way superior to corticosteroids(7). Cyclosporine had been found to be useful but necessitates long term therapy. Doxycycline can be used in chronic cases(7). The average duration of illness has shown to be 3-8 weeks with corticosteroids and 4-9 weeks without it.
The rarity of the syndrome with yet unidentified etiology and not a single case documentation in Indian children prompted us to report this case.
We thank the Department of Pathology, J.N. Medical College, Belgaum for histopathological study and Dr. V.D. Patil, Head of Department of Pediatrics and Dr. Mokashi, District Surgeon, for their encouragement.
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