Sweet Syndrome Flashcards
Compare the classic clinical presentation with other sweet variants
Classic : Pink/ erythymatous edematous papules and plaque, might enlarge and coalesce. surface might appear mamillated/ pseudopustular/ pseudovesicular due to edema.
Other possible features
* vesicles
* pustules
* vesiculobullous variant, which is most frequently associated with acute myelogenous
Leukemia, can progress to ulceration resembling superficial pyoderma gangrenosum (PG). In this form, there may be a single lesion or multiple, asymmetrically distributed lesions.
* ulcers - might appear like superficial PG.
- targetoid appearance with central yellowish discoloration.
- A facial erysipelas-like appearance.
- giant cellulitis-like variant.
- Papulonodules involving the lower legs may resemble erythema nodosum.When isolated, the latter is often referred to as subcutaneous Sweet syndrome.
Compare the classic clinical presentation with other sweet variants
TENDER NON -pruritic Pink/ erythymatous edematous papules and plaque, might enlarge and coalesce to form irregularly shaped plaques.surface might appear mamillated/ pseudopustular/ pseudovesicular due to edema.
Other possible features
* vesicles
* pustules
* vesiculobullous variant, which is most frequently associated with acute myelogenous
Leukemia, can progress to ulceration resembling superficial pyoderma gangrenosum (PG). In this form, there may be a single lesion or multiple, asymmetrically distributed lesions.
* ulcers - might appear like superficial PG.
- targetoid appearance with central yellowish discoloration.
- A facial erysipelas-like appearance.
- giant cellulitis-like variant.
How to diagnose sweet ?
Both of the major and two minor criteria ( out of 4) are needed for the diagnosis.
Major :
1) Abrupt onset of typical cutaneous lesions
2) histopathology consistent with Sweet syndrome
Minor :
- Preceded by one of the associated infections or vaccinations;
accompanied by one of the associated malignancies or inflammatory
disorders; associated with drug exposure or pregnancy - Presence of fever and constitutional signs and symptoms
- Leukocytosis
- Excellent response to systemic corticosteroids
Describe distribution pattern of sweet
The cutaneous eruption of Sweet syndrome favors the head, neck and upper extremities (including the dorsal aspect of the hands),
but can occur anywhere.
In true malignancy-associated cases, the lesions tend to have a more widespread distribution.
Are Oral lesions common ?
No, they are uncommon.
except in patients with hematologic disorders; initially they appear pseudopustular, and later ulcerate and appear as aphthae.
What happens if sweet lesions are left untreated ?
The cutaneous eruption of Sweet syndrome usually resolves spontaneously within 5 - 12 weeks but recurs in up to 30% of patients.
What happens if sweet lesions are left untreated ?
The cutaneous eruption of Sweet syndrome usually resolves spontaneously within 5 - 12 weeks but recurs in up to 30% of patients.
What is a common prodrome in Sweet ?
An upper respiratory tract infection or flu-like illness frequently
precedes the development of the syndrome.
Fever occurs in 40-80% of patients and can be intermittent.
Extracutaneous involvement is also frequently seen (Fig. 26.4 ; Table 26.3).
SYSTEMIC MANIFESTATIONS OF SWEET SYNDROME
Common and less common
SYSTEMIC MANIFESTATIONS OF SWEET SYNDROME
Common (> 50%)
Fever
Leukocytosis
Less common (20-50%)
Arthralgias
Arthritis: asymmetric, non-erosive, sterile, favors knees and wrists
Myalgias
Ocular involvement: conjunctivitis, episcleritis, limbal nodules, iridocyclitis
SYSTEMIC MANIFESTATIONS OF SWEET SYNDROME
Uncommon
Unusual / rare
Uncommon
Neutrophilic alveolitis: cough, dyspnea and pleurisy; radiographic findings
include interstitial infiltrates, nodules, pleural effusions
Multifocal sterile osteomyelitis, a subtype of SAPHO (see Table 26.18)
Renal involvement (e.g. mesangial glomerulonephritis): hematuria, proteinuria, renal insufficiency, acute renal failure
‐——————–‐————-‐———————————–
Unusual/rare
Acute myositis
Hepatitis, pancreatitis, ileitis, colitis
Aseptic meningitis, encephalitis, bilateral sensorineural hearing loss
Other: aortitis, oral aphthae, pharyngitis, pharyngeal edema
Workup of Sweet
CBC -look for leukocytosis and neutrophilia.
In hematologic malignancy or myelodysplasia, there may be a high or low white cell count, lymphocytosis or lymphopenia, and thrombocytosis or thrombocytopenia.
CRP , ESR - many times elevated
hepatic function test
Chemistry : look for renal function (possible renal involvement ), hepatic function tests, electrolytes
Hepatitis B C serology
CMV serology
HIV serology
Bacteriologic examinations and repeated cultures of wound swabs.
ANA ( due to collagen disease association )
Serum electrophoresis with immunofixation .
Urine light chains - Bence Jones
workup for an underlying solid tumor or lymphoproliferative disorder.
elevated serum levels of antineutrophil cytoplasmic antibody (ANCA) have been reported14, in particular pANCA or an atypical ANCA, there is no strong evidence that it is a serologic marker for this disease.
Biopsy : marked edema in the upper dermis Neutrophilic infiltrate Fragmented nuclear “dust” of neutrophils (leukocytoclasia) in “top-heavy” fashion may be present No LCV
Skin biopsy for tissue culture
Periodic acid–Schiff and Ziehl-Neelsen stains for fungi and mycobacteria.
In bullous lesions include DIF. ( should be neg in sweet ).
SWEET SYNDROME - ASSOCIATED DISORDERS AND TRIGGERS
Infections
———-‐—–
Viruses: upper respiratory tract infections, cytomegalovirus, hepatitis B
virus, hepatitis C virus, HIV
Bacteria: Yersenia, streptococci
Mycobacteria: atypical mycobacteria, BCG vaccination, M. Tuberculosis, M.leprae
Fungi: dimorphic, including sporotrichosis and coccidiomycosis
Malignancies
—–‐—————-
Hematologic (10-20% ofcases*), in particular acute myelogenous leukemia
Myelodysplasia
Solid organ, predominantly the more common primary carcinomas
————–‐————-‐————–
Gastrointestinal disorders
—‐————-‐———————–
. Inflammatory bowel disease: Crohn , ulcerative colitis.
Drugs
‐———-
Antibiotics (e.g. minocycline, trimethoprim-sulfamethoxazole),
antihypertensives (e.g. furosemide, hydralazine), antineoplastics (e.g.
ipilimumab, pembrolizumab, FLT3 inhibitors, vemurafenib), colony
stimulating factors (e.g. G-CSF), contraceptives, immunosuppressant
medications (e.g. azathioprine), NSAIDs, retinoids (e.g. all-frans-retinoic
acid), bortezomid, lenalidomide
—‐————-‐———————–
Autoimmune disorders
—‐————-‐———————–
Autoimmune connective tissue diseases (e.g. systemic lupus
erythematosus SLE, rheumatoid arthritis, dermatomyositis, relapsing
polychondritis, Sjogren syndrome), autoimmune thyroid disease
Sarcoidosis
Behcet disease
SWEET SYNDROME - ASSOCIATED DISORDERS AND TRIGGERS
Infections
———-‐—–
Viruses: upper respiratory tract infections, cytomegalovirus, hepatitis B
virus, hepatitis C virus, HIV
Bacteria: Yersenia, streptococci
Mycobacteria: atypical mycobacteria, BCG vaccination, M. Tuberculosis, M.leprae
Fungi: dimorphic, including sporotrichosis and coccidiomycosis
Malignancies
—–‐—————-
Hematologic (10-20% ofcases*), in particular acute myelogenous leukemia
Myelodysplasia
Solid organ, predominantly the more common primary carcinomas
————–‐————-‐————–
Gastrointestinal disorders
—‐————-‐———————–
. Inflammatory bowel disease: Crohn , ulcerative colitis.
Drugs
‐———-
Antibiotics (e.g. minocycline, trimethoprim-sulfamethoxazole),
antihypertensives (e.g. furosemide, hydralazine), antineoplastics (e.g.
ipilimumab, pembrolizumab, FLT3 inhibitors, vemurafenib), colony
stimulating factors (e.g. G-CSF), contraceptives, immunosuppressant
medications (e.g. azathioprine), NSAIDs, retinoids (e.g. all-frans-retinoic
acid), bortezomid, lenalidomide
—‐————-‐———————–
Autoimmune disorders
—‐————-‐———————–
Autoimmune connective tissue diseases (e.g. systemic lupus
erythematosus SLE, rheumatoid arthritis, dermatomyositis, relapsing
polychondritis, Sjogren syndrome), autoimmune thyroid disease
Sarcoidosis
Behcet disease
characteristic histologic presentation of Sweet :
The characteristic histologic presentation is a diffuse nodular and perivascular neutrophilic infiltrate without evidence of vasculitis (Fig. 26.5), although occasionally leukocytoclastic vasculitis (LCV) can be observed. Leukocytoclasia with endothelial swelling, but without the fibrinoid necrosis that fulfills the criteria for LCV, is the usual finding.
What are other histologic variants in sweet syndrome ?
Three histologic variants of Sweet syndrome
1) histiocytoid,
2) lymphocytic and
3) eosinophilic
The histiocytoid variant is characterized by a dermal, and sometimes subcutaneous, infiltrate composed of histiocyte-like immature myeloid cells. These cells have myeloperoxidase activity (Fig. 26.6 t© ) and must be distinguished from those of leukemia cutis.
The lymphocytic variant is often associated with, and sometimes precedes,
underlying myelodysplasia. More recently, an eosinophilic variant has been described.
