Neutrophilic Dermatoses Flashcards
Sweet syndrome 3 subtypes
@ Acute febrile neutrophilic dermatoses
Classical
Malignancy associated
Drug induced
Sweet syndrome associated diseases
INFECTIONS (classical)
Upper resp tract infections esp Streptococcal
GI infections i.e. salmonella, Yersinia
Mycobacterial infections
IBD (classical)
Ulcerative colitis
Crohns disease
ENDOCRINE
Pregnancy
Autoimmune thyroid disease
IMMUNOLOGICAL
Lupus erythematosus
Sjogren syndrome
Collagen vascular disorders
OTHER INFLAMMATORY CONDITIONS *Sarcoidosis *RA Still’s disease SAPHO
OTHER NEUTROPHILIC DERMATOSES —> concurrently or sequentially PG Neutrophilic dermatosis of the dorsal hands Behcet’s disease Erythema elevatum diutinum Erythema nodosum Relapsing polychondritis Neutrophilic eccrine hidradenitis Subcorneal pustular dermatoses Various vasculitis
HAEM MALIGNANCIES (malignancy associated) —> majority have malignancy prior to Sweet syndrome *AML *Promyelocytic leukaemia *CML *Multiple myeloma *Myelodysplastic syndrome Polycythaemia Aplastic anaemia Fanconi’s anaemia Monoclonal gammopathy Lymphomas (less common)
SOLID ORGAN MALIGNANCIES (malignancy associated) —> majority have Sweet syndrome prior to occurence of malignancy, but malignancy typically Dx within 12 months Breast Larynx GIT GUT Prostate
MEDICATIONS (drug induced) G-CSF Chemotherapy Imatinib (KIT inhibitor) Neutrophil maturation drugs Retinoids Antibiotics Antiepileptics HAART (highly active antiretroviral therapy) Anti-HTN Diuretics NSAIDS Contraceptives Propylthiouracil
Classical Sweet syndrome clinical features
Hx —> any prior hx of fevers or infectious illness
Most have fevers and history of infection
Tender red papules, nodules, plaques
Head, neck, upper trunk, upper arms
Often oedematous —> May become studded with pseudovesicles or pseudopustules (due to the odema)
Subsequently definite vesicles and pustules —> ulceration
Arthralgia
Conjunctivitis, episcleritis
Oral, genital mucosa ulceration uncommon
Systemic (extracutaneous) manifestations
- Bone
- Muscle
- Tendons
- Neuro-Sweet’s (CNS) —> benign encephalitis, aseptic meningitis, brainstem lesions, psych symptoms
- Heart
- lung —> neutrophilic inflammation of bronchi, aseptic pulmonary effusion with abundant neuts (SOB)
- Liver
- Intestine
- Spleen
- Kidney
- Subcutaneous (Sweet’s panniculitis)
COURSE
Spontaneous resolution may occur often within 3 months
1/3 untreated cases —> pattern of fluctuating exacerbations and recurrence
COMPLICATIONS
Resolves with no sequelae (majority)
Severe skin lesions with ulceration or delayed Rx —> scarring
UNUSUAL CONSEQUENCES
*Acquired cutis laxa
Mid-dermal elastolysis
Elastophagocytosis
Malignancy-associated Sweet syndrome clinical features
Hx —> current or previous malignant disease
More widespread distribution
Skin lesions more likely to be
- Vesicular
- Bullous
- Ulcerative
Oral lesions/ulceration
Frequent assoc with
- Abnormal platelet counts
- Anaemia
Haem malignancies —> occur before onset Sweet syndrome
Solid tumour malignancies —> Sweet syndrome mostly presented prior to malignancy, but Dx within 12 months of omset of Sweet syndrome
Drug-induced Sweet syndrome clinical features
Hx —> new drug administration
2 main types
- GCSF-associated (more common)
- Other drugs
Should be a close relationship between drug ingestion and clinical symptoms
3 Clinical variants
Neutrophilic dermatosis of the dorsal hands
Subcutaneous Sweet syndrome
Histiocytoid Sweet syndrome
Neutrophilic dermatosis of the dorsal hands (clinical variant)
Identical clinical lesions
Identical histo
Identical demographics
Identical disease associations
Bluish, haemorrhagic papules, bullae, nodules
On the dorsal hands
Typical Sweet syndrome lesions seen at other sites in 50%
Reaponds promptly to
- prednisolone OR
- dapsone
DDX
Bullous PG
Pustular vasculitis
ASSOCIATED DISEASES —> may have greater assoc with malignancy Myeloproloferative disorders Occult malignancy IBD RA
Subcutaneous Sweet syndrome (clinical variant)
Erythema nodosum-like
Tender, subepidermal nodules
Extremities, usually legs
Assoc with Haem malignancy —> atypical skin lesions with histology of Sweet syndrome
Histiocytoid Sweet syndrome (clinical variant)
HISTO
Infiltrate composed of large histiocytoid mononuclear cells (look like histiocytes)
IHC FOR MYELOPEROXIDASE
Positive —> suggests immature myeloid cells and neut precursors
SKIN LESIONS
May resemble classical Sweet syndrome or with subcutaneous erythema nodosum type lesions
ASSOCIATIONS
Classical/idiopathic subtype
Haem malignancy assoc
Drug induced
Sweet syndrome Diagnostic criteria
MAJOR
Acute onset of typical lesions
Histo findings consistent with Sweets syndrome
MINOR
Fever > 38
Assoc with
- Malignancy
- Inflammatory disorder
- Pregnancy
- Antecedent respiratory or GI infection
Excellent respinse to systemic CS or potassium iodide
Abnormal lab values at presentation (3 of 4 required)
- ESR < 20mm
- Elevated CRP
- Leukocytes > 8000
- Neuts > 70%
Sweet syndrome Clinical DDx
INFECTIOUS DISORDERS
Erysipelas
Cellulitis
HSV (vesicular)
INFLAMMATORY Panniculitides PG Syphilis TB
NEOPLASTIC
Mets
REACTIVE ERYTHEMAS
Erythema nodosum (EN)
Erythema multiforme (EM)
Urticarial
SYSTEMIC DISEASE
Behcet disease
Lupus
Bowel bypass syndrome
VASCULITIS
Erythema elevatum diutinum (EED)
Polyarteritis nodosa (PAN)
Granuloma faciale (if on the face)
Sweet syndrome Histo DDx
Leukaemia cutis
Leukocytoclastic vasculitis
Neutrophilic eccrine hidradenitis
Sweet syndrome Investigations
FULL HX
MEDICAL EXAM —> malignancy screen Lymph nodes Breasts (women) Pelvis (women) Prostate (men) Testicles (men)
SKIN BX
ROUTINE BLOODS FBC (leukocytosis, neutrophilia, haem malignancy) LFT (systemic manifestation) U/E (systemic manifestation) CRP (raised) ESR (raised)
ADDITIONAL BLOODS (as guided by clinical findings) ASOT (strep infection) TFT (autoimmune thyroid disease) Rh factor (RA)
OTHER
Sigmoidoscopy (in age > 50 yrs) —> malignancy screen
Futher Ix to exclude malignancy may be considered if no apparent cause
Sweet syndrome Pathogenesis
External or internal trigger causing alterations in cell signalling and cytokine production
Leads to increase in neut production
Leads to migration into skin and occasionally other tissues
Elevated levels of GCSF
Pathergy (disease triggered at a site following minimal trauma)
Sweet syndrome Histo
Prominent dermal oedema
+/- subepidermal vesicles
Dense infiltrate of neuts in the dermis
+/- lymphocytes, eso, histiocytes
Leukocytoclasis
Endothelial swelling without fibrinoid necrosis
Genuine vasculitis not seen
Cell infiltrate Usually diffuse
Perivascular and dermal band patterns seen
Overlying epidermis usually normal
Exocytosis of neuts may be seen
Spread of neuts to subcut fat possible
