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
Sweet syndrome treatment ladder
FIRST LINE
Systemic CS prednisolone 0.5-1mg/kg/day for 4-6 weeks
- adjuvant bone protection i.e. Vit D, calcium
- if remission has not been induced after 3 months —> add steroid sparing agent
Potent TCS (mild localised disease)
ILCS (mild localised disease)
SECOND LINE —> no evidence base as to which should be tried first
Dapsone 50-100mg/day
- close monitoring of FBC for haemolysis
- cautious approach with 50mg taking time to be effective but not triggering haemolysis
- haem S/E frequent with 100mg or more
Potassium iodide 300mg TDS
Colchicine 0.5mg TDS
- GI S/Es dose related i.e. nausea, diarrhoea, vomiting
THIRD LINE
Ciclosporin
Clofazimine
IVIg
Indomethacin (NSAID) Cyclophosphamide (cytotoxic) Chlorambucil (cytotoxic) Anti-TNF Thalidomide (beware child bearing F) Interferon-alpha
Pyoderma gangrenosum subtypes
Classical ulcerative form
Parastomal
Pustular
Bullous (Atypical)
Granulomatous superficial
Neutrophilic dermatosis of the dorsal hands (see under sweet syndrome variant)
Extracutaneous PG
Classical PG Diagnostic criteria
MAJOR CRITERIA
Rapid progression
of a painful necrolytic skin ulcer
with an irregular violaceous and undermined border
Exclusion of other causes of cutaneous ulceration
MINOR CRITERIA (2 should be met) A hx suggestive of pathergy, or the clinical finding of cribroform scarring
Systemic disease known to be assoc with PG
Histopathological findings
- Sterile dermal neutrophilia +/- mixed inflammation +
- Lymphocytic vasculitis (leukocytoclasia, endothelial swelling, no fibrinoid necrosis)
Rx response
- Rapid response to systemic CS
PG associated diseases
IBD (especially in the pustular variant of PG)
- Crohn disease
- Ulcerative colitis
RA
Other seronegative arthritis
Haem malignancy (especially in the bullous form of PG) Monoclonal gammopathy
Other visceral malignancies
DISEASE ENTITIES WITH PG PAPA syndrome - Pyogenic arthritis - PG - Acne
PASH syndrome (lacks genetic abnormalities and arthritis of PAPA syndrome)
- PG
- Cystic Acne
- Hidradenitis
PG CO-MORBIDITIES (either predispose to PG or a result from PG) Diabetes (long term pred) Peripheral vascular disease Obesity Depression Anaemia Renal impairment Thyroid disease
PG predisposing factors
Pathergy (skin trauma provokes lesions, or first onset of dosease is at the site of injury)
- following surgical wound
- following penetrating injury
In ulcerative colitis patients with PG
- Smoking
- Appendicectomy
PG pathogenesis
MULTIFACTORIAL Prediposition to inflammatory cascade, including - Activation of innate immunity - Autoinflammtory pathways - Cytokines
If drug-induced esp thiouracils —> may be C-ANCA or P-ANCA positive
GENETICS
PAPA syndrome —> PSTPIP1/CD2BP1 mutation
Classic ulcerative PG (PG subtype)
Commonest, best recognised variant
Small tender red blue papules, plaques, pustules
Evolve into painful ulcers
Characteristic violaceous undermined edge
Ulcer base —> there may be granulation tissue, necrosis, purulent exudate
Solitary or multiple ulcers
Most commonly on the legs, may affect any body site i.e. genitals, mucosae
Healing —> occurs with an atrophic cribriform scar
Associated symptoms
- Fever
- Malaise
- Myalgia
- Arthralgia
Parastomal PG (PG subtype)
May arise as pathergy response to
- Trauma of appliances
- Faecal irritation
Most common with ileostomy for active IBD
Risk factors
- Higher BMI
- F
- Autoimmune disorders
Pustular PG (PG subtype)
Assoc with IBD —> Often occurs during acute exacerbations of IBD
Discrete painful pustules with surrounding halo of erythema develop on normal skin
Commonly arise scattered on the extensor aspects of the limbs
DDX OF OTHER PAINFUL PUSTULAR ERUPTIONS IN THE CONTEXT OF IBD
BADAS (Bowel-associated dermatitis-arthritis syndrome)
Subcorneal pustular dermatosis
Pyostomatitis vegetans (predominanly mucosal pustules)
Bullous PG (Atypical PG, PG subtype)
Assoc with myeloproliferative disorders
Rapidly arising
Superficial haemorrhagic bullae
Often located on the arms
Shares clinical and histological findings with Sweet syndrome but
Typically ulcerates
Heals with scarring
ASSOCIATED DISEASES
Myeloproliferative disorders —> PG can precede the onset of malignancy —> should be investigated with high index of suspicion for haem malignancy
Granulomatous superficial PG (vegetative PG, PG subtype)
Superficial lesions of PG
With a granulomatous histology
Begins with single superficial ulcer with granulations and elevated edge
Most often on trunk
Lower incidence of associated conditions (idiopathic)
More responsive to Rx than other variants
- TCS
- ILCS
- systemic CS
- minocycline
- Dapsone
Extracutaneous PG (PG subtype)
Aseptic abscessus
Lungs (most common) Bones Liver Heart Brain GIT Muscle
Non-classical PG DDx
Vascular occlusive or venous disease
Vasculitis
Malignancy
Primary infection —> Bx and tissue culture
- Fungi
- atypical mycobacteria
- Sporotrichosis
- Mucormycosis
- Histoplasmosis
- Blastomycosis
Drug-induced
- Nicorandil
Exogenous tissue injury
PG Complications
Mortality ? from secondary infection, immunosuppressive Rx, or combo
PG COMPLICATIONS/CO-MORBIDITIES (either predispose to PG or a result from PG) Diabetes (long term pred) Peripheral vascular disease Obesity Depression Anaemia Renal impairment Thyroid disease
PG course and prognosis
Chronic condition
Takes many months or years to completely resolve
May be recurrent —> consider long term systemic preventive Rx
PG investigations
EXAM
Lymphadenopathy (malignancy)
SKIN BX
Not diagnostic, but performed in all (except classical Ulcerative PG) to exclude other conditions that may minic PG particularly infection
Bx for H&E, and
Bx for tissue culture (bacterial, mycobacterial, fungal, viral)
BLOODS
FBC (haem malignancy)
ELFT
Blood culture (infection)
IMAGING
CXR (TB, malignancy)
CT scan (malignancy)
OTHER
Endoscopy (IBD, malignancy)
BMAT (malignancy)
PG histology
No typical histology
Skin bx taken to exclude other causes of ulceration esp infection that mimic PG clinically
Neutrophilic pustules (early lesions)
Features generally non-specific
Presence of vasculitis debatable —> may be secondary to ulceration
If vasculitis is present —> exclude vasculitides and infective causes —> tissue culture, special stains of tissue for fungi, mycobcteria
TYPICAL FINDINGS
Central necrosis
Ulceration of the epidermis + dermis
Surrounded by intense mixed to chronic inflammatory cell infiltrate
CLASSIC ULCERATIVE SUBTYPE
Massive dermal epidermal neutrophilic infiltrate with suppurative/abscess formation
PUSTULAR SUBTYPE
Perifollicular neutrophilic infiltrate with subcorneal pustule formation
BULLOUS SUBTYPE
Neutrophilic infiltrate with intraepidermal vesicle formation
SUPERFICIAL GRANULOMATOUS (VEGETATIVE) SUBTYPE Granulomatous reaction with peripheral palisading histiocytes and giant cells
PG management
FIRST LINE
Analgesia
Supportive Rx to promote wound healing
- Dressings
- Topicals for cleansing and debriding and keeping wound moist
- Compression
Potent TCS (small lesions)
ILCS (small lesions)
Topical tacrolimus ointment (small lesions)
SECOND LINE (more severe dosease, or PG not responding to simple measures)
- Systemic CS (mainstay Rx)
- Oral prednisolone 0.5 - 1mg/kg/day
- CSA 5mg/kg
- Combined oral prednisolone + CSA
Pulsed oral prednisolone 1mg/kg/day for 5 days
MTX
MMF
Dapsone
Others
- Tetracyclines
- Thalidomide
- Tacrolimus
- Cyclophosphamide
- Chlorambucil
- AZA
- Colchicine
THIRD LINE mainly d/t cost (Biological Rx)
Anti-TNF (should be comsidered more often as 1st line esp those with IBD)
- Infliximab**
- Adalimumab —> risk of paradoxical onset of PG
- Etanercept —> risk of paradoxical onset of PG
IL12/23
- Ustekinumab
Anti- IL1
- Anakinra (successful for PAPA, PASH syndrome)
IVIg
Topical platelet derived growth factor
ALTERNATIVE RX Skin graft (although surgery should be avoided in the inflammatory stage and can grequently induce PG, skin graft can be successful if inflammation has already resolved following systemic Rx with oral CS)
Granulocyte apheresis
Topical sodium cromoglycate
Topical nicotine
Hyperbaric oxygen
BADAS (Bowel-associated dermatitis-arthritis syndrome)
DEFINITION
Pustular vasculitic lesions
Assoc with blind loops of bowel
Or other causes of stasis of bowel content
CLINICAL FEATURES
Begins as small macular lesions
Progress into papules —> pustules on an erythematous purpuric base
Arms, other areas on upper body
Pustules typically occur in crops
Each crop lasts ~ 2 weeks
Recurring at intervals of several months
Other lesion types
- larger erythema nodosum-like lesions
- larger PG-like pustular lesions
Pathergy
Lesions May be preceded by constitutional symptoms -
- Fever
- Flu-like symptoms
- Myalgia
- GIT upset
Arthralgia or non-erosive polyarthritis —> acute inflammatory with tenderness, swelling
- Hands
- Wrists
- Other peripheral joints
Ocular
- Episcleritis
GUT
- Haematuria
- Proteinuria
DDX Behcet disease (oral aphthae, lesions of pustular vasculitis)
PATHOGENESIS
Proliferation of bowel flora antigen (peptidoglycans) —> circulating immune complexes —> immune complex-mediated blood vessel damage
PREDISPOSING FACTORS causing blind loops of bowel or stasis of bowel contents Jejuno-ileal bypass surgery Gastric resection Blind loops of bowel Defunctioning ileo-anal pouch Bilio-pancreatic diversion
IBD
- Crohn disease
- Ulcerative colitis
Diverticulitis of the colon
Jejunal diverticula
Appendicitis
Achalasia of the gastric cardia
Combined causes i.e. surgery for IBD
DX
Requires clinicopathologic correlation
IX
Swab MCS of pustules
Urinalysis (haematuria, proteinuria —> immune complex initiated glomerulonephritis)
HISTO
Pustular vasculitis —> similar to Sweet syndrome
MX
Surgical correction of bowel anatomy (for patients following bowel bypass surgery) —> often eliminates signs and symptoms
Surgical correction difficult for patients with blind loops of bowel —> resolution of sumptoms less likely
SYSTEMIC RX (first line —> antibacterials —> suggests role of bacterial colonisation in trigerring systemic response in BADAS) Oral tetracycline Oral metronidazole Oral ciprofloxacin Oral erythromycin
SYSTEMIC RX (second line) —> may need to be combined with appropriate AB
Oral prednisolone
- usually unnecessary
- but may be justified depending on degree of skin, joint, systemic symptoms, or manage IBD)
Oral colchicine
Oral dapsone
Oral MMF
Subcorneal pustular dermatosis @ Sneddon-Wilkinson disease
DEFINITION
Sterile subcorneal pustules
Affecting flexural areas of trunk and proximal extremities
CLINICAL FEATURES
Sterile oval pea-sized pustules on a normal or erythematous base
May see characteristic fluid level
- Pus lower half
- Clear fluid upper half
Pustules may be isolated or grouped, coalescing to form annular or serpiginous patterns
Successive waves may pass ofer the same area
Pustules may rupture in the flexures —> becomes indistinct
Acute flares lasts several days or weeks
Flexures of the trunk, proximal limbs (axillae, groin, submammary, neck, apron)
Spares face, mucous membrane
ASSOCIATED DISEASES Benign monoclonal gammopathy (more commonly IgA) Multiple myeloma Lymphomas IBD PG RA Connective tissue disease incl. SLE
PATHOGENESIS Obscure Linked to - excessive production of TNF-alpha - Neutrophil activation —> in common with other neutrophilic dermatosis
? Overlap with pemphigus (but most cases DIF megative)
—> a subset has IgA deposited in the upper dermis directed against desmocollin 1 —> IgA pemphigus —> relationship between IgA pemphigus and classic subcorneal pustular dermatosis unclear
DDX
Impetigo
Pustular psoriasis (spongiform pustules, unlike subcorneal pustular dermatosis)
AGEP (fevers, recent drug exposure)
Pemphigus foliaceus
IgA Pemphigus (DIF intercellular IgA)
Dermatitis herpetiformis (this is usually on extensor surfaces, rather than flexures)
IX
Skin swab MCS (exclude impetigo)
Skin biopsy of early pustules (most recent pustules)
Skin bx of perilesional skin for DIF (DDx IgA pemphigus)
Anti-skin antibodies (Pemphigus)
FBC + diff, SPEP, UPEP + immunofixation, serum free light chains (Exclude multiple myeloma)
Rh factor, anti-CCP (Exclude RA) —> only if indicated by hx
ANA (exclude autoimmunity) —> only if indicated by hx
Faecal calprotectin (exclude IBD) —> only if indicated by hx
HISTO
Perivascular inflammatory infiltrate with occasional EOSINOPHILS
Pustules sit on the surface of the viable epidermois
No spongiosis
No spongiotic pustules
IHC neg (positive in intercellular IgA pemphigus)
MX
Rx underlying associated disease
Rx condition
FIRST LINE RX
Dapsone 50-150mg daily —> partial or complete response
SECOND LINE RX Potent TCS —> not really effective Oral CS —> not really effective Acitretin Tacalcitol NbUVB PUVA Oral retinoids + PUVA CSA + oral pred
THIRD LINE RX
Oral ketoconazole
Anti-TNF (case reports)
- Etanercept
- Adalimumab (combined with other immunosuppressant i.e. MMF)
- Infliximab (combined with other immunosuppressants i.e. pred, AZA)
COURSE/PROGNOSIS
Benign but chronic
Pyodermatitis-Pyostomatitis vegetans @ Pyoderma vegetans
Some consider this the oral mucosal form of pustular PG
CLINICAL FEATURES
Oral mucosal thickening with multiple pustules, Snail track superficial ulceration on an erythematous base
Any site of oral mucosa
But tongue less affected
Skin lesions may be present
- often flexural
- Clinically suggestive of pemphigus vegetans with verrucous plaques studded with pustules
- may be assoc dorsal hand lesions morphologically similar to neutrophilic dermatosis and rash resembling Sweet syndrome
- May display pathergy
ASSOCIATED DISEASES IBD esp ulcerative colitis (strongest assoc) Leukaemias Lymphomas Follicular occlusion syndromes - Acne conglobata - HS - Dissecting cellulitis Immunosuppression Malnutrition
PATHOGENESIS
Unknown
Suggested immunological and microbial factors
May represent abnormal response to infection (various bcteria reported as causative)
IX
Total IgE (raised)
FBC (peripheral eosinophilia)
HISTO ORAL MUCOSAL LESIONS
Intraepithelial and/or subepithelial abscesses containing large numbers of EOSINOPHILS
Deeper tissue have mixed infiltrate of neuts, eos
Often assoc peripheral blood EOSINOPHILIA
HISTO SKIN LESIONS (IF PRESENT) —> verrucous plaques studded with pustules Pseudoepitheliomatous hyperplasia Neutrophilic abscesses Negative DIF —> unlike pemphigus vegetans
MX
Antimicrobials (if cause uncertain)
RX FOR ORAL DISEASE First line - TCS - TCI (tacrolimus) - Oral pred when topicals fail
Second line
- Dapsone
- Oral pred +/- AZA
- CSA
- Adalimumab
- MTX + infliximab (Crohn assoc cases)
Amicrobial pustulosis of the skin folds
Most cases occur in patients with SLE
CLINICAL FEATURES
Rapidly evolving small semi-confluent pustules
Pustules —> erosions, crusts
Predominatly affect flexures —> 1 major (axilla, inguinal) + 1 minor skin fold (face, scalp) affected
Alopecia if affects scalp
ASSOCIATED DISEASES (mainly autoimmune conditions) SLE (most cases) Coeliac disease Autoimmune thyroid disease Autoimmune hepatic disease Crohn disease Sjogren disease IgA nephropathy
DIAGNOSTIC CRITERIA
Major criteria
- Pustulosis involving 1 or more major skin fold
- Pustulosis involving 1mor more minor skin folds in the anogenital area
- Histo pattern with intraepidermal spongiform pustules + mainly neutrophilic dermal infiltrate
- Negative culture from unopened non-ruptured pustule
Minor criteria
- Assoc with 1or more autoimmune disorders
- Positive ANA 1:160 or higher
- Autoantibodies ENA, anti-dsDNA, anti-smooth muscle, anti-mitochondrial, antiendomesial
DDX
Subcorneal pustular dermatosis
Pustular psoriasis
Erosive pustular dermatosis of the scalp (if involves scalp)
IX
Neutrophilic epidermal and ostial exocytosis
Spongiform pustules
Neutrophilic dermal infiltrate
Variable DIF positivity for IgM, C3, IgG (endothelium or lupus band)
FIRST LINE RX
Oral pred
SECOND LINE RX Dapsone CSA Oral zinc TCS Colchicine Cimetidine Ascorbic acid Anakinra (if IL-1 demonstrated to be involved)
