Neutrophilic dermatosis

by : Dr. Gamal Soltan

3. A heterogeneous but linked spectrum
diseases, with significant overlapping
histopathologicfindings and similar
pathogenicmechanisms and
therapeuticapproaches. often
associated with underlying internal
diseases, which may have significant
morbidity and mortality
DR. GAMAL SOLTAN

4. Histologically, these disorders
characterized by perivascularand
diffuse neutrophilicinfiltrates
without any identifiable
infectious agents.
The cutaneous manifestations
vary from vesiculo-pustules and
plaques to nodules and ulcers.
DR. GAMAL SOLTAN

5. The neutrophilis a terminally differentiated,
non-dividing cell which is packed with
granules whose contents kill and degrade
target micro-organisms
Neutrophilsare produced in the bone marrow
at a rate to supply baseline requirements for
sufficient numbers of circulating cells.
Mature neutrophilscirculate in the peripheral
bloodstream for only 3–12 hours before
migrating into tissues,andsurvive for 2–3days.
DR. GAMAL SOLTAN

6. DR. GAMAL SOLTAN

7. SWEET’S SYNDROME √
PYODERMA GANGRENOSUM √
BEHCET’S DISEASE √
BOWEL-ASSOCIATED DERMATOSIS –
ARTHRITIS SYNDROME
SYNOVITIS, ACNE, PUSTULOSIS,
HYPEROSTOSIS AND OSTEITIS (SAPHO)
SYNDROME
DR. GAMAL SOLTAN

8. Synonym:
Acute febrile neutrophilicdermatosis
An uncommon, acute and recurrent,
cytokine-induced skin reaction
associated with various etiologies.
DR. GAMAL SOLTAN

9. Age of Onset: Most 30–60 years.
Sex: Women > men.
Etiology:
•Unknown, possibly hypersensitivity reaction.
Inflammatory bowel disease
Associated Disorders :
•Febrile upper respiratorytract infection. In some
cases associated with Yersiniainfection.
•Hematologic malignancy; drugs
DR. GAMAL SOLTAN

10. The original theories included
and
, but
have not been supported by
experimental data.
One current hypothesis is a local or
systemic
secretion.
DR. GAMAL SOLTAN

11. The initial cutaneous
lesions are ,
,
plaques or papules,
which may enlarge or
coalesce to form plaques
with mammillated
surface. Because of the
pronounced associated
edema
DR. GAMAL SOLTAN

12. The cutaneous eruption of
Sweet’s syndrome favors
the head, neck and upper
extremities, but can occur
anywhere.
In true malignancy-
associated cases, the lesions
have a more widespread.
Papulonodulesinvolving
lower legs may resemble
erythemanodosum, a small
number of patients have
concurrent erythema
nodosum
DR. GAMAL SOLTAN

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16. Marked oedemain papillary
dermis
Neutrophilicinfiltrate:
◦Reticular dermis
◦Diffuse, dense
◦Nuclear dust (leukocytoclasis)
No vasculitis
Epidermis
◦Normal; or mild spongiosis
◦Blistering in clinically vesicular
lesions
DR. GAMAL SOLTAN

17. 1. Abrupt onset of
2. consistent with Sweet’s syndrome
1. one of the associated infections or
vaccinations; accompanied by one of the associated
malignancies or inflammatory disorders; associated with
drug exposure or pregnancy
2. Presence ofand constitutional signs and symptoms
3.
4. Excellent
DR. GAMAL SOLTAN

18. Sweet’s syndrome is a benign condition
which, if left untreated, may persist for
weeks or months.
Cutaneous lesions then involute
spontaneously, leaving no scars However,
recurrences occur in approximately 30%
of patients (with or without treatment)
DR. GAMAL SOLTAN

19. The most effective therapy is oral prednisone (0.5–1.0
mg/kg/day) for 4–6 weeks. When the lesions are few
and localized, topical superpotentor intralesional
corticosteroids and/or topical calcineurininhibitors
may be helpful.
The major alternative drugs are potassium iodide (900
mg/day), dapsone(100–200 mg/day) and colchicine
(1.5 mg/day).
They can also be used as corticosteroid-sparing agents.
Non-steroidal anti-inflammatory drugs, clofazimine,
cyclosporine, thalidomide and interferon-ɤ have also
been lead to improvement
DR. GAMAL SOLTAN

20. DR. GAMAL SOLTAN

21. An uncommon, chronic, recurrent
cutaneous ulcerative disease with a
distinctive morphologic presentation.
The laboratory and histopathologic
findings can vary and therefore the
diagnosis requires clinico-pathologic
correlation.
DR. GAMAL SOLTAN

22. Age: any age but most commonly afflicts
women between 20 and 50 years of age.
50% of patients have an underlying systemic
disease, most commonly inflammatory bowel
disease, arthritis or myelo-proliferative
disorders.
4% of cases of PG occur in infants and
children.
DR. GAMAL SOLTAN

23. Idiopathicin 25–50% of patients
Defects in cell-mediated immunity,
neutrophiland monocytefunction, and
humoralimmunity have been reported,
but none of these findings have been
demonstrated consistently
DR. GAMAL SOLTAN

24. Which is a reflection
of trivial trauma, not
only initiates the
cutaneous lesions but
also aggravates them,
and is seen in 20–30%
of patients with PG.
DR. GAMAL SOLTAN

25. Acute: acute onset with painful
hemorrhagic pustule or painful nodule
either de novo or after minimal trauma.
Chronic: slow progression with
granulation and hyperkeratosis. Less
painful
Superficial hemorrhagic pustule surrounded by
erythematoushalo; very painful
Breakdown occurs with ulcer formation, whereby
ulcer borders are dusky-red or purple, irregular
and raised, undermined, boggy with perforations
that drain pus
DR. GAMAL SOLTAN

26. The base of the ulcer is
purulent with hemorrhagic
exudate, partially covered
by necrotic eschar, with or
without granulation tissue.
Pustules both at the
advancing border and in
the ulcer base; a halo of
erythemaspreads
centrifugally at the
advancing edge of the
ulcer
DR. GAMAL SOLTAN

27. Chronic: lesions may
slowly progress, grazing
over large areas of the
body and exhibiting
massive granulation
within the ulcer from the
outset and crusting and
even hyperkeratosis on
the margins.
DR. GAMAL SOLTAN

28. DR. GAMAL SOLTAN
Lesions are usually
solitary but may be
multiple and form
clusters that coalesce.
Most common sites:
lower extremities
buttocks > abdomen
face. Healing of ulcers
results in thin atrophic
cribriformscars.

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30. Up to 50% of cases occurewithout disease.
Remainder of cases associated with
◦large-and small-bowel disease (Crohndisease, ulcerative
colitis),
◦Diverticulosis(diverticulitis)
◦arthritis
◦paraproteinemia
◦myeloma, leukemia
◦active chronic hepatitis
◦Behcetsyndrome.
DR. GAMAL SOLTAN

31. non-specific, and may
be nondiagnostic.
In early lesions, there is
a neutrophilicvascular
reaction, which can be
folliculocentric.
Neutrophilicinfiltrates,
often with
leukocytoclasia, are
seen in active untreated
expanding lesions
DR. GAMAL SOLTAN

32. 1. history and physical examination
2. Sterile skin biopsy of active skin lesion with sufficient depth
(panniculus) and sufficient tissue for special stains and culture
3. Gastrointestinal tract studies –stool for occult blood and
parasites, colonoscopy, biopsy, radiography, liver function tests
and
4. Hematologic studies-CBC, and bone marrow examination
5. Serologic studies –serum protein electrophoresis,
immunofixationelectrophoresis, antinuclear antibodies,
antiphospholipidantibodies
6. Chest X-ray and urinalysis
DR. GAMAL SOLTAN

33. Treat underlying disease.
PG
High doses of oral glucocorticoidsor IV glucocorticoid
pulse therapy (1–2 g/d prednisolone)
Sulfasalazine(particularly in cases associated with Crohn
disease), sulfones, cyclosporine,
infliximab, etanercept, adalimumabhave been shown to be
effective in uncontrolled studies.
Topical: In single lesion, topical tacrolimusointment
or intralesionaltriamcinolone.
DR. GAMAL SOLTAN

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35. Behçet’sdisease is a multisystem,
polysymptomaticdisease with
unpredictable exacerbations and
remissions.
All organs of the body can be affected, all
subspecialties can be involved in the care
of these patients.
DR. GAMAL SOLTAN

36. Ageof Onset: 3rd and 4th decades.
Prevalence:Highest in Turkey (80–420
patients in 100,000), Japan, Southeast Asia,
the Middle East, southern Europe. Rare in
northern Europe, United States (0.12–0.33 in
100,000).
Sex :Males > females, but dependent on
ethnic background.
DR. GAMAL SOLTAN

37. Etiology unknown.
In the eastern Mediterranean and East Asia,
HLA-B5 and HLA-B51 association; in the
United States and Europe, no consistent HLA
association.
The lesions are the result of leukocytoclastic
(acute) and lymphocytic (late) vasculitis.
DR. GAMAL SOLTAN

38. Painful ulcers erupt
in a cyclic fashion in
the oral cavity and/or
genital mucous
membranes. oral
ulcers may persist/
recur weeks to
months before other
symptoms appear.
DR. GAMAL SOLTAN

39. Skin and Mucous
Membranes Aphthous
Ulcers
Punched-out ulcers (3 to
>10 mm) with rolled or
overhanging borders and
necrotic base; red rim;
occur in crops (2–10) on
oral mucous membrane
(100%), vulva, penis, and
scrotum ; very painful.
DR. GAMAL SOLTAN

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42. ErythemaNodosum-Like Lesions Painful
inflammatory nodules on the arms and legs
(40%). Other Inflammatory pustules,
superficial thrombophlebitis, inflammatory
plaques resembling those in Sweet
syndrome, pyodermagangrenosum-like
lesions, palpable purpuriclesions of
necrotizing vasculitis.
DR. GAMAL SOLTAN

43. Eyes:Leading cause of morbidity. Posterior uveitis, anterior
uveitis, retinalvasculitis, vitreitis, hypopyon, secondary
cataracts, glaucoma, neovascularlesions.
Musculoskeletal: Nonerosive, asymmetric oligoarthritis.
Neurologic: Onset delayed, Meningoencephalitis, benign
intracranial hypertension, cranial nerve palsies, brainstem
lesions, pyramidal/extrapyramidallesions, psychosis.
Vascular: Aneurysms, arterial occlusions, venous
thrombosis, varices; hemoptysis. Coronary vasculitis:
myocarditis, coronary arteritis, endocarditis, valvulardisease.
GI Tract: Aphthousulcers throughout.
DR. GAMAL SOLTAN

44. Dermatopathology:Leukocytoclastic
vasculitiswith fibrinoidnecrosis of blood
vessel walls in acute early lesions;
lymphocytic vasculitisin late lesions.
Positive pathergytest read by physician
at 24 or 48 h, after skin puncture with a
sterile needle.
HLA Typing.
DR. GAMAL SOLTAN

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46. AphthousUlcers:
◦Potent topical glucocorticoids. Intralesional
triamcinolone, 3–10 mg/mL, injected in ulcer base.
◦Thalidomide, 50–100 mg PO in the evening.
◦Colchicine, 0.6 mg PO 2 to 3 times a day.
◦Dapsone, 50–100 mg/d PO.
Systemic Involvement:
◦Prednisone with or without azathioprine,
cyclophosphamide, azathioprinealone,
chlorambucil, cyclosporine.
DR. GAMAL SOLTAN

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