Professional Documents
Culture Documents
Candidate:
TIMISOARA
2023
STERILE EOSINOPHILIC PUSTULE, SUBCORNEAL PUSTULAR DERMATITIS, BIANCA AMALIA KÖRTVELYESSY
HYPEREOSINOPHILIC SYNDROME, IDIOPATHIC STERILE GRANULOMA
CONTAINED
1. STERILE EOZINOFILIC PUSTULA .......................................................................... 2
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STERILE EOSINOPHILIC PUSTULE, SUBCORNEAL PUSTULAR DERMATITIS, BIANCA AMALIA KÖRTVELYESSY
HYPEREOSINOPHILIC SYNDROME, IDIOPATHIC STERILE GRANULOMA
The cause and pathogenesis are unknown. Peripheral eosinophilia, sterile tissue
eosinophilia, and the ability to react to systemic glucocorticoids that characterize this syndrome
suggest that it may be immune-mediated. However, intradermal skin testing, hypoallergenic diets
and immunopathological studies have not been helpful in elucidating etiopathogenesis. Cats also
rarely have clinically and pathologically sterile eosinophilic folliculitis; However, the clinical
presentation is different and lesions are associated with an underlying hypersensitivity such as
atopic disease, food hypersensitivity, hypersensitivity to flea bites and hypersensitivity to
mosquito bites. [25]
There is no apparent predilection of age, race or gender. The onset of clinical signs is acute,
and the distribution of lesions is multifocal (especially involving the trunk) or generalized. Pruritic,
erythematous, follicular and non-follicular papules and pustules evolve into annular erosions with
epidermal collars. Peripheral spread, central healing and hyperpigmentation of lesions lead to
numerous target lesions. Although most dogs are otherwise healthy, fever, anorexia, depression
and peripheral lymphadenopathy may be present.
1.3. DIAGNOSTIC
eosinophilia (up to 5.6 × 103/ml). Direct smears reveal numerous eosinophils, nondegenerative
neutrophils, occasional and micro-free acantholytic keratinocytes. [23] Carefully made cultures
are negative. Biopsy reveals one or often a combination of subcornian, intraepidermal, or follicular
eosinophilic pustules. [8] Eosinophilic folliculitis and furunculosis may occur. Flame figures are
occasionally visible in the surrounding dermis. Direct and indirect immunofluorescence results are
negative. Serum α, β and γ globulins may be increased. [26]
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STERILE EOSINOPHILIC PUSTULE, SUBCORNEAL PUSTULAR DERMATITIS, BIANCA AMALIA KÖRTVELYESSY
HYPEREOSINOPHILIC SYNDROME, IDIOPATHIC STERILE GRANULOMA
Most dogs respond well to systemic glucocorticoids (oral prednisone or prednisolone, 2.2
to 4.4 mg/kg once daily) within 5 to 10 days. However, stopping treatment constantly leads to
relapses, so long-term morning alternative therapy is indicated, and cure is unlikely. In two dogs
that could not be treated with glucocorticoids, it was good success with dapsone, or the
combination of an antihistamine (diphenhydramine) and a supplement of omega-6 and omega-3
fatty acids.
There is no apparent predilection of age or gender. Although many breeds were affected,
miniature schnauzers accounted for about 40% of cases. Dachshunds have also been reported as a
suspected breed. [8]
Affected dogs usually have generalized multifocal dermatitis, pustular to seborrhea. The
head and torso, in particular, are affected in a symmetrical way. Intact pustules are usually
nonfollicular, greenish yellow and transient, persisting for only 2 to 4 hours at a time. Thus,
affected dogs often have only circular areas of alopecia, erosion, scaling, crusts and epidermal
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STERILE EOSINOPHILIC PUSTULE, SUBCORNEAL PUSTULAR DERMATITIS, BIANCA AMALIA KÖRTVELYESSY
HYPEREOSINOPHILIC SYNDROME, IDIOPATHIC STERILE GRANULOMA
collars. The lesions tend to heal centrally, often with hyperpigmentation, and spread peripherally,
producing annular and serpiginous formations. Rarely, footrests are affected and peel off
superficially. Itching ranges from nonexistent to extreme. The course of dermatosis is often to
erupt and regress. Usually these dogs are otherwise healthy. Occasional dogs have peripheral
lymphadenopathy; rarely, they have pyrexia, anorexia and depression.
1.3. DIAGNOSIS
Since this dermatosis is diagnosed by ruling out other conditions, improved diagnostic
techniques should make it rare.
Differential diagnosis includes bacterial folliculitis, pemphigus foliacea, linear IgA pustular
dermatosis, systemic lupus erythematosus, sterile eosinophilic pustulosis, superficial pustular drug
rash and superficial pustular dermatophytosis. The definitive diagnosis is based on history,
physical examination, exclusion by laboratory tests, and response to therapy. Subcornian pustular
dermatosis responds poorly to systemic antibiotics, systemic glucocorticoids and topical agents.
Direct smears from intact pustules usually reveal numerous nondegenerate neutrophils, occasional
acantolytic keratinocytes, and no microorganisms. Carefully made cultures of intact pustules are
usually negative, but a few colonies of coagulase-negative or coagulase-positive staphylococci are
occasionally isolated. The results of immunofluorescence tests are negative. Skin biopsy reveals
intraepidermal (subcornian) pustular dermatitis. [12] Acantholysis is usually minimal, but is
occasionally marked. Neutrophils do not show degenerative changes. Hair follicles are rarely
involved.
Up to half of affected dogs may have mild to moderate mature neutrophilia (13.8–21.1 ×
10.3/ml). Serum protein electrophoresis occasionally reveals increased amounts of α1, α2, and β
globulins. [6]
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STERILE EOSINOPHILIC PUSTULE, SUBCORNEAL PUSTULAR DERMATITIS, BIANCA AMALIA KÖRTVELYESSY
HYPEREOSINOPHILIC SYNDROME, IDIOPATHIC STERILE GRANULOMA
In dogs, the major side effects of dapsone were hematological and hepatic. Many dogs
experience mild nonregenerative anemia and leukopenia, as well as mild to moderate increases in
serum alanine aminotransferase during induction therapy. If these laboratory abnormalities are not
associated with clinical signs, it is not necessary to stop therapy; Levels will return to normal when
maintenance doses are reached. Dapsone also caused fatal thrombocytopenia in a dog, profuse
leukopenia, occasional vomiting and generalized, erythematous, maculopapular diarrhea and itchy
rash. Dapsone is not authorised for use in dogs. [12]
Very rarely, dogs apparently become resistant to dapsone. They may or may not benefit from
oral administration of sulfasalazine 10 to 20 mg/kg every 8 hours until dermatosis is controlled
and then as needed. Chronic administration of sulfasalazine may be associated with
keratoconjunctivitis sicca. A dog that did not respond to dapsone was successfully treated with
injectable gold salts. [3]
Hypereosinophilic syndrome is a rare disorder of cats. [26] , [19] It has also been recognized
in dogs, but the skin is usually not affected. It is characterized by chronic idiopathic
hypereosinophilia associated with a diffuse infiltration of various organs by mature eosinophils.
[11]
The cause and pathogenesis of this syndrome are unknown. Hypereosinophilia is present
in humans when peripheral blood eosinophil counts exceed 1.5 × 109/L, and hypereosinophilic
syndrome involves idiopathic hypereosinophilia persisting for more than 6 months, non-clonal
eosinophil expansion, and organ damage caused by eosiniphils. [28] It represents a non-neoplastic
leukoproliferative process with migration of eosinophils to different organs. Hypereosinophilia
greater than 1.5 × 109/L occurred secondary to a number of diseases, but was most common in
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STERILE EOSINOPHILIC PUSTULE, SUBCORNEAL PUSTULAR DERMATITIS, BIANCA AMALIA KÖRTVELYESSY
HYPEREOSINOPHILIC SYNDROME, IDIOPATHIC STERILE GRANULOMA
cats allergic to fleas and dogs with scabies when skin disease was present. [14] It has been
recommended that peripheral blood eosinophils exceed 5 × 109/L to be considered
hypereosinophilia in dogs and cats.
The disease is more common in middle-aged female cats. [19] No breed or sex predilection
has been found, but rottweiler dogs appear to be at risk. [13] Infiltration of tissues with maturity
eosinophils lead to dysfunction of multisystem organs. The bone marrow, lymph nodes, liver,
spleen, and gastrointestinal tract are usually affected. Rarely, cardiac abnormalities are found; In
a cat, restrictive cardiomyopathy was a clinical feature. [22] The most common clinical signs
reflect gastrointestinal involvement and include diarrhea, weight loss, vomiting, and anorexia. [10]
Physical examination often reveals thickened intestinal loops, lymphadenopathy, and
hepatosplenomegaly.
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STERILE EOSINOPHILIC PUSTULE, SUBCORNEAL PUSTULAR DERMATITIS, BIANCA AMALIA KÖRTVELYESSY
HYPEREOSINOPHILIC SYNDROME, IDIOPATHIC STERILE GRANULOMA
3.3. DIAGNOSTIC
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STERILE EOSINOPHILIC PUSTULE, SUBCORNEAL PUSTULAR DERMATITIS, BIANCA AMALIA KÖRTVELYESSY
HYPEREOSINOPHILIC SYNDROME, IDIOPATHIC STERILE GRANULOMA
The prognosis is unfavorable; In most cases, survival times are short and patients do not
respond to any treatment. One dog was reported to go into remission, and another was successfully
treated with hydroxyurea and prednisolone. [18] A cat also responded to hydroxyurea and
prednisolone. [14] Cats with skin lesions may have a slowly progressive disease and longer
survival times (2-4 years). These cats may have a favorable but transient response to high doses
of glucocorticoids. The chemotherapeutic agent hydroxyurea also appears beneficial in dogs and
cats when used together with prednisolone. Hydroxyurea is a ribonucleotide reductase inhibitor
that leads to decreased DNA synthesis. It is mainly lethal to cells in phase S of the growth cycle.
Hydroxyurea led to onychomadesis. [15] Hydroxyurea and interferon alfa are used in humans
when glucocorticoids alone are ineffective. In cases resistant to these therapies, T-cell suppressing
drugs, cyclosporine, or the c-kit inhibitor imatinib mesilate may be useful. [1]
The cause and pathogenesis of this syndrome is unknown. It has been speculated that an
immune dysfunction or aberrant response to unidentified infectious agents or antigens from
infectious agents may initiate the response. [7] This is supported by several observations. Some
cases respond to tetracycline or doxycycline, although it is possible that the drugs work through
anti-inflammatory mechanisms. [20] Studies in 46 cases previously diagnosed as SGPS showed
that 21 (46%) are positive for Leishmania by PCR testing and an immunohistochemical technique.
[5] The characteristic granulomatological histopathological appearance, the absence of microbial
agents and foreign material, and the good response to systemic glucocorticoids and cyclosporine
suggest an aberrant inflammatory histiocytological response. [19] In human sarcoidosis, a classic
"sterile" granulomatous disease, polymerase chain reaction (PCR) technology has shown that
mycobacterial DNA (several species) is present in 80% of skin lesions. PCR testing for
mycobacterial antigen was negative in all 46 cases examined in a single study. [5] Other infectious
agents, such as tickborne infectious disease, may also be good candidates to evaluate in these cases.
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STERILE EOSINOPHILIC PUSTULE, SUBCORNEAL PUSTULAR DERMATITIS, BIANCA AMALIA KÖRTVELYESSY
HYPEREOSINOPHILIC SYNDROME, IDIOPATHIC STERILE GRANULOMA
4.2.1. Dog
The disorder can occur in dogs of all ages, breeds, and genders, but collies, dachshunds,
Doberman pinschers, English bulldogs, Weimaraners, Great Danes, boxers, and golden retrievers
may be predisposed. [7] , [20] The lesions are firm, painless, nonpruritic dermal papules, plaques
and nodules. The lesions can develop into donut-shaped circular lesions and become alopecia,
ulcerated and secondarily infected. The lesions are usually multiple and usually affect the head
(especially the bridge of the nose, muzzle and periocular region), pinnae and paws. One patient
had lesions involving the tongue, in addition to more typical lesions. [4] Injuries have also been
observed involving the foreskin. Animals are usually otherwise healthy. A dog with sterile
pyogranuloma syndrome had associated hypercalcemia.
Figure 4.1. Alopecia and erythematosus dermatitis with ulcerative wounds on the head (A), right limb (B),
dorsal region (C), ventral region (D), [Kawarai, 2014]
4.1.1. Cat
In cats, papules firm to nodules develop and can coagulate into plaques. The lesions are
usually erythematous to purplish in color, although some may be orange-yellow. These plates
become reddish purple on palpation. Lesions are often found on the head, muzzle and pinna, but
can also appear on the paws and less commonly, truncally. In some cats, lesions may be itchy, but
this is a variable finding.
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STERILE EOSINOPHILIC PUSTULE, SUBCORNEAL PUSTULAR DERMATITIS, BIANCA AMALIA KÖRTVELYESSY
HYPEREOSINOPHILIC SYNDROME, IDIOPATHIC STERILE GRANULOMA
4.3. DIAGNOSTIC
taken orally at 2.2 mg/kg once daily until remission, then on alternate days. Once remission has
been maintained for several months, therapy can be successfully discontinued in some cases.
Others may require long-term low-dose therapy. We were occasionally able to reduce the dose and
frequency of azathioprine to 0.25 mg/kg once weekly. In cats, similar regimens can be used, except
that chlorambucil is used instead of azathioprine. In cats, lesions often resolve spontaneously after
about 9 months. [26]
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STERILE EOSINOPHILIC PUSTULE, SUBCORNEAL PUSTULAR DERMATITIS, BIANCA AMALIA KÖRTVELYESSY
HYPEREOSINOPHILIC SYNDROME, IDIOPATHIC STERILE GRANULOMA
BIBLIOGRAPHY
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STERILE EOSINOPHILIC PUSTULE, SUBCORNEAL PUSTULAR DERMATITIS, BIANCA AMALIA KÖRTVELYESSY
HYPEREOSINOPHILIC SYNDROME, IDIOPATHIC STERILE GRANULOMA
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HYPEREOSINOPHILIC SYNDROME, IDIOPATHIC STERILE GRANULOMA
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Web site-uri
3. Kawarai, Shinpei & Matsuura, Shinobu & Yamamoto, Saburo & Kiuchi, Akio &
Kanemaki, Nobuyuki & Madarame, Hiroo & Shirota, Kinji. 2014,
https://www.researchgate.net/publication/262582557_A_Case_of_Cutaneous_Sterile_Py
ogranulomaGranuloma_Syndrome_in_a_Maltese, A Case of Cutaneous Sterile
Pyogranuloma/Granuloma Syndrome in a Maltese, Journal of the American Animal
Hospital Association, accesat 24.05.2023.
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