Sie sind auf Seite 1von 5

VDE210.

fm Page 209 Monday, July 16, 2001 3:36 PM

Veterinary Dermatology 2001, 12, 209 213

Case report

Blackwell Science, Ltd

Idiopathic linear pustular acantholytic dermatosis in


a young Brittany Spaniel dog
KARYN E. BENINGO and DANNY W. SCOTT
Department of Clinical Sciences, College of Veterinary Medicine, Cornell University, Ithaca, NY, USA
(Received 26 July 2000; accepted 29 January 2001)

Abstract A unilateral, linear, papularpustular dermatosis is described in a young Brittany Spaniel dog. The
dermatosis appeared to follow Blaschkos lines and extended from the left inguinal region to the medial aspect
of the left metatarsal area. The predominant histological finding was an eosinophilic and neutrophilic pustular
mural folliculitis with prominent acantholysis of infundibular epithelium. There was a rapid and long-lasting
(> 15 months) resolution after oral administration of methylprednisolone (1.6 mg kg1).
Keywords: acantholysis, Blaschkos lines, Brittany Spaniel, dog, eosinophilic and neutrophilic pustular mural
folliculitis, linear dermatosis.

INTRODUCTION
Linearity is a simple but important feature of skin
lesions because it often indicates an exogenous cause.1,2
Examples include the drawing of an irritant or allergen
across the skin, or excoriations produced by scratching.
Linearity may also be explained by the involvement of
blood or lymphatic vessels.1,2 Linear dermatoses may
also follow dermatomes (e.g. herpes zoster) or Blaschkos
lines (e.g. linear nevi).1,2 In humans, other linear dermatoses include lichen striatus, incontinentia pigmenti,
linear scleroderma (morphea) and linear porokeratosis.3
Linear dermatoses are rare in dogs, and include linear
sebaceous nevus,4 linear epidermal nevus,4,5 linear
organoid nevus,6,7 linear scleroderma8 and congenital
follicular parakeratosis.9 This study describes an idiopathic linear pustular acantholytic dermatosis in a young
Brittany Spaniel dog.

CASE REPORT
A 15-month-old, neutered female Brittany Spaniel dog,
weighing 12.3 kg, was referred to the Cornell University
College of Veterinary Medicine (CUCVM) for evaluation of an unusual skin lesion. The owner had first
noticed a rash in the left inguinal region 1 month
prior to presentation. The lesion was apparently mildly
pruritic, as the dog would lick the affected area. Within
a few days of being noticed, the lesion had spread
down the leg. The dog was otherwise healthy and had
no previous history of illness or drug administration.
Correspondence: Danny W. Scott. Department of Clinical Sciences,
College of Veterinary Medicine, Cornell University, Ithaca, NY, USA.
Tel.: +1 607 253 3015; Fax: +1 607 253 3534; E-mail: shb3@cornell.edu
2001 Blackwell Science Ltd

The dog had been vaccinated almost 1 year prior to the


onset of the skin lesion.
The dog was used for hunting and travelled from its
home in Pennsylvania to Michigan and Ontario
(Canada). The owner had noticed ticks on the dog
2 months prior to the appearance of the dermatosis,
but not in the area of the skin lesion. The ticks were
removed manually. There was another hunting dog in
the household that travelled with the patient, and it was
reported to be normal. Both dogs were housed in an
indoor kennel when the owner was not home, and had
free run of the home when the owner was present. It
was not known if littermates or parents had any history
of skin disease.
The referring veterinarian had made a diagnosis of
bacterial dermatitis and treated the dog with cephalexin
(22 mg kg1 twice daily orally for 3 weeks), to which there
was no response. A skin biopsy was performed and the
specimen evaluated at a local veterinary diagnostic
laboratory. The histopathological diagnosis was eosinophilic, histiocytic and neutrophilic dermatitis with focal
dermal pustules and ulcerations. The pathologist commented that the predominance of eosinophils suggested
an allergic reaction, such as can be seen with insect
bites. The dog was referred to the CUCVM for further
evaluation.
Physical examination revealed a healthy dog except
for the skin lesion. A linear, well-circumscribed, elevated
plaque, 1.55 cm in width, extended continuously from
the left inguinal region to the medial aspect of the left
metatarsal area (Fig. 1). The plaque consisted of clustered and coalescent erythematous to hyperpigmented
papules, many of which appeared umbilicated (Fig. 2).
Numerous annular brown crusts and occasional yellow
pustules were also present. Some areas of the skin
were lichenified. There was no palpable involvement
209

VDE210.fm Page 210 Monday, July 16, 2001 3:36 PM

210

K. E. Beningo and D. W. Scott

Figure 1. Linear plaque extending from left inguinal region to


medial aspect of left medial metatarsus.

Figure 2. Close-up of Fig. 1. Multiple clustered, coalescent papules


and pustules, some of which are umbilicated (arrow).

of blood or lymphatic vessels, and no palpable involvement of the subcutis. The regional lymph nodes
were normal upon palpation. The differential diagnosis
included a nevoid malformation and/or viral (especially
herpesvirus or poxvirus) or fungal (especially sporotrichosis) infection.
Cytological examination of impression smears of
pustular contents showed numerous eosinophils and
acantholytic keratinocytes, and occasional nondegenerate neutrophils. No microorganisms (fungal or
2001 Blackwell Science Ltd, Veterinary Dermatology, 12, 209 213

Figure 3. Pustular mural folliculitis with acantholysis (bar = 340 m).

bacterial) or cytopathic effects consistent with viral


infection (e.g. inclusion bodies within the cytoplasm
and/or nucleus of keratinocytes; ballooning degeneration
of keratinocytes; multinucleate keratinocytes) were seen.
The cytological findings were compatible with a sterile
acantholytic dermatitis, such as superficial pemphigus.
We were surprised and confused by these findings, and
recommended repeating the skin biopsy procedure.
Multiple biopsy specimens were collected using a
6-mm biopsy punch. Skin biopsy specimens were fixed
in 10% neutral phosphate-buffered formalin and routinely processed. The dominant histopathological finding
was an eosinophilic and neutrophilic pustular mural
folliculitis with marked acantholysis (Figs 3 and 4).
Eosinophils were the predominant inflammatory cell
within follicular microabscesses and pustules. Many
of the acantholytic keratinocytes contained keratohyalin granules. The surface epidermis and hair follicle
infundibula showed marked regular-to-irregular hyperplasia, mild-to-moderate orthokeratotic or parakeratotic
hyperkeratosis, and focally intense areas of intercellular
oedema and exocytosis of eosinophils. Neutrophils
were also a component of the follicular microabscesses
and pustules and the cellular exocytosis. The dermal
inflammatory response was superficial perivascular to
interstitial, and consisted of eosinophils with smaller
numbers of neutrophils, plasma cells, and lymphocytes.

VDE210.fm Page 211 Monday, July 16, 2001 3:36 PM

Linear acantholytic dermatosis

211

DISCUSSION

Figure 4. Eosinophilic and neutrophilic pustular mural folliculitis


with numerous acantholytic keratinocytes, many of which contain
keratohyalin granules (arrow) (bar = 34 m).

Special stains (acid-orcein Giemsa, Grocotts methenamine silver, periodic acid Schiff, Brown and Brenn
stain) revealed no microorganisms. The histopathological
findings were compatible with a diagnosis of superficial
pemphigus (foliaceus or erythematosus). Because of the
histopathological resemblance to superficial pemphigus,
direct immunofluorescence testing was performed as
described previously,10 and was negative for immunoglobulin (Ig)G, IgM, IgA and complement.
The final diagnosis was an idiopathic linear pustular
acantholytic dermatosis. Various therapeutic options and
observation without treatment were discussed with the
owner, and it was decided to treat the dog with systemic
glucocorticoids. Because of the histopathological resemblance to superficial pemphigus, an immunosuppressive
dose of methylprednisolone (1.6 mg kg1 once daily orally)
was chosen. The referring veterinarian re-examined the
dog 2.5 weeks later and reported that all skin lesions,
other than the hyperpigmentation, had resolved. The
oral methylprednisolone therapy was tapered slowly and
eventually stopped. The hyperpigmentation disappeared
slowly over the course of the next 3 months.
It has now been 15 months since the skin lesions
resolved. The dog continues to hunt and be healthy,
and has had no recurrences of the dermatosis. The
owner contacted the breeder, who then contacted the
owners of the patients siblings. No similar skin lesions
have been seen in either the parents or the siblings.

The linear pustular acantholytic dermatosis reported in


this dog is unique. We could find no reports of analogous
dermatoses in the veterinary or medical literature. Clinically, the dermatosis appeared to follow Blaschkos lines,
and was reminiscent of linear nevi.47 However, the
pustules and umbilicated papules were a unique feature.
The umbilicated papules were suggestive of poxvirus
lesions. Cytologically, the eosinophils, acantholytic
keratinocytes and nondegenerate neutrophils suggested
a sterile acantholytic disease such as superficial
pemphigus.2
Histopathologically, the eosinophilic, neutrophilic,
acantholytic process was compatible with superficial
pemphigus.2 Many of the acantholytic keratinocytes
contained keratohyalin granules, suggesting that the
acantholytic process may have begun in the stratum
granulosum. This is also strongly suggestive of superficial pemphigus.2 However, isolated linear lesions are
not reported as a manifestation of superficial pemphigus
in animals or humans. In addition, direct immunofluorescence testing was negative. The other feature of
this disorder that is inconsistent with pemphigus is the
one-time occurrence and the short duration of therapy
that led to remission.
The aetiology of this linear pustular acantholytic
dermatosis is unknown. Although linearity often indicates exogenous causes,1,2 the history and histopathological findings in our patient mitigate against a contact
or self-induced dermatitis. In addition, although eosinophilic microabscesses may be seen in biopsy specimens
from dogs with contact hypersensitivity,11 these are
intraepidermal, not follicular. Linear lesions may be
seen with vasculitis and lymphangitis.1,2 However, there
was neither clinical nor histopathological evidence of
vascular inflammation.
Necrotizing eosinophilic mural folliculitis has been
associated with insect stings and arthropod bites.2,12
The dermatosis in our patient began in December, an
unlikely time to encounter insects in upstate New York.
Although ticks had been present on our patient
2 months prior to the recognition of skin lesions, they
were not seen at the site of the lesion, nor have tick bites
been associated with linear dermatoses or necrotizing
eosinophilic mural folliculitis. In addition, necrotizing
mural folliculitis was not a feature of the biopsy specimens from our patient. Rather, the key histopathological
finding in our patient would be classified as an eosinophilic and neutrophilic pustular mural folliculitis.12
Trichophyton pustular dermatophytosis can mimic
superficial pemphigus, both clinically and histologically.13 Special stains for fungi were negative in our
patient. In addition, linear dermatophytosis has not
been reported. Lastly, the rapid, enduring response to
systemic glucocorticoids observed in our patient would
not have been achieved in dermatophytosis.
In humans, linear lesions also result from the so-called
isomorphic effect (Koebner phenomenon).14,15 This
phenomenon involves the development in previously
2001 Blackwell Science Ltd, Veterinary Dermatology, 12, 209213

VDE210.fm Page 212 Monday, July 16, 2001 3:36 PM

212

K. E. Beningo and D. W. Scott

normal skin, which has been traumatized either externally or internally, of lesions of the very same (iso-)
form (-morphic) that is characteristic of an existing
dermatosis. Although the isomorphic effect has been
described in numerous skin disorders of humans, it
occurs most commonly with psoriasis and lichen
planus.14,15 The pathogenesis of this phenomenon is
not known. Our patient had no pre-existing dermatosis,
thus eliminating the possibility of koebnerization.
The dermatosis in our patient appeared to follow
Blaschkos lines. These lines determine the distribution
pattern of many congenital and acquired skin diseases.16 18
The exact nature of Blaschkos lines is unknown, but
many believe that they represent the pattern of embryonic migration of skin cells and can often be produced
by genetic mosaicism. The young age of our patient
when the dermatosis was recognized suggests the possibility of a developmental defect, possibly hereditary
in nature. However, the dogs siblings and parents
were reported to not have a similar dermatosis.
Relapsing linear acantholytic dermatosis is an
extremely rare, idiopathic skin disorder reported in
humans.19,20 These patients have childhood-onset,
unilateral, linear lesions composed of erythematous
papules and plaques that follow Blaschkos lines. To
this extent, the dermatosis resembles that seen in our
patient. However, the human disorder is also characterized by a life-long relapsing course, a poor response
to systemic glucocorticoids, and acantholytic microvesicles and clefts within the stratum spinosum of the
epidermis, all of which were not features of our patients
dermatosis.
In conclusion, we describe a patient with what appears
to be a previously unreported linear dermatosis. It is
unilateral, linear, papulopustular, appears to follow
Blaschkos lines, and is characterized histologically by
an eosinophilic and neutrophilic pustular mural folliculitis. Response to systemic glucocorticoid therapy
was rapid and long-lasting. Until more is learned
concerning the pathogenesis of this disorder, we propose
to refer to it as idiopathic linear pustular acantholytic
dermatosis.

3.

4.

5.

6.

7.
8.

9.

10.

11.

12.

13.

14.

15.

16.

17.

18.

REFERENCES
1. Fitzpatrick, T.B., Bernhard, J.D., Cropley, T.G. The
structure of skin lesions and fundamentals of diagnosis.
In: Freedberg, I.M., Eisen, A.Z., Wolff, K. et al., eds.
Fitzpatricks Dermatology in General Medicine, 5th
edn. New York: McGraw-Hill, 1999: 1341.
2. Scott, D.W., Miller, W.H. Jr, Griffin, C.E. Muller and

19.

20.

Kirks Small Animal Dermatology, 5th edn. Philadelphia:


W.B. Saunders, 1995: 55173.
Waisman, M. Lichen striatus. In: Demis, D.J., ed. Clinical
Dermatology, Vol. 1 Unit 111. Philadelphia: J.B. Lippincott,
1994: 14.
Scott, D.W., Yager-Johnson, J.A., Manning, T.O. et al.
Nevi in the dog. Journal of the American Animal Hospital
Association 1984; 20: 50512.
White, S.D., Rosychuk, R.A.W., Scott, K.V. et al. Inflammatory linear verrucous epidermal nevus in four dogs.
Veterinary Dermatology 1993; 3: 10714.
Paradis, M., Scott, D.W. Nevi rcemment reconnus chez
le chien: nevus comdonien, nevus organode linaire et
nevus du follicule pileux. Point Vtrinaire 1989; 21: 48993.
Scarampella, F., von Tscharner, C., Noli, C. Linear organoid
nevus in a dog. Veterinary Dermatology 2000; 11: 6973.
Scott, D.W. Localized scleroderma (morphea) in two dogs.
Journal of the American Animal Hospital Association
1986; 22: 20711.
Scott, D.W., Miller, W.H. Jr. Congenital follicular parakeratosis in a rottweiler and a Siberian husky. Canine
Practice 2000; 25: 1619.
Seiler, R.J., Scott, D.W., Lewis, R.M. Comparative
immunodermatology. In: Beutner, E.H., Chorzelski, T.P.,
Kumar, V., eds. Immunopathology of the Skin, 3rd edn.
New York: Churchill Livingstone, 1987: 30320.
Walder, E.J., Conroy, J.D. Contact dermatitis in dogs and
cats: pathogenesis, histopathology, experimental induction,
and case reports. Veterinary Dermatology 1994; 5: 14962.
Gross, T.L., Stannard, A.A., Yager, J.A. An anatomical
classification of folliculitis. Veterinary Dermatology
1997; 8: 14756.
Parker, W.M., Yager, J.A. Trichophyton dermatophytosis
a disease easily confused with pemphigus erythematosus.
Canadian Veterinary Journal 1997; 38: 5025.
Leider, M., Rosenblum, M. A Dictionary of Dermatological
Words, Terms, and Phrases. New York: McGraw-Hill,
1965: 232.
Boyd, A.S., Neldner, K.H. The isomorphic response of
Koebner. International Journal of Dermatology 1990; 29:
40110.
Bolognia, J.L., Orlow, S.J., Glick, S.A. Lines of Blaschko. Journal of the American Academy of Dermatology
1994; 31: 15790.
Soma, Y., Fujimoto, M. Frontoparietal scleroderma (en
coup de sabre) following Blaschkos lines. Journal of the
American Academy of Dermatology 1998; 38: 3668.
Restano, L., Cambiaghi, S., Tadini, G. et al. Blaschkos lines
of the face: a step closer to completing the map. Journal of
the American Academy of Dermatology 1998; 39: 102830.
Vakilzadeh, F., Kolde, G. Relapsing linear acantholytic
dermatosis. British Journal of Dermatology 1985; 112:
34955.
Duschet, P., Happle, R., Schwarz, T. et al. Relapsing
linear acantholytic dermatosis. Journal of the American
Academy of Dermatology 1995; 33: 9202.

Rsum Cet article dcrit une dermatose unilatrale, linaire, papulo-pustuleuse chez un jeune Epagneul breton.
La dermatose semble suivre les lignes de Blaschkos et stend de la rgion inguinale gauche jusqu la zone mdiale
du mtatarse gauche. Lexamen histopathologique a montr une folliculite murale pustuleuse osinophilique
et neutrophilique avec une acantholyse de lpithlium infundibulaire. Une rsolution rapide et prolonge
(> 15 mois) des lsions a t observe aprs ladministration orale de methylprednisolone (1.6 mg kg1). [Beningo
K. E., Scott, D. W. Idiopathic linear pustular acantholytic dermatosis in a young Brittany Spaniel dog. (Dermatose
linaire pustuleuse acantholytique chez un jeune chien Epagneul breton.) Veterinary Dermatology 12: 209213.]
2001 Blackwell Science Ltd, Veterinary Dermatology, 12, 209 213

VDE210.fm Page 213 Monday, July 16, 2001 3:36 PM

Linear acantholytic dermatosis

213

Resumen Se describe en un perro joven de raza Brittany Spaniel una dermatosis unilateral, linear, papular
pustular. La dermatosis pareci seguir las lneas de Blaschko y se extendi desde la regin inguinal izquierda al
aspecto medial del rea metatarsiana izquierda. El hallazgo histolgico predominante fue una foliculitis mural
eosinoflica y neutroflica pustular con acantolisis marcada del epitelio infundibular. Se produjo una resolucin
rpida y duradera (> 15 meses) despus de la administracin oral de metilprednisolona (1.6 mg kg1). [Beningo
K. E., Scott, D. W. Idiopathic linear pustular acantholytic dermatosis in a young Brittany Spaniel dog. (Dermatosis
linear pustular acantoltica en un perro Brittany Spaniel joven.) Veterinary Dermatology 12: 209213.]
Zusammenfassung Eine unilaterale, lineare, papulre-pustulse Dermatose wird bei einem jungen Brittany
Spaniel beschrieben. Die Dermatose folgte den Blaschko-Linien und erstreckte sich von der linken Inguinalgegend zur medialen Seite der linken Metatarsalgegend. Histologisch dominierte eine eosinophile und neutrophile,
pustulre, murale Follikulitis mit prominenter Akantholyse des infundibulren Epithels. Schnelle und andauernde
Remission (> 15 Monate) erfolgte nach oraler Behandlung mit Methylprednisolon (1.6 mg kg1). [Beningo K. E.,
Scott, D. W. Idiopathic linear pustular acantholytic dermatosis in a young Brittany Spaniel dog. (Idiopathische lineare
pustulre akantholytische Dermatose bei einem jungen Brittany Spaniel.) Veterinary Dermatology 12: 209213.]

2001 Blackwell Science Ltd, Veterinary Dermatology, 12, 209213

Das könnte Ihnen auch gefallen