Beruflich Dokumente
Kultur Dokumente
Case report
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
210
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
211
DISCUSSION
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.
212
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.
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
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.]