Veterinary Dermatology 2002, 13, 331 336

Case Report

Blackwell Science, Ltd

Pemphigus foliaceus in a goat

ERSILIA PAPPALARDO*, FRANCESCA ABRAMO and CHIARA NOLI*
*Studio Dermatologico Veterinario, Milan, Italy; Department of Animal Pathology,
University of Pisa, Pisa, Italy
(Received 16 April 2002; accepted 15 August 2002)

Abstract A 7-year-old-female goat was referred with a 3-month history of chronic dermatitis, which partially
responded to combined corticosteroid and antibiotic therapy. At dermatological examination diffuse alopecia,
pustules and crusts were observed on the head, neck, dorsum and perianal area. Dermatophyte culture and skin
scrapings were negative. Trichoscopic examination revealed a concurrent infestation with Damalinia caprae,
which was successfully treated with ivermectin. Cytological examination of pus from intact pustules revealed
nondegenerate neutrophils, absence of bacteria and numerous nucleated, irregularly shaped keratinocytes.
Histopathological examination of lesional skin revealed intracorneal pustules containing neutrophils and
acantholytic cells, and a mixed cell superficial perivascular dermatitis. Immunohistochemical stains of lesional
skin showed intercellular IgG deposits in the spinous layer. Remission of the dermatitis was obtained with
injectable dexamethasone-21-isonicotinate, every two months for one year. This is the first report to describe
the cytological appearance of impression smears from intact pustules of pemphigus foliaceus in a goat and to
document the presence of IgG deposits in lesional skin by means of immunohistochemistry.
Keywords: goat, pemphigus foliaceus, skin

INTRODUCTION
Pemphigus foliaceus (PF) is a rarely described autoimmune dermatosis in the goat, characterized by widespread pustules and scaling. Only three cases have been
reported to date.13 We describe a further case of PF
in the goat, in which the diagnosis was obtained by
cytological examination of smears, by standard histopathology and by immunohistochemistry.

CASE REPORT
A 7-year-old female nulliparous Sardinian goat was
referred with a 3-month history of a chronic dermatitis,
characterized by generalized nonpruritic pustular and
exfoliative lesions and widespread multifocal alopecia.
In the past, the goat had suffered from recurrent
bacterial mastitides. A single injection of a long-acting
glucocorticoid (dexamethasone-21-isonicotinate, 0.04
mg kg1 IM) and antibiotic therapy for 7 days (penicillin
40 000 IU kg1 and dihydrostreptomycin 50 mg kg1
once daily IM) had been administered eight weeks
before by the referring veterinarian, resulting in marked

Correspondence: Chiara Noli, Strada Madonna 58, 12016 Peveragno

(CN), Italy. E-mail: pitnoli@iol.it
Present address: Via A. da Messina 75, Acicastello (CT), Italy.
2002 Blackwell Science Ltd

Figure 1. Sardinian goat with pemphigus foliaceus: patchy alopecia

and scaly lesions localized on the head, neck and dorsum.

improvement. The goat lived with a healthy horse which

had no dermatological lesions.
On clinical examination, the goat appeared in good
general health. No lymphoadenomegaly was found.
On dermatological examination a diffuse alopecic
pustular and crusting dermatitis was observed on the
head, pinnae, neck, dorsum and perianal area (Figs 13).
The ventral areas and the limbs were not affected.
On the dorsum the coat was dull. The main lesions
consisted of pustules of variable sizes (Fig. 4), some
of which formed by the confluence of a number of
pustules. Most pustules were surrounded by an erythematous collarette. Large yellowish crusts and scales
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E. Pappalardo et al.

Figure 2. Diffuse alopecia, crust, scales and pustules involving the

head, muzzle and pinnae.

Figure 4. Large coalescing pustules, crusts and scales on the

temporo-mandibular area.

Figure 5. Patchy alopecia and exfoliation on the back.

Figure 3. Exfoliating alopecia, crusts and pustules in the perineal

region.

were present, and the hair was easily epilated, leaving

large patches of alopecia (Fig. 5).
Based on the history and clinical findings the possible
differential diagnoses included pyoderma, dermatophilosis,
zinc-responsive dermatosis, ectoparasite infestations,
dermatophytosis and pemphigus foliaceus.
Multiple skin scrapings were negative for the presence of ectoparasites. Microscopy examination of perilesional hair revealed the presence of adult Damalinia
caprae and their eggs. Hair samples from multiple
sites were submitted for dermatophyte culture, which
yielded negative results. A pus specimen was collected
from intact pustules and submitted for cytological
examination, which revealed the presence of intact
2002 Blackwell Science Ltd, Veterinary Dermatology, 13, 331 336

neutrophils and no bacterial elements. A number of

acantholytic cells was observed among neutrophilic
granulocytes and corneocytes (Figs 6 and 7). The
acantholytic cells appeared as nucleated keratinocytes,
with a light halo of cornification surrounding the
nucleus, and had an irregular, polygonal shape, in
contrast to equine, canine and feline acantholytic cells,
which are small and rounded. In spite of the unusual
appearance of the acantholytic cells, their presence in
high numbers, the presence of nondegenerate neutrophils
and the absence of intra- or extracellular bacteria,
allowed a tentative diagnosis of pemphigus foliaceus,
complicated by louse infestation, to be made.
In order to confirm the diagnosis, several skin biopsies were obtained from lesional skin on the trunk and
in the perineal area, by means of an 8-mm punch. Skin
samples were fixed in a 10% buffered formalin solution
and routinely embedded in paraffin. Five-micrometre
thick sections were stained with haematoxylin and eosin
(H&E) for histological evaluation, additional sections
were used for immunohistochemistry. A streptavidin
biotin peroxidase method was performed using a
biotinylated pan-specific antimouse/antirabbit/antigoat
IgG as primary antibody (Vector Laboratories, Burlingame,
CA, USA). A sample from normal goat skin was used as
a control. The histopathological findings included:

Pemphigus foliaceus in a goat

333

Figure 6. Cytological appearance of a Tzanck preparation from an

intact pustule from a goat with pemphigus foliaceus: numerous
nondegenerate neutrophils and acantholytic cells are seen.
(Hemacolor, original magnification 10.)

Figure 8. Histological preparation of an intact pustule of a goat

affected by pemphigus foliaceus. An intracorneal pustule containing
neutrophilic granulocytes and acantholytic cells may be observed.
(H&E, original magnification 10.)

Figure 7. Same slide as Fig. 6. At higher magnification the irregular,

poliedrical shape of the acantholytic cells, the presence of corneocytes
and the absence of bacteria can be appreciated. (Hemacolor, original
magnification 40.)

lamellar orthokeratotic hyperkeratosis, irregular

epidermal hyperplasia, intracorneal pustules containing
intact neutrophils and free irregularly shaped nucleated
keratinocytes and corneocytes, mild neutrophilic
exocytosis, and a superficial perivascular infiltrate of
neutrophils, lymphocytes and macrophages (Figs 8 and 9).
Using immunohistochemistry, intercellular IgG deposits
were observed in lesional skin sections in the spinous
and granular layers of the epidermis (Fig. 10). IgG
deposition was not found in the normal goats skin (not
shown). The histopatholgical lesions were considered
diagnostic of pemphigus foliaceus, thus confirming the
cytological preliminary diagnosis.
The louse infestation was treated with two injections
of ivermectin (0.2 mg kg1) two weeks apart. The owner
refused daily oral steroid therapy and injectable gold
therapy. Thus, one injection of long-acting glucocorticoid (dexamethasone-21-isonicotinate 0.04 mg kg1 IM)
was administered, resulting in marked improvement of
the cutaneous condition within one week. Two weeks
later the skin had recovered completely and after one
month the coat had grown back. About two months
after the injection, the skin condition recurred and
the injectable therapy was repeated obtaining the same
results. The same long-acting glucocorticoid was
administered every two months for one year, and was
able to keep the disease in remission. After this time
the goat was euthanized because of a severe relapse of
the bacterial mastitis.
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E. Pappalardo et al.

Figure 9. Same slide as Fig. 8. At higher magnification the irregular

shape of the acantholytic cells, similar to the cytological appearance,
may be appreciated. (H&E, original magnification 40.)

Figure 10. IgG-immunostaining of lesional skin of a goat affected

by pemphigus foliaceus. Antibody deposition is apparent in the
intercellular spaces of the spinous layer. (Original magnification
20.)

DISCUSSION
Pemphigus foliaceus (PF) is the most commonly recognized form of pemphigus in all domestic animals, and
has been described in dogs, cats, horses and goats.14
This autoimmune disease is characterized by the production of autoantibodies directed against desmosomal
antigens located in the intercellular spaces of the
suprabasal epidermis. In human beings and dogs, the
major PF antigen is desmoglein 1, a 150 kDa glycoprotein
2002 Blackwell Science Ltd, Veterinary Dermatology, 13, 331 336

of the cadherin group of adhesion molecules. 5,6

Even though the pathogenesis is still not well understood,
the production of autoantibodies against this antigen
seems to be responsible for the destruction of suprabasal epidermal intercellular bridges and for cellcell
detachment (acantholysis). Acantholytic cells are
typically observed in cytological and histological specimens
from intact pustules.
Only three cases of pemphigus foliaceus have been
reported in the goat.13 The history, type and distribution
of lesions described in these animals are similar to those
observed in our case, except for the absence of lesions
on the abdomen and udder. In contrast to the other
cases, in which the disease occurred in young animals
(2036 months of age), in our goat it appeared at an
older age (7 years). Pemphigus foliaceus does not seem
to have an age predilection in other species,7 and occurrence at a young age in the previously reported cases
may be a coincidence.
The lack of pruritus, a common finding in the other
cases,13 could have been masked by the injectable depot
corticosteroid that had been administered by the referring
veterinarian prior to our examination.
It is the first time that the cytological appearance of
an impression smear of pemphigus foliaceus is described
in the goat. Diagnosis of pemphigus foliaceus from
pustule content is based on the following findings:
presence of well-preserved neutrophils, absence of intracellular bacteria and presence of acantholytic cells.
The shape of the acantholytic cells observed cytologically
and histologically in our goat was different from that
observed in other animals. The acantholytic cells in
our case looked irregularly shaped, polygonal, less
basophilic and more differentiated, compared with
the rounded basophilic keratinocytes observed in
domestic carnivores and horses.810 It is suggested that
the acantholytic process in goats may occur at a more
superficial location in the epidermis, yielding more
differentiated acantholytic cells. However, this hypothesis is in contrast to the immunohistochemical results,
showing IgG deposition at all suprabasal epidermal
layers.
This is the first time that an immunohistochemical
technique has been reported for the diagnosis of pemphigus foliaceus in goats. Because of the lack of typical
acantholytic cells, immunohistochemistry appeared to
be a useful tool to confirm the diagnosis. In fact, IgG
deposition was clearly documented in the intercellular
spaces of the suprabasal layer of the epidermis.
Previous successful treatments described in caprine
pemphigus foliaceus include intramuscular prednisolone,3
intravenous and intramuscular dexamethasone,2 intramuscular triamcinolone2 and topical corticosteroid
ointment.1 Gold salts were useful in one case2 and
unsuccessful in another.3 The good response to steroid
treatment in our case confirms previous observations.
However, it may be that chronic depot dexamethasone
administration might have precipitated a bacterial mastitis,
to which our goat was predisposed. The administration
of other nonsteroidal immunosuppressive drugs

Pemphigus foliaceus in a goat

for maintenance therapy would be desirable for future
cases.
Finally, our goat had a concurrent louse infestation,
caused by Damalinia caprae. This parasite is usually
observed in debilitated animals, and generally does not
occur in healthy goats.11 It is not known how long the
goat had harboured the parasites, and if their infestation had occurred due to the recurrent mastitis or
because of the autoimmune disease. The pediculosis
seemed to be asymptomatic in this animal, and it was
easily eradicated with a standard antiparasitic treatment,
once the autoimmune skin condition was kept in
remission.

CONCLUSION
Pemphigus foliaceus is a rare autoimmune disease in
the goat, but it has features similar to the canine and
feline disease. Lesions are represented by pustules,
scales and patchy alopecia. Pustules contain irregularly
shaped acantholytic cells and intact neutrophils, and
immunohistochemical stains reveal IgG deposition
in the intercellular spaces. Pemphigus foliaceus in
the goat appears to respond well to corticosteroid
treatment.

ACKNOWLEDGEMENTS
The authors would like to thank Dr Giuseppe Barbieri
for referring this case and Dr Paola Roccabianca
for providing histological skin sections of a healthy
goat.

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REFERENCES
1. Jackson, P.G.G., Lloyd, S., Jefferies, A.R. Pemphigus
foliaceus in a goat. Veterinary Record 1984; 114: 479.
2. Scott, D.W., Smith, M.C., Smith, C.A. et al. Pemphigus
foliaceus in a goat. Agri-Practice 1984; 5: 3845.
3. Valdez, R.A., Gelberg, H.B., Morin, D.E. et al. Use of
corticosteroids and aurothioglucose in a pigmy goat with
pemphigus foliaceus. Journal of the American Veterinary
Medical Association 1995; 207: 7615.
4. Scott, D.W., Manning, T.O., Smith, C.A. et al. Pemphigus and pemphigoid in dogs, cats and horses. Annals of
the New York Academy of Science 1983; 420: 35360.
5. Kolou, L., Kusumi, A., Steinberg, M.S. et al. Human
autoantibodies against a desmosomal core protein in
pemphigus foliaceus. Journal of Experimental Medicine
1984; 160: 150918.
6. Suter, M.M., Ziegra, C.J., Cayatte, S.M. et al. Identification of canine pemphigus antigens. In: Ihrke, P.J.,
Mason, I.S., White, S.D., eds. Advances in Veterinary
Dermatology, Vol. II. Oxford: Pergamon Press, 1993:
36780.
7. Scott, D.W., Miller, W.H. Jr., Griffin, C.E. Small Animal
Dermatology, 6th edn. Philadelphia: W.B. Saunders,
2001.
8. Rosenkrantz, W.S. Pemphigus foliaceus. In: Griffin,
C.E., Kwochka, K.W., MacDonald, J.M., eds. Current
Veterinary Dermatology. St. Louis, MO: Mosby Year
Book, 1993: 1418.
9. Yager, J.A., Wilcock, B.P. Color Atlas and Text of Surgical Pathology of the Dog and Cat. London: Mosby Year
Book Europe, 1994: 1656.
10. Scott, D.W. Large Animal Dermatology. Philadelphia:
W.B. Saunders, 1988: 30611.
11. Smith, M.C. Dematologic diseases of goats. Veterinary
Clinics of North America: Large Animal Practice 1983; 5:
44955.

Rsum Une chvre ge de 7 ans est rfre pour une dermatite chronique, voluant depuis 3 mois, qui na
rpondu que partiellement un traitement antibiotique et corticode. A lexamen dermatologique, une alopcie
diffuse, des pustules et des crotes taient nots sur la tte, le cou, le dos et la region prianale. Une culture
fongique et des raclages cutanes taient ngatifs. Le trichogramme a montr une infestation par Damalinia
caprae, qui a t efficacement traite avec livermectine. Lexamen cytologique de pustules intactes a montr des
polynuclaires neutrophiles non dgnrs, labsence de bactrie et la presence de kratinocytes nucls nombreux
de taille irrgulire. Lexamen histopathologique de biopsies cutanes a montr des pustules intracornes,
contenant des neutrophiles et des cellules acantholytiques, et une dermatite privasculaire superficielle bigarre.
Des marquages immunohistochimiques ont montr un dept dIgG intercellulaire dans la couche pineuse.
Lutilisation de dexamethasone-21-isonicotinate injectable, tous les 2 mois pendant un an a permis la rmission
de la dermatose. Il sagit de la premire description de laspect cytologique du pemphigus foliac chez la chvre,
avec dmonstration de la prsence de depts dIgG par immunohistochimie dans la peau lse.
Resumen Una cabra hembra de 7 aos fue remitida con una historia clnica de 3 meses de dermatitis crnica
que respondi parcialmente a una terapia combinada con corticoides y antibiticos. El examen dermatolgico
mostr alopecia, pstulas y costras en la cabeza, rea dorsal del cuello y tronco, y rea perianal. El cultivo para
dermatofitos y los raspados cutneos fueron negativos. El tricograma revel una infestacin concomitante con
Damalinia caprae, que respondi a una terapia con ivermectina. El examen citolgico del pus de pstulas intactas
revel neutrfilos no-degenerados, ausencia de bacterias y numerosos queratinocitos nucleados de forma irregular. La histopatologa mostr pstulas intracorneales con neutrfilos y clulas acantolticas, y una dermatitis
perivascular superficial mixta. Las tinciones inmunohistoqumicas de piel lesionada mostraron depsitos
intercelulares de IgG en la capa espinosa. Se consigui la remisin de la dermatitis mediante inoculacin de
dexametasona-21-isonicotinato, cada dos meses durante un ao. Esta es la primera descripcin de las caractersticas citolgicas mediante impronta a partir de pstulas de pnfigo foliceo en una cabra, y de la presencia de
depsitos de IgG en piel lesionada mediante inmunohistoqumica.
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E. Pappalardo et al.
Zusammenfassung Eine siebenjhrige Ziege wurde nach siebenmonatiger chronischer Dermatitis, die partiell
auf eine Kombination von Kortikosteroiden und Antibiotika ansprach, vorgestellt. Eine dermatologische
Untersuchung ergab diffuse Alopezie, Pusteln und Krusten am Kopf, Nacken, Rcken und in der Perianalgegend.
Pilzkultur und Hautgeschabsel waren negativ. Trichoskopie ergab einen Befall mit Damalinia caprae, der mit
Ivermektin erfolgreich behandelt wurde. Zytologische Untersuchung intakter Pusteln ergab nicht-degenerierte
neutrophile Granulozyten und zahlreiche kernhaltige Keratinozyten unterschiedlicher Form, Bakterien waren
nicht vorhanden. Histopathologische Untersuchung von betroffener Haut ergab intrakorneale Pusteln, die neutrophile Granulozyten und akantholytische Zellen enthielten, sowie eine gemischte, oberflchliche, perivaskulre
Dermatitis. Immunhistochemische Frbung von betroffener Haut zeigte interzellulre IgG Ablagerung in der
Stachelzellschicht. Remission der Dermatitis wurde mit Dexamethason-21-Isonikotinsureinjektionen erzielt,
die alle zwei Monate fr ein Jahr gegeben wurden. Dies ist der erste Bericht, der Abklatschprparate von durch
Pemphigus foliaceus hervorgerufenen intakten Pusteln bei einer Ziege beschreibt und durch Immunhistochemie
festgestellte IgG Ablagerungen in betroffener Haut dokumentiert.

2002 Blackwell Science Ltd, Veterinary Dermatology, 13, 331 336