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160 DISC
Veterinary Dermatology 2000, 11, 6973

Case report
Linear organoid nevus in a dog
FABIA SCARAMPELLA,* CLAUDIA VON TSCHARNER{ and CHIARA NOLI*
*Via Sismondi 62, 20133 Milano, Italy
{Institut fur Tierpathologie, Langgasstr. 122, 3012 Bern, Switzerland
(Received 16 March 1998; accepted 5 February 1999)

Abstract A skin lesion classied as linear organoid nevus is reported in a female standard Schnauzer. The
dog was brought to the clinic with multiple hyperpigmented, hyperkeratotic linear or ovoid plaques on the
head, neck, trunk, ears, and limbs. Histological ndings included severe orthokeratotic hyperkeratosis, focal
parakeratosis and hyperplasia of both the epidermis and the follicular infundibular epithelium, and marked
sebaceous hyperplasia. No improvement was noted with systemic retinoid therapy. This is the second linear
organoid nevus described in a dog, and the rst report of retinoid therapy for this disease.
Keywords: comedones, dog, linear organoid nevus, nevus, retinoids, sebaceous hyperplasia.

INTRODUCTION
Organoid nevi have been dened in people as
localized faults in development of skin and appendages, that appear at birth, usually on the scalp.1
These lesions, also improperly called `nevus sebaceous', are characterized by an abnormal arrangement
and hyper- or hypoplasia of two or more skin
components that are normally present at the site.1
Linear organoid nevi have been recognized and
studied in human dermatology,2 but only one case
has been reported in veterinary medicine.3 This article
describes another case of linear organoid nevus in a
dog, which has been followed for more than 20
months and treated with systemic retinoids.
CASE STUDY
A 10-month-old female standard Schnauzer was
presented with a history of multiple mildly pruritic
hyperkeratotic plaques, which appeared for the rst
time at 3 months. The lesions were ovoid or linear in
shape, and were present on the head, neck (Fig. 1),
trunk, ears, and limbs. The most impressive lesion was
a linear, 20-cm-long plaque, localized on the medial left
thigh (Fig. 2). All lesions were hyperpigmented,
hyperkeratotic and mildly alopecic. Dilated follicular
ostia were engorged with keratin (comedones), and in
some areas multiple keratin horns were extruding
from them. No signs of systemic disease were present.
A complete haematology and serum biochemical
analysis were within normal limits. Microscopy of

Correspondence and reprint requests: C. Noli.

# 2000 Blackwell Science Ltd

skin scrapings and fungal cultures for dermatophytes were negative. Skin biopsy specimens were
collected by means of a 6-mm biopsy punch, xed in
10% formalin and processed for routine histopathology. On histological examination (Figs 3
and 4) diuse basket-weave, focally compact orthokeratotic hyperkeratosis, focal parakeratosis, and
regular epidermal hyperplasia were observed. The
follicular infundibula were dilated with abundant
ortho- and parakeratotic hyperkeratosis, forming
several comedones. Marked hyperplasia of sebaceous glands, pigmentary incontinence and a mild
periadnexal mononuclear inammatory inltrate
were also observed. Due to hyperplasia and abnormal arrangement of at least two skin components
(hair follicles and sebaceous glands) the lesion was
classied as linear organoid nevus. No therapy was
prescribed at this time.
At a subsequent visit 3 months later, the owner
reported increasing pruritus, particularly of the
lesions on the left thigh. Macroscopically the lesions
appeared to be inamed and mildly exudative. Heatxed smears of follicular keratin were stained with a
rapid, modied Wright's stain (Hemacolor, Merck)
and examined microscopically. Many intra- and
extracellular cocci and abundant (more than eight
per high-power eld (HPF)) oval unipolar budding
yeasts, tentatively identied as Malassezia, were present. Because of the presence of bacteria and yeasts, the
dog was treated with amoxycillin and clavulanic acid
(20 mg kg71 twice daily orally) and ketoconazole (10
mg kg71 once daily orally) for 3 weeks. A 2.5%
benzoyl peroxide shampoo (Fatroxyd, Fatro) was
also prescribed once a week for 3 weeks. Good results
in controlling hyperkeratosis and almost complete
absence of pruritus were obtained with this treatment.
69

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70

F. Scarampella, C. von Tscharner and C. Noli

Figure 1. Focal, hyperpigmented, hyperkeratotic

lesion located on the lateral aspect of the neck.
Several feather-shaped keratin horns, arising from
the follicular ostia, are visible.

Figure 2. A 20-cm-long linear hyperkeratotic,

hyperpigmented alopecic lesion on the medial
left thigh. The knee is almost in the middle of the
photograph.

Figure 3. Histological appearance of the organoid

nevus at 10 months: epidermal hyperplasia with
basket-weave hyperkeratosis and dilation and
hyperkeratosis of hair follicle infundibula
(comedo
formation)
(light
microscope,
H&E,640).

When the dog reached adulthood (18 months of

age), systemic retinoid therapy was initiated. Retinoids were chosen because of their ecacy in the
treatment of some keratinization disorders4 and of
# 2000 Blackwell Science Ltd, Veterinary Dermatology, 11, 6973

some epidermal nevi5 in dogs. Etretinate was

administered at a dosage of 2 mg kg71 once daily
per os for 4 months. No other systemic or topical
therapy was administered. No side eects were

160 DISC
Linear organoid nevus in a dog

71

ever, 3-week courses of antibiotic therapy are

occasionally needed to control secondary bacterial
infection. The dog is now 30-months-old and the
lesions remain grossly unchanged.
DISCUSSION

Figure 4. Histological appearance of the lesion located on the

medial aspect of the left hindleg at 10 months: marked sebaceous
hyperplasia, dilated hyperkeratotic hair follicles, mild periadnexal
inammatory inltrate and mild pigmentary incontinence can be
observed (light microscope, H&E,640).

reported during the treatment period. Tear production measured via a Schirmer tear test monthly was
normal, and the plasma triglyceride level was in the
normal range at the end of the therapy. Unfortunately, no clinical improvement of the lesions was
observed during this period.
The lesions were sampled for histopathology again
at the end of the 4 months of retinoid therapy (and 12
months after the rst biopsies). At histopathologic
examination, the appearance of the lesions was the
same as in the previous sections, except for the
presence of a more severe mononuclear inammatory
inltrate around the hyperplastic sebaceous glands,
an increased epidermal papillomatosis, and a marked
hypergranulosis of the epidermis and the follicular
infundibulum (Fig. 5).
At 24 months of age the dog was given
isotretinoin at a dosage of 2 mg kg71 once daily
per os for 3 months. No side eects were reported
during the treatment period. The tear production
measured via the Schirmer tear test monthly was
normal and the plasma triglyceride level was in the
normal range at the end of the treatment period.
No improvement was obtained with this therapy.
Permission for further skin biopsies was refused by
the owner.
In order to prevent secondary infections and
comedo formation, the lesions are currently treated
with weekly 2.5% benzoyl peroxide shampoo. How-

We report here a case of linear organoid nevus in a

standard Schnauzer. A nevus is dened as `a
circumscribed developmental defect of the skin,
characterized by hyperplasia of one or more skin
components'.6 If the hyperplastic skin components
are two or more, then the nevus is called `organoid'.6
Organoid nevi have been reported very rarely in dogs.
Paradis et al. were the rst to report a linear organoid
nevus, in a female 2-month-old Old English Sheepdog.3 The lesions in this dog were observed on the
head, neck, shoulders and thorax, and had a yellow,
greasy and crusting appearance. Histologically, both
follicular and sebaceous hyperplasia were present.
The dog in our report shares a number of clinical and
histological features with the one described by
Paradis et al. The lesions in both dogs were linear
and hyperkeratotic, and were observed at a very
young age. Histologically, both cases had hyperplasia
of both hair follicles and sebaceous glands. However,
the lesions in our case were more hyperkeratotic,
featured more comedones and follicular plugging,
and were less greasy. Histologically, more prominent
infundibular distension and hyperkeratosis were
observed. Unfortunately, the dog described by
Paradis et al. was euthanized before any therapeutic
attempt was undertaken. Our case was followed for
more than 20 months, and several treatment options
were tried (see below).
Organoid nevi were described in humans more
than 100 years ago by Jadassohn, who called this
lesion `nevus sebaceous'.7 In 1965, Mehregan and
Pinkus collected 150 specimens of human organoid
nevi and determined three stages in the life history of
these lesions.2 The rst one, before puberty, is
characterized by hypoplasia of hair follicles and
sebaceous glands. The second phase, at puberty, leads
to important development of sebaceous and apocrine
glands, and to papillomatous epidermal hyperplasia.
The third stage, after puberty, is characterized, in
20% of the cases, by the development of benign or
malignant neoplasias at the original lesion site.
The dog in our report had lesions at 3 months of
age, but was referred to us only at puberty. Marked
sebaceous hyperplasia was observed at that time, but
we do not know if sebaceous hyperplasia was present
before, or developed at, puberty. In the skin biopsies
collected 12 months later, epidermal papillomatosis
and hypergranulosis were observed, which were not
present in the rst skin biopsies. This evolution is
similar to that observed in the second phase of human
organoid nevi. These alterations may also be caused
by chronic inammation, as periadnexal lymphocytes
# 2000 Blackwell Science Ltd, Veterinary Dermatology, 11, 6973

160 DISC
72

F. Scarampella, C. von Tscharner and C. Noli

Figure 5. Histological appearance of the lesion at

the same site as Fig. 4 at 22 months: there is
increased dermal mononuclear inammatory
inltrate,
pigmentary
incontinence
and
epidermal papillomatosis, when compared to the
samples taken 12 months previously (light
microscope, H&E,640).

were observed in the same biopsies, or may be the

eect of the retinoid therapy. The dog is now 30months-old, and no benign or malignant tumours
have been observed yet on the site of the lesions.
Multiple skin samples for histopathology before,
at, and after puberty should be taken, in order to
determine whether the lesions change during the
dog's life, as happens in humans. Unfortunately, the
owner of the dog in this case refused further biopsies.
In humans, extensive linear organoid nevi have been
repeatedly reported in association with internal
diseases, particularly those of the central nervous
system.8 No systemic or neurologic abnormalities were
found in our dog, nor in the one previously described.3
Linear lesions in humans often follow Blaschko
skin development lines, which do not follow any
vascular or neural structure in the skin.9 Blaschko
lines have not yet been denitively characterized in
dogs, and it is tempting to assume they might exist
in this species too. Linear lesions in genodermatoses, as in our case, have been previously
described.3,5,10
Bianchine,11 in 1970, recorded ndings in four
siblings whose father was mentally impaired, suggesting
a hereditary transmission of this disease. In our case no
other dogs with similar lesions were observed by the
breeder among siblings or relatives of this patient.
Retinoids have been successfully used in disorders
of cornication, diseases involving the sebaceous
glands and featuring comedones and follicular plugging.4 Isotretinoin and etretinate have also been used
with good results in three cases of inammatory
linear verrucous epidermal nevus in dogs.5 In our
case, however, no improvement was noticed with the
administration of etretinate for 4 months or with
isotretinoin for 3 months. This could depend on the
nature of the nevus, dierent from the one described
by White et al. or on the lower dosage. In our case,
we administered the retinoids at a dosage of 2 mg
kg71 once daily, whereas White et al. prescribed the
drugs at up to 9.72 mg kg71 once daily. It may be
that a higher dose could have been eective in our
# 2000 Blackwell Science Ltd, Veterinary Dermatology, 11, 6973

case. However, we chose not to increase the dosage in

our case, due to the cost of the drug and to its
possible side eects. Furthermore, the owners were
able to control the lesions with a 2.5% benzoyl
peroxide shampoo and occasional antibiotic treatment for secondary pyoderma.
REFERENCES
1. Steen, C., Ackermann, A.B. Neoplasms with
sebaceous dierentiation. In: Ackermann, A. B., ed.
Histologic Diagnosis of Neoplastic Skin Diseases: a
Method by Pattern Analysis Lea & Febiger,
Philadelphia, 1994: 89144.
2. Mehregan, A.H., Pinkus, H. Life history of organoid
nevi. Special reference to nevus sebaceous of
Jadassohn. Archives of Dermatology 1965; 91: 57488.
3. Paradis, M., Scott, D.W. Nevi recemment reconnus
chez le chien: Nevus comedonien, nevus organoide
lineaire et nevus du follicule pileux. Le Point
Veterinaire 1989; 21: 48993.
4. Kwochka, K.W. Retinoids and Vitamin A Therapy. In:
Grin, C. E., Kwochka, K.W., MacDonald, J.M.,
eds., Current Veterinary Dermatology. 1st edn. Mosby
Year Book, St. Louis, 1993: 20310.
5. White, S.D., Rosychuk, R.A.W., Scott, K.V., Hargis,
A.M., Trettien, A. Inammatory linear verrucous
epidermal nevus in four dogs. Veterinary Dermatology
1993; 3: 10713.
6. Scott, D.W., Miller, W.H., Grin, C.E. Muller and
Kirk's Small Animal Dermatology, 5th edn. W.B.
Saunders, Co., Philadelphia, 1995: 11029.
7. Jadassohn, J. Bemerkungen zur Histologie der
Systematisierten Nevi und uber `Talgdrusen-Nevi',
part II. Archiv Dermatologie Syphilopathologie 1895;
33: 355408.
8. Hashimoto, K., Lever, F. Tumors of skin appendages.
In: Fitzpatrick, T. B., Eisen, A. Z., Wol, K.,
Freedberg, I. M., Austen, K. F., eds. Dermatology in
General Medicine 3rd edn. McGraw-Hill Information
Services Company, New York, 1987: 787.
9. Fitzpatrick, T.B., Bernhard, J.D. The structure of skin
lesions and fundamental of diagnosis. In: Fitzpatrick,
T. B., Eisen, A. Z., Wol, K., Freedberg, I. M., Austen,

160 DISC
Linear organoid nevus in a dog
K. F., eds. Dermatology in General Medicine, 3rd edn.
McGraw-Hill Information Services Company, New
York, 1987: 38.
10. 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.

73

11. Bianchine, J.W. The nevus sebaceous (sic) of

Jadassohn: a neurocutaneous syndrome and a
potentially pre malignant lesion. American Journal of
Diseases of the Children 1970; 120: 2238.

Resume Une lesions cutanee classiee comme un naevus organo de lineaire est rapportee chez un Schnauzer
femelle. Le chien a ete presente a la consultation pour de multiples plaques, hyperpigmentees, epaisses,
lineaires ou ovo des, localisees sur la tete, le cou, le tronc, les oreilles et les membres. Les examens
histopathologiques ont montre une hyperkeratose orthokeratosique marquee, une parakeratose focale, une
hyperplasie de l'epiderme et de l'epithelium folliculaire, et une hyperplasie sebacee severe. Aucune
amelioration n'a ete observee avec un traitement a base de retino des systemiques. Il s'agit du second cas
de naevus organo de lineaire decrit chez le chien, et du premier rapport de l'utilisation des retino des pour cette
dermatose. [Scarampella, F., Von Tscharner, C. et Noli, C. (Un naevus organo de lineaire chez un chien.)
Veterinary Dermatology 2000; 11: 6973.]
Resumen Se describe una lesion clasicada como nevo organoide linear en un Schnauzer estandard hembra.
El perro fue llevado a la consulta con multiples placas lineares u ovoides, hiperpigmentadas e
hiperqueratoticas en la cabeza, cuello, tronco, pabellones auriculares y extremidades. Los hallazgos
histologicos inclu an hiperqueratosis ortoqueratotica intensa, paraqueratosis focal, hiperplasia de la epidermis
y del epitelio del infund bulo folicular e hiperplasia sebacea marcada. No se observo mejor a con terapia
sistemica de retinoides. Este es el segundo nevo linear organoide descrito en un perro, y la primera descripcion
de terapia con retinoides para esta enfermedad. [Scarampella, F., Von Tscharner, C. y Noli, C. (Nevo
organoide linear en un perro.) Veterinary Dermatology 2000; 11: 6973.]
Zusammenfassung Eine als linearer organoider Naevus klassizierte Hautlasion bei einem
Schnauzerweibchen wird beschrieben. Der Hund wurde mit mehreren, hyperpigmentierten,
hyperkeratotischen, linearen oder ovoiden Plaques auf Kopf, Nacken, Rumpf, Ohren und Beinen in der
Klinik vorgestellt. Histologische Befunde ergaben schwere orthokeratotische Hyperkeratose, fokale
Parakeratose, Hyperplasie der Epidermis und des follikularen Infundibulumepithels und ausgepragte
Talgdrusenhyperplasie. Systemische Retoinoidtherapie fuhrte nicht zur Besserung. Dies ist der zweite
Bericht eines linearen organoiden Naevus beim Hund und der erste Bericht der Retinoidtherapie.
[Scarampella, F., Von Tscharner, C. und Noli, C. (Linearer Organoider Naevus bei einem Hund.)
Veterinary Dermatology 2000; 11: 6973.]

# 2000 Blackwell Science Ltd, Veterinary Dermatology, 11, 6973