ARTICLE
Auteur(s) : F Ballanger1, S
Barbarot1, C Mollat2, C
Bossard3, E Cassagnau3, F Renac1,
JF Stalder1
1Clinique dermatologique, CHU Hôtel Dieu, 44 093
Nantes, France
2Laboratoire de virologie, CHU Hôtel Dieu, Nantes
3Service d’anatomie pathologique A, CHU Hôtel Dieu,
Nantes
accepté le 2 Octobre 2005
Orf is a disease of sheep and goats caused by parapoxvirus. Cases
have also been reported among people in close contact with infected
animals. Immunocompromised transplant patients are prone to
opportunist infection by viruses such as Herpes simplex virus,
cytomegalovirus and varicella-zona virus. However, very few cases
of orf infection have been described in this population.This report
concerns an uncommon case of two very large exophytic lesions in an
immunocompromised patient treated with tacrolimus, mycophenolate
mofetil and prednisone.
Case report
In December 2003, a 31-year-old man was referred for investigation
and management of two red/purple exophytic cutaneous lesions. The
first had appeared on his right hand two weeks previously and had
gradually increased in size. The second lesion had appeared on the
left cheek. He was an animal breeder whose work brought him into
contact with sheep and rabbits.
The patient had a history of cystic fibrosis and had undergone a
heart and lung transplantation in 1992. Since that time, he had
been receiving tacrolimus 24 mg/d, mycophenylate mofetil 3 g/d and
prednisone 20 mg/d.
Clinical examination revealed painful red/purple nodules
surrounded by inflammatory reactions. Both lesions took the form of
botriomycoma and had grown rapidly to 70 × 50 × 40 mm and 35 ×
25 mm (figures 1A
and B). The patient had no general complaints, no fever,
and was free of lymphadenopathy. Biological examinations including
neutrophil count, lymphocyte count, and CRP were normal. Bacterial
cultures were negative.
Histopathology of skin biopsy samples showed an acanthotic
epidermis with some ballooning keratinocytes and eosinophilic
inclusions. The dermis exhibited areas of oedematous granulation
tissue and abundant thin-walled blood vessels (figures 2A and B).
The clinical examination was very suggestive of orf infection,
and the diagnosis was confirmed by electron microscopic
demonstration of the virus (figure 3).
Because of the large size and rapid growth of the lesions, total
excision was undertaken 24 days after the disease appeared. No
signs of recurrence were observed 14 months after surgery.
Discussion
This is the first reported case of orf in a transplanted patient
treated with oral tacrolimus. Orf (also known as contagious
pustular dermatitis and ecthyma contagiosum of the sheep) is an
infectious disorder caused by the parapox virus. It affects sheep,
goats and, particularly, young lambs. In animals, the cutaneous
lesions manifest as papulovesicular eruptions in the nose or groin.
Orf was first described in man by Newson and Cross in 1934 [1].
The virus is transmitted to humans by direct or indirect contact
[2], and most cases are seen in relatively well-defined ‘at-risk’
populations, such as veterinary surgeons, shepherds and abattoir
workers. However, orf is not only an occupational disease: during
the Muslim celebration of Aid el Kebir, religious customs may cause
endemic-type spread in the spring [3].
The clinical appearance of orf is usually characterised by
solitary cutaneous lesions at contact sites, particularly the hands
(79%) and arms (48%); lesions on the face are less common (11%)
[4]. Its course usually begins with a 3-14-day incubation period,
after which a painless red-blue papule 1-2 cm in diameter
appears. This develops into a haemorrhagic blister or pustule that
later becomes ulcerated, with scab formation. In
non-immunocompromised individuals, spontaneous resolution can be
expected in about 3 weeks.
Immunocompromised patients often exhibit lesions that are
atypical because of their large size or multifocal nature [5-7].
Pyogenic granuloma-like lesions have also been described, as have
forms of bullous lesion, and papulovesicular eruptions or satellite
lesions [4]. In immunocompromised patients, lesions also continue
to grow rather than regressing spontaneously.
Orf is diagnosed by history and clinical examination, and
confirmed by electron microscopy. There are no established
guidelines for treatment, but the priorities are to prevent
secondary bacterial infection and reassure the patient about the
benign nature of the complaint. Medical treatment of
immunocompromised patients is often frustrating, but there is one
report of the successful use of cidofovir cream [8]. Most patients
require surgery, and the high risk of frequent local recurrences
after excision [9] has led to combined medical/surgical approaches
involving idoxuridine 40% [10], interferon therapy, and cryotherapy
[11].
The observations presented here are original in the following
respects:
- – Few cases of orf have been reported in patients
receiving immunosuppressive treatment [12]. This is the first
reported case in an immunocompromised individual treated with oral
tacrolimus as an alternative to cyclosporine.
- – Very large orf lesions are known to occur in
immunocompromised patients, but the present case is unusual because
of the range of atypical characteristics: large size, multifocal
appearance, and localization on the face – a rarely reported
phenomenon [13, 14]. Furthermore, botriomycoma is a very uncommon
manifestation of the infection [15].
- – The efficacy of early surgery as initial treatment
with no relapse after 14 months is unexpected. The few cases of
relapse in the literature involve patients given cyclosporine.
Indeed, cyclosporine appears to promote relapse by inhibiting
recruitment of B- and T-cells [6, 16], which is not true of
tacrolimus. The present findings suggest that early excision may
reduce the risk of relapse in immunocompromised patients with very
large orf lesions.
References
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