Giant orf on the nose

ARTICLE

Case report

A 9-year-old boy was admitted to the dermatology department outpatient clinic because of a giant tumoral mass on the nose. A careful history revealed that this lesion had appeared 3 weeks before as a small papule that developed later to form a bleeding and crusted tumoral mass. It was unresponsive to antibiotics. The boy lived in a village and his family kept lambs, which he sometimes fed.

Examination revealed a giant, bleeding mass on the nose. The tumor was firm and painful and bled easily but was not accompanied by fever or other symptoms. It measured 5 cm by 4 cm and was attached to the right ala nasi by a base 2 cm in diameter (Fig. 1). It completely covered the nose and right nostril but there was no evidence of involvement of the inner mucosa. Serosanguinous material exudate was crusted on the lesion. Physical examination was unremarkable.

Gram stains of swabs from the lesion showed no bacteria. An excisional biopsy specimen was taken from the edge of the pedunculated mass. Histology showed acanthosis, spongiosis and pseudoepitheliomatous hyperplasia (Fig. 2). Some epidermis cells contained eosinophilic inclusion bodies (Fig. 3). The underlying dermis showed marked vascular hyperplasia and dilatation. In the areas of endothelial cells, hyperchromatic cell nuclei undergoing mitosis were observed. Electron microscopic examination of a paraffin-embedded biopsy specimen demonstrated cylindrical viral particles between the keratinocytes with a dense core and two less dense outer layers consistent with the parapox virus (Fig. 4).

Our first clinical impression on clinical diagnosis was giant pyogenic granuloma or malignancy. The lesion's unusual location and size led to initial uncertainty in the diagnosis. The patient's history of feeding lambs, the viral particles in the biopsy specimen and the clinical features suggested orf. Diagnosis was confirmed by electron microscopy.

The patient was seen at weekly intervals. The lesion was managed conservatively and no specific therapy was undertaken other than local wound care. Within one week, the oozing and bleeding had decreased. One week later, the patient presented a smaller, dried and crusted lesion. The crust was removed easily and the free edge of the lesion appeared as pinkish skin. Over the next 2 weeks, the mass evolved to a papillomatous stage and subsequently decreased in size (Fig. 5). Two months later, the skin had slight macular erythema, and it healed with minimal scarring (Fig. 6). The entire cycle lasted about 3 months.

Discussion

Orf (also called: ecthyma contagiosum, scabby mouth, sore mouth, contagious pustular dermatitis, contagious pustular stomatitis, and contagious bovine ecthyma) is an infectious mucocutaneous disease of sheep and goats caused by a virus of the subgroup parapox of the poxvirus that is transmissible to man. In lambs, it is characterized by a vesiculopapular eruption affecting chiefly the mouth, nose, udder and feet. Transmission to man usually occurs by direct contact with an infected animal or, less often, indirectly through objects [1-3].

The prevalence of human orf infection is underestimated. This may be because orf is a common, self-limiting disease that is recognized by the population at risk; therefore, medical care is not sought and many infections are not reported. It is considered to be an occupational disease in farmers, shepherds, veterinarians and abattoir workers [3-5]. However, religious habits may also be a source of contamination. Every year, an outbreak of orf is observed in Turkey, occurring 2 or 3 weeks after the Feast of Sacrifice. During this religious feast, each Muslim family sacrifies a sheep or cow, and viral contamination from an infected lamb may occur easily. Gunes described an orf epidemic in Izmir, Turkey, after a Feast of Sacrifice [6, 7].

In man, the disease is manifested as a solitary papular skin lesion on exposed body areas. Usually there is a single lesion located on a finger or other part of the hand, with other sites such as the face only occasionally being involved [8, 9], although multiple lesions on various body parts have also been reported [2, 3, 10, 11]. An orf lesion on the tip of the nose has been reported but it was not giant [12].

The orf lesion usually appears after an incubation period of less than four weeks. Spontaneous resolution often occurs within six weeks. The disease progresses through six distinct clinical and histopathologic stages: maculopapular, target, acute, regenerative, papillomatous, and regressive. The maculopapular stage consists of an erythematous macule or papule. In the target stage, the lesion has a red center, a central white ring, and an outer red halo. The acute stage consists of an erythematous weeping nodule. In the regenerative stage the lesion is dry with small black dots on the outside surface. The papillomatous stage is characterized by papillomas appearing on the surface. A dry crust characterizes the regressive stage. Residual scarring is unusual [1, 3, 13].

Only rarely, systemic symptoms or complications occur. The most commonly reported complications of orf are fever, superinfection, erysipelas, lymphadenitis, ocular damage, and erythema multiforme [2, 3, 9, 13].

Orf is a self-limiting disease in immunocompetent patients and no specific treatment is required. Immunocompromised patients, however, can develop very large and atypical orf lesions which do not always regress spontaneously and may recur [14-16]. Granulomatous giant lesions in normal individuals similar to that of our patient have been reported [17] and healed. Our patient did not present the typical features of orf and was suspected of having pyogenic granuloma and malignancy. There was no clinical evidence of general ill-health nor any reason to suppose that he was immunologically compromised.

Antibiotics are widely used against possible bacterial infection but they do not affect the course of the disease [3]. Cryotherapy [18], surgical excision, interferons [15], and 40% topical idoxuridine [15, 19] have been reported in the treatment of orf.

We are of the opinion that human infection of orf will continue to occur and all physicians should remain aware that infection may occur anywhere and consider it in the differential diagnosis of cases with relevant animal exposure. Although orf is generally a trivial and self-limiting process, it is important that it be diagnosed correctly because inappropriate treatment may cause long-term disability or a scar [11, 12].

Article accepted on 17/9/01

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