human orf (ecthyma contagiosum, contagious pustular dermatosis) m 72, farmer, sheep handler, left...

7/31/2019 Human Orf (Ecthyma Contagiosum, Contagious Pustular Dermatosis) M 72, Farmer, Sheep Handler, Left Wrist

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Human orf (Ecthyma contagiosum, Contagious pustular dermatosis)M 72,

farmer, sheep handler, left wrist

Deba P Sarma, MD, Omaha

M 72, farmer, sheep handler, left wrist crusted nodule, 0.9 cm

Diagnosis:

Human orf (Ecthyma contagiosum, Contagious pustular dermatosis)

Comment:

Ecthyma contagiosum is a zoonotic disease caused by the parapoxvirus that causes sore mouth in sheep and goats and orf in

human.

A 61-year-old sheep farmer presented with a painful non-pruritic lesion on the left hand that had been present for approximately 5

weeks. Physical examination demonstrated a 1 cm pearly, umbilicated papule with raised borders.

A biopsy showed an asymmetrical nodule with parakeratotic crust and acanthosis with thin epidermal strands extending deeply in

the underlying dermis. Marked edema, capillary proliferation and extensive lymphocytic infiltration was also present. One red

intranuclear inclusion was identified in an epidermal keratinocyte. A diagnosis of human orf (ecthyma contagiosum) was made.

Conclusion. Infected sheep and freshly vaccinated sheep or goats are the reservoir for human infection. After an incubation period

of 37 days, parapoxvirus infections produce 13 painful lesions measuring 1-2 cm in diameter. The natural history of the disease

is complete resolution and no treatment is indicated. Prevention of echthyma contagiosum in ruminants through vaccination is

thought to be the best way to control infection.

Ref:

Sarma DP, Cox M, Walter P, Crisler W, Huerter C (2010). A man with an umbilicated papule of the hand: what is your

diagnosis?. Case Reports in Medicine 2010;524021. PMID 20585366 [Pubmed- indexed for MEDLINE].

Case Reports in MedicineVolume 2010 (2010), Article ID 524021, 3 pagesdoi:10.1155/2010/524021
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Case Report

A Man with an Umbilicated Papule of the Hand: What Is Your Diagnosis?

Deba P. Sarma,1Meredith Cox,1Paige Walter,2William Crisler,2 andChristopher Huerter2

1Department of Pathology, Creighton University Medical Center, Omaha, NE 68131, USA2Department of Dermatology, Creighton University Medical Center, Omaha, NE 68131, USA

Received 9 March 2010; Accepted 2 April 2010

Academic Editor: Stephen A. Klotz

Copyright 2010 Deba P. Sarma et al. This is an open access article distributed under theCreative Commons

Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the

original work is properly cited.

Abstract

Introduction. Ecthyma contagiosum is a zoonotic disease caused by the parapoxvirus that causes sore mouth in

sheep and goats and orf in human. Case Presentation. A 61-year-old sheep farmer presented with a painful non-

pruritic lesion on the left hand that had been present for approximately 5 weeks. Physical examination

demonstrated a 1cm pearly, umbilicated papule with raised borders. A biopsy showed an asymmetrical nodule withparakeratotic crust and acanthosis with thin epidermal strands extending deeply in the underlying dermis. Marked

edema, capillary proliferation and extensive lymphocytic infiltration was also present. One red intranuclear

inclusion was identified in an epidermal keratinocyte. A diagnosis of human orf (ecthyma contagiosum) was made.

Conclusion. Infected sheep and freshly vaccinated sheep or goats are the reservoir for human infection. After an

incubation period of 37 days, parapoxvirus infections produce 13 painful lesions measuring 1-2cm in diameter.The natural history of the disease is complete resolution and no treatment is indicated. Prevention of echthyma

contagiosum in ruminants through vaccination is thought to be the best way to control infection.

1. Case Synopsis

A 61-year-old sheep farmer presented with a painful nonpruritic on the left hand that lesion had been present for

approximately 5 weeks. He neither had previous history of similar lesions nor did he have a history of cancer or

other dermatologic conditions. Physical examination demonstrated a 1 cm pearly, umbilicated papule with raisedborders (Figure1).

Figure 1: Clinical picture.

A biopsy (Figure2) showed an asymmetrical nodule with parakeratotic crust and acanthosis with thin epidermal

strands extending deeply in the underlying dermis. The upper dermis showed marked edema and capillary

proliferation. The deeper dermal part of the lesion showed extensive lymphocytic infiltration. One red intranuclearinclusion was identified in an epidermal keratinocyte (Figure3).
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Figure 2: Microscopic appearance: raised papule with parakeratotic crust and acanthosis with thin epidermal

strands extending deeply in the underlying dermis. The upper dermis is markedly edematous with marked capillary

proliferation. The deeper dermal part of the lesion is composed of reactive lymphoid infiltrates.

Figure 3: One red intranuclear inclusion in the keratinocyte of the epidermis.

2. What Is Your Diagnosis?

2.1. Diagnosis: Human Orf (Ecthyma Contagiosum)

Case Synopsis

A 61-year-old sheep farmer presented with a painful nonpruritic ulcerated lesion on the left hand that had been

present for approximately 5 weeks. He neither had previous history of similar lesions nor did he have a history of

cancer or other dermatologic conditions. Physical examination demonstrated a 1 cm pearly, umbilicated papule withraised borders (Figure1).

A biopsy (Figure2) showed an asymmetrical nodule with parakeratotic crust and acanthosis with thin epidermalstrands extending deeply in the underlying dermis. The upper dermis showed marked edema and capillary

proliferation. The deeper dermal part of the lesion showed extensive lymphocytic infiltration. One red intranuclear

inclusion was identified in the epidermal keratinocyte (Figure3).

3. Discussion

Our patient was a sheep farmer who presented with a raised umbilicated pearly lesion of his left hand that was

ulcerated with raised borders. Clinically, it could easily be mistaken for a basal cell carcinoma or squamous cell

carcinoma. The central umbilication could also suggest keratoacanthoma or a lesion caused by molluscum infection

though such lesions are usually much smaller. Milkers nodules should be considered too. Both the clinical history of

sheep farming and the microscopic features including the eosinophilic intranuclear inclusion body in the

keratinocyte suggest a diagnosis of human orf (ecthyma contagiosum). The diagnosis may be further confirmed by

electron microscopy done on the fluid obtained from the orf lesion showing ovoid cross-hatched virions [1].Polymerase chain reaction (PCR), although not readily available, can definitely identify orf virus from frozen tissue

specimens, vesicle material, or scab debris from orf lesions [2].

Ecthyma contagiosum is a zoonotic disease caused by the parapoxvirus that causes sore mouth in sheep and

goats and orf in human. In ruminants, it is evidenced by exudative lesions found on the muzzle, eyelids, oral

cavity, feet, or external genitalia. It is more common in younger animals. The disease in ruminants is highly

contagious to humans and other animals; infected sheep and also freshly vaccinated sheep or goats are the source

of infection to people. Transmission can be by direct contact with lesions or indirectly from contaminated object

such as hair or clothing [3].

Parapoxvirus is made up of a dense DNA core surrounded by a less dense capsid and 2 narrow electron dense

outer layers. After an incubation period of 37 days, parapoxvirus infections produce 13 painful lesions measuring

1-2cm in diameter. During the next 68 weeks, the lesion passes through 6 clinical stages: maculopapular, target,acute weeping, nodular, papillomatous, and finally regressive stages [4].

Microscopically, in the maculopapular stage, there is vacuolization of cells in the upper third of the stratum

malpighii leading to multilocular vesicles. Eosinophilic intranuclear or cytoplasmic inclusion bodies can be seen.

Vacuolated epidermal cells with inclusion bodies characterize the target stage. Ballooning degeneration also occurs

in the target stage and affects keratinocytes rupture with a tendency to coalesce and produce reticulated vesicles.

Additionally, in the epidermis, there is an elongation of the rete ridges. Many newly formed dilated capillaries and a

mononuclear infiltrate are present in the dermis. This is followed by the acute weeping stage which is characterized

by necrosis and a massive infiltrate of mononuclear cells throughout the dermis. Some biopsies of orf may have a

marked reactive lymphoid infiltrate with CD30 positive T cells mimicking lymphoma. On progression to the nodular
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stage, a lichenoid reaction with a high percentage of histiocytes is seen in the skin. In the final papillomatous stage

finger-like downward projections are displayed in the epidermis along with vasodilatation and chronic inflammation

in the dermis. This results in resolution and regression of the lesion [5].

The natural history of the disease is complete resolution and no treatment is indicated. But antiseptic agents to

prevent the superinfection and in some selected cases imiquimod can be applied [6]. Although, immunity is short

lived, reinfection frequently appears but no human-to-human transmission occurs. Investigation into prevention of

ecthyma contagiosum in ruminants through vaccination is thought to be the best way to control infection. Vaccinesare available that offer some efficacy in sheep but do not prevent disease in goats. Research into effective and

economical vaccines is ongoing. If infection is controlled in the ruminant population, human infection and its

economic and environmental consequences should decrease [5,7].

References

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170, 1985.

2. E. G. Torfason and S. Gunadottir, Polymerase chain reaction for laboratory diagnosis of orf virusinfections,Journal of Clinical Virology, vol. 24, no. 1-2, pp. 7984, 2002.View at PublisherView at

Google Scholar

3. UCD Occupational Health Program, Species Specific Guide Care and Use of Goats, October 2007,http://safetyservices.ucdavis.edu/occupational-health-services/acu/educational-materials/zoonosis-

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4. D. E. Elder, Histopathology of the Skin, Lippincott Williams and Wilkins, Philadelphia, Pa, USA, 10thedition, 2009.

5. M. Hosamani, A. Scagliarini, V. Bhanuprakash, C. J. McInnes, and R. K. Singh, Orf: an update on currentresearch and future perspectives,Expert Review of Anti-Infective Therapy, vol. 7, no. 7, pp. 879893,

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