DR SARMA'S DERMPATH: A Man With an Umbilicated Papule of The Hand: What Is Your Diagnosis?

7/30/2019 DR SARMA'S DERMPATH: A Man With an Umbilicated Papule of The Hand: What Is Your Diagnosis?

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A Man with an Umbilicated Papule of the Hand:

What Is Your Diagnosis?

Deba P. Sarma, Meredith Cox, Paige Walter, William

Crisler, and Christopher Huerter

Creighton University Medical Center, Omaha


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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 farmerpresented with a painful non-pruritic lesion on the left hand thathad been present for approximately 5 weeks. Physicalexamination demonstrated a 1cm pearly, umbilicated papule withraised borders. A biopsy showed an asymmetrical nodule with

parakeratotic crust and acanthosis with thin epidermal strandsextending deeply in the underlying dermis. Marked edema,capillary proliferation and extensive lymphocytic infiltration wasalso present. One red intranuclear inclusion was identified in anepidermal keratinocyte. A diagnosis of human orf (ecthymacontagiosum) 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 infectionsproduce 13 painful lesions measuring 1-2cm in diameter. Thenatural history of the disease is complete resolution and notreatment is indicated. Prevention of echthyma contagiosum in

ruminants through vaccination is thought to be the best way tocontrol infection.


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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 didhe have a history of cancer or other dermatologic conditions.

Physical examination demonstrated a 1cm pearly, umbilicated

papule with raised borders (Figure 1).

Figure 1

Clinical

picture.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878684/figure/fig1/


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A biopsy (Figure 2) 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 intranuclear inclusion was

identified in an epidermal keratinocyte (Figure 3).

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. Thedee er dermal art of the lesion is com osed of reactive l m hoid infiltrates.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878684/figure/fig2/http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878684/figure/fig3/http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878684/figure/fig2/


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Figure 3

One red intranuclear inclusion in the

keratinocyte of the epidermis.


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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 forapproximately 5 weeks. He neither had previous history of similarlesions nor did he have a history of cancer or other dermatologicconditions. Physical examination demonstrated a 1cm pearly,umbilicated papule with raised borders (Figure 1).

A biopsy (Figure 2) showed an asymmetrical nodule withparakeratotic crust and acanthosis with thin epidermal strandsextending deeply in the underlying dermis. The upper dermisshowed marked edema and capillary proliferation. The deeper

dermal part of the lesion showed extensive lymphocyticinfiltration. One red intranuclear inclusion was identified in theepidermal keratinocyte (Figure 3).
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878684/figure/fig3/http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878684/figure/fig2/http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878684/figure/fig1/http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878684/figure/fig3/http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878684/figure/fig2/http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878684/figure/fig1/


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3. Discussion Our patient was a sheep farmer who presented with a raised

umbilicated pearly lesion of his left hand that was ulcerated withraised borders. Clinically, it could easily be mistaken for a basal

cell carcinoma or squamous cell carcinoma. The centralumbilication could also suggest keratoacanthoma or a lesioncaused by molluscum infection though such lesions are usuallymuch smaller. Milker's nodules should be considered too. Boththe clinical history of sheep farming and the microscopic features

including the eosinophilic intranuclear inclusion body in thekeratinocyte suggest a diagnosis of human orf (ecthymacontagiosum). The diagnosis may be further confirmed byelectron microscopy done on the fluid obtained from the orf lesionshowing ovoid cross-hatched virions [1]. Polymerase chainreaction (PCR), although not readily available, can definitely

identify orf virus from frozen tissue specimens, vesicle material, orscab debris from orf lesions [2].
http://www.ncbi.nlm.nih.gov/pubmed/11744431http://www.ncbi.nlm.nih.gov/pubmed/3883947http://www.ncbi.nlm.nih.gov/pubmed/11744431http://www.ncbi.nlm.nih.gov/pubmed/3883947


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Ecthyma contagiosum is a zoonotic disease caused by theparapoxvirus that causes sore mouth in sheep and goats and orfin human. In ruminants, it is evidenced by exudative lesions foundon the muzzle, eyelids, oral cavity, feet, or external genitalia. It ismore common in younger animals. The disease in ruminants ishighly contagious to humans and other animals; infected sheepand also freshly vaccinated sheep or goats are the source ofinfection to people. Transmission can be by direct contact withlesions 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. Afteran incubation period of 37 days, parapoxvirus infections produce13 painful lesions measuring 1-2cm in diameter. During the next68 weeks, the lesion passes through 6 clinical stages:maculopapular, target, acute weeping, nodular, papillomatous,and finally regressive stages [4].


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Microscopically, in the maculopapular stage, there isvacuolization of cells in the upper third of the stratum malpighiileading to multilocular vesicles. Eosinophilic intranuclear or

cytoplasmic inclusion bodies can be seen. Vacuolated epidermalcells with inclusion bodies characterize the target stage.Ballooning degeneration also occurs in the target stage andaffects keratinocytes rupture with a tendency to coalesce andproduce reticulated vesicles. Additionally, in the epidermis, thereis an elongation of the rete ridges. Many newly formed dilatedcapillaries and a mononuclear infiltrate are present in the dermis.This is followed by the acute weeping stage which ischaracterized by necrosis and a massive infiltrate ofmononuclear cells throughout the dermis. Some biopsies of orfmay have a marked reactive lymphoid infiltrate with CD30

positive T cells mimicking lymphoma. On progression to thenodular stage, a lichenoid reaction with a high percentage ofhistiocytes is seen in the skin. In the final papillomatous stagefinger-like downward projections are displayed in the epidermisalong with vasodilatation and chronic inflammation in the dermis.This results in resolution and regression of the lesion [5].
http://www.ncbi.nlm.nih.gov/pubmed/19735227


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The natural history of the disease is complete resolution and no

treatment is indicated. But antiseptic agents to prevent thesuperinfection and in some selected cases imiquimod can beapplied [6]. Although, immunity is short lived, reinfection frequentlyappears 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 controlinfection. Vaccines are available that offer some efficacy in sheepbut do not prevent disease in goats. Research into effective andeconomical vaccines is ongoing. If infection is controlled in theruminant population, human infection and its economic andenvironmental consequences should decrease [5, 7].
http://www.ncbi.nlm.nih.gov/pubmed/19735227http://www.ncbi.nlm.nih.gov/pubmed/18828697http://www.ncbi.nlm.nih.gov/pubmed/18828697http://www.ncbi.nlm.nih.gov/pubmed/19735227http://www.ncbi.nlm.nih.gov/pubmed/18191701


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References 1.Sanchez RL, Hebert A, Lucia H, Swedo J. Orf. A case report with

histologic, electron microscopic, and immunoperoxidase studies.Archives of Pathology and Laboratory Medicine. 1985;109(2):166

170. [PubMed] 2.Torfason EG, Gunadottir S. Polymerase chain reaction forlaboratory diagnosis of orf virus infections. Journal of ClinicalVirology. 2002;24(1-2):7984. [PubMed]

3.UCD Occupational Health Program. Species Specific Guide Careand Use of Goats. October 2007, http://safetyservices.ucdavis.

edu/occupational-health-services/acu/educational-materials/zoonosis-information. 4.Elder DE. Histopathology of the Skin. 10th edition. Philadelphia,

Pa, USA: Lippincott Williams and Wilkins; 2009. 5.Hosamani M, Scagliarini A, Bhanuprakash V, McInnes CJ, Singh

RK. Orf: an update on current research and future perspectives.Expert Review of Anti-Infective Therapy. 2009;7(7):879893.[PubMed]

6.Ara M, Zaballos P, Sanchez M, et al. Giant and recurrent orf virusinfection in a renal transplant recipient treated with imiquimod.Journal of the American Academy of Dermatology. 2008;58(2,supplement 1):S39S40. [PubMed]

7.Musser JMB, Taylor CA, Guo J, Tizard IR, Walker JW.Development of a contagious ecthyma vaccine for goats.American
http://www.ncbi.nlm.nih.gov/pubmed/18191701http://www.ncbi.nlm.nih.gov/pubmed/19735227http://www.ncbi.nlm.nih.gov/pubmed/19735227http://www.ncbi.nlm.nih.gov/pubmed/19735227http://safetyservices.ucdavis.edu/occupational-health-services/acu/educational-materials/zoonosis-informationhttp://safetyservices.ucdavis.edu/occupational-health-services/acu/educational-materials/zoonosis-informationhttp://safetyservices.ucdavis.edu/occupational-health-services/acu/educational-materials/zoonosis-informationhttp://safetyservices.ucdavis.edu/occupational-health-services/acu/educational-materials/zoonosis-informationhttp://safetyservices.ucdavis.edu/occupational-health-services/acu/educational-materials/zoonosis-informationhttp://safetyservices.ucdavis.edu/occupational-health-services/acu/educational-materials/zoonosis-informationhttp://safetyservices.ucdavis.edu/occupational-health-services/acu/educational-materials/zoonosis-informationhttp://www.ncbi.nlm.nih.gov/pubmed/11744431http://www.ncbi.nlm.nih.gov/pubmed/3883947http://www.ncbi.nlm.nih.gov/pubmed/18828697http://www.ncbi.nlm.nih.gov/pubmed/18191701http://www.ncbi.nlm.nih.gov/pubmed/19735227http://safetyservices.ucdavis.edu/occupational-health-services/acu/educational-materials/zoonosis-informationhttp://safetyservices.ucdavis.edu/occupational-health-services/acu/educational-materials/zoonosis-informationhttp://safetyservices.ucdavis.edu/occupational-health-services/acu/educational-materials/zoonosis-informationhttp://www.ncbi.nlm.nih.gov/pubmed/11744431http://www.ncbi.nlm.nih.gov/pubmed/3883947


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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].
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878684/?tool=pubmedhttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878684/?tool=pubmedhttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878684/?tool=pubmedhttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878684/?tool=pubmedhttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878684/?tool=pubmedhttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878684/?tool=pubmed

DR SARMA'S DERMPATH: A Man With an Umbilicated Papule of The Hand: What Is Your Diagnosis?

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