Unusual Recalcitrant Classic Kaposi’s sarcoma in a Non-AIDS Yemeni Man: Is It a Zoonotic!

I report a case of a 43-year-old non-HIV infected Yemeni shepherd, who presented with a fungating, multipigmented plaque of several, purplish/dark-brownish, friable nodules on the right index finger, cutaneous dissemination, and lymphatic infiltrates. Chronicity, number and morphology of the lesions, local lymphatic infiltration and the characteristic histopathological findings were identical with Kaposi’s sarcoma (KS); the classic variant. Due to rarity of non human immunodeficiency virus (HIV)-associated KS, here I report a case of classic KS in an immune competent middle-aged Asian male. I also raise a question about human KS-associated herpesvirus (KSHV) and its zoonotic potential for cross-species infection. *Corresponding author: Mohamed A. Al-Kamel, MD, Regional Leishmaniasis Control Center, P.O.Box 12692, Sana’a, Yemen. Tel: +967 777223535. E-mail: dralkamel@hotmail.com Citation: Al-Kamel, M.A. Unusual Recalcitrant Classic Kaposi’s Sarcoma in aNon-AIDSYemeni Man: Is It a Zoonotic! (2016) Invest Dermatol Venereol Res 2(3): 14. Unusual Recalcitrant Classic Kaposi’s sarcoma in a Non-AIDS Yemeni Man: Is It a Zoonotic! Al-Kamel Mohamed Ahmed1,2 Received date: Feb 27, 2016 Accepted date: June 03, 2016 Published date: June 09, 2016 DOI: 10.15436/2381-0858.16.796 Invest Dermatol Venereol Res | Volume 2: Issue 3 Al-Kamel, M.A


Introduction
easily bleed on injury, on the palmar aspect of his right index finger ( Figure. 1). The condition started 8 months as an erythematous macule that gradually developed to a bigger nodule forming a multifocal tumor. The condition worsen over the time and the lesions progressed to cover most of the finger. Afterwards, asymptomatic violaceous nodule developed under the left eyelid, as well. Two months ago, numerous, smaller, firm, deep-cutaneous 0.5 -1 cm nodules started to develop along the lymphatic drainage of the same finger, up to the left mid-forearm. No regional lymph nodes, oral or genital involvement.
Direct questioning regarding his occupational history revealed that he is a farmer and a sheep shepherd with animals living in the same household. He reported that some of his sheep have complained of unknown infectious skin lesions, in the form of erosions and blisters that spontaneously rupture spreading the infection to healthy ones.
Medical history revealed that the patient roughly received many courses of antibiotics, corticosteroids and other unknown treatment modalities.
Past history revealed, 16 months before (September 26 th , 2014), the patient presented with similar brownish-red nodules on both aspects of the neighboring right middle finger (but without lymphatic involvement or distal dissemination), of 6 months duration, that was treated and responded well to cryotherapy with complete regression without scarring ( Figure. 2). Two weeks later, he noticed similar lesions started to appear on the palmar aspect of the presenting right index finger, and thus the current condition started to develop. History of a previous hand trauma was irrelevant. Except for a mild eosinophilia, investigations revealed normal hemogram, HIV, HBV, HCV, LFT, KFT and internal imaging scans.
An excisional 2 x 1.5 cm biopsy was obtained. Pending the H&E stained specimen examination results, differential diagnosis included KS, Orf and Milker's nodules, bacillary angiomatosis, cutaneous lymphoma and leprosy nodule, was taken in consideration.
Histopathological examination ( Figure. 3) shows hyperkeratotic epidermis with focal parakeratosis and focal thinning in the middle part. In the dermis, there is a nodule of proliferation of small vascular spaces, some of the rounded, and others slit-like with some small bundles of spindle cells, red blood cells extravasation, is also seen, and hemosiderin and dilated vascular spaces in the periphery are occasionally seen. These findings suggest KS, with uncertain malignant potential. PCR and HHV-8 testing were not carried out.  On basis of the clinical and characteristic histopathological findings, a definite diagnosis of Kaposi's sarcoma was made, and the patient was referred to a general hospital for further management.

Discussion
Kaposi's sarcoma (KS) is a soft tissue cancer; characterized histologically by endothelial-lined vascular spaces and spindle-shaped cells. It can present with cutaneous lesions with or without internal involvement. The cutaneous lesions can be solitary, localized or disseminated. The erythematous to violaceous cutaneous lesions seen in KS have several morphologies; patch, plaque, nodular, lymphadenopathic, infiltrative, and florid. Typically, the disease is multifocal and recurs despite treatment. The diagnosis can be made with a tissue biopsy and, if clinically indicated, internal imaging should be done [3,8] .
Endemic KS has been originally described in young African people, mainly from sub-Saharan Africa, however it may be present worldwide. This variant often affects human immunodeficiency virus (HIV)-negative individuals, including children, and can take an aggressive form involving the lymph nodes [9] .
Iatrogenic KS is a type associated with immunosuppressive drug therapy, typically seen in renal allograft recipients. This variant tends to be aggressive, involving lymph nodes, mucosa, and visceral organs in about half of patients, sometimes in the absence of skin lesions [7] .
Epidemic KS (AIDS-associated) typically presents with cutaneous lesions, with a predilection for the head, neck, back, trunk and mucous membranes. In more advanced cases, they can be found in the stomach and intestines, the lymph nodes, and the lungs [8] .
Classic Kaposi sarcoma (CKS) was described in 1872 by Moriz Kaposi, as an idiopathic, multipigmented sarcoma of the skin. Itis a rarevariant, typically presents much more common in men, tends to be indolent and chronic, and though in the majority of cases it is benign.
CKS typically appears initially on the hands and feet as reddish, violaceous, or bluish-black well-demarcated, painless macules and patches that progress slowly and may merge into nodules, plaques or fungi-formed, brownish-red tumors. The latter are characterized mostly by spindle-shaped tumor cells that are compressed with vascular slits, often in association with extravasated erythrocytes, hemosiderin and fibrosis. Unilateral involvement generally is observed at the onset of KS; however, subsequently the disease tends to be bilateral and multifocal with centripetal spreading. As KS lesions progress, they become brownish in color and may display verrucous and hyperkeratotic surfaces. At advanced stages of the disease, large, partly eroded, and ulcerated plaques that involve the feet, hands, and even entire extremities can occur. Enlargement of the lymph nodes, both superficial and deep, also is an early manifestation of the disease and may precede the cutaneous manifestations. A small percentage of patients may have visceral and/or mucosal involvement [6,7,10] .
Unlike KS in AIDS patients, non-HIV associated KS is a rather localized process which rarely involves lymph nodes or organs and in most cases responsive on local or systemic therapeutic strategies. African endemic KS, AIDS-related, and iatrogenic KS tend to be more aggressive than CKS [7,11] .
Sheep and cattle parapox virus infections cause in human beings diseases of very similar aspect, named Orf and milker's