Eponymous Terminologies Related to Kaposi

INTRODUCTION

Born in 1837 in Hungary of Jewish origin, Moritz Kaposi is a well-known dermatologist for his contributions to dermatology and oncology. Known initially as Moritz Kohn, he later changed his name to Kaposi after his birthplace, Kaposvar, in Hungary. Another explanation is based on his comments to colleagues that he changed his name to avoid confusion with five other similarly named physicians on the Vienna faculty. He studied at the University of Vienna and worked under the mentorship of Ferdinand Von Hebra, after which he succeeded as the professor and director of skin diseases. He made numerous contributions to the field of dermatology, including authoring books such as Lehrbuch der Hautkrankheiten (Textbook of Skin Disease) along with Hebra. He later authored other books, such as Pathology and Therapy of Skin Disease and Atlas of Skin Diseases.^[1] He is well remembered for the descriptions of numerous skin conditions that bear his name, described below.

NAMED CONDITIONS AND SIGNS AFTER KAPOSI

Kaposi sarcoma (KS)

Initially termed ‘idiopathic pigmented sarcoma of the skin’ and it was first described in 1872 by Kaposi in five patients with this condition. It is caused by human herpes virus-8 (HHV8) also termed KS herpes virus. It is classified into various clinical types such as classical type, endemic or African type, epidemic or HIV-associated KS and KS associated with immunosuppression or solid organ transplantation. Mostly seen in older adults, it also affects young children, especially in the case of endemic KS. It can be polymorphic in presentation, presenting as asymptomatic or tender bluish-red macules [Figure 1a], plaques or nodules usually affecting the extremities and involving the mucosa. Dermoscopic features of KS include white dots and lines, white clods four-dot clods, collarette sign [Figure 1b], serpentine vessels and polychromatic lustre termed as rainbow pattern.^[2,3] Histologically KS sarcoma is characterised by a proliferation of irregular, jagged vascular channels, which partly surround pre-existing blood vessels in some areas. This characteristic appearance of a small vessel protruding into an abnormal vascular space has been termed ‘promontory sign’. Treatment depends on the type of KS and includes surgical, chemotherapy and radiotherapy. While small, localised lesions can be excised, larger multifocal lesions respond well to cytotoxic agents such as vinblastine, paclitaxel and doxorubicin with radiotherapy if required. HIV/AIDS-related KS responds well to highly active antiretroviral therapy.^[4]

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Figure 1:

(a) Violaceous macules of Kaposi sarcoma in a patient with AIDS. (b) Reddish pink structureless areas with four dot clods and collarette sign in Kaposi sarcoma (Dermlite DL4, ×10).

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Kaposi varicelliform eruption

Kaposi varicelliform eruption also termed eczema herpeticum, is an acute viral eruption most caused by herpes simplex virus in individuals with pre-existing dermatoses. It can also be caused by the coxsackie virus (eczema coxsackium), varicella-zoster virus, vaccinia virus (eczema vaccinatum) and other viruses. The underlying conditions are mainly atopic dermatitis, pemphigus foliaceous, Darier’s disease, benign familial pemphigus, allergic contact dermatitis and Sezary syndrome. It presents as multiple vesiculopustular lesions with central umbilication, forming punched-out polycyclic erosions [Figure 2] with haemorrhagic crusting. It is associated with fever and systemic symptoms based on visceral involvement, such as hepatic, neurological, pulmonary and renal disease. Diagnosis can be confirmed by cytological methods with Tzanck smear demonstrating multinucleated giant cells and by more sensitive methods such as viral polymerase chain reaction and viral culture. Treatment includes antivirals, mainly acyclovir dosed intravenously, and optimisation of the underlying condition.^[5,6]

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Figure 2:

Punched out polycyclic erosions of eczema herpeticum in pemphigus vulgaris.

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Kaposi-Stemmer sign

Patients with chronic lymphoedema developed progressive swelling of the extremities, which is non-pitting in early stages, progressing to thickening and marked hyperkeratosis with papillomatosis referred to as elephantiasis nostras verrucosa cutis. Clinical signs of lymphoedema can help to distinguish it from other causes of leg oedema. KaposiStemmer sign refers to the inability to pinch the base of the second toe [Figure 3] and occurs due to the progressive thickening and fibrosed skin beneath. It is a usual clinical marker that is specific for diagnosing lymphoedema and can be confirmed further by lymphoscintigraphy.^[12]

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Figure 3:

Kaposi-stemmer sign in filarial lymphoedema.

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CONCLUSION

Kaposi died at the age of 65 years in the year 1902 at Vienna. He is still remembered for his rich legacy in the field of dermatology for the conditions that bear his name as enumerated above. Hence, the dermatologist must be aware of these conditions as a tribute to Kaposi.