Dermatology Online Journal

Classic Kaposi sarcoma mimicking chilblains
Sergio Vano-Galvan^1,2, Carmen Moreno³, Manuel Fernández-Lorente², Pedro Jaén^1,2
Dermatology Online Journal 17 (2): 16

1. Dermatology Dept, Ramon y Cajal Hospital, University of Alcala, Madrid, Spain. sergiovano@yahoo.es
2. Grupo de Dermatología Clínic, Madrid, Spain
3. Pathology Dept, Ramon y Cajal Hospital, Madrid, Spain

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Abstract

Kaposi Sarcoma (KS) is a low-grade vascular tumor associated with human herpes virus-8 infection. It usually presents in elderly patients as violaceous plaques of the lower limbs. However, KS may present in a wide spectrum of atypical clinical forms, leading to misdiagnoses. We report a case of classic KS mimicking chilblains.

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Figure 1	Figure 2
Figure 1. Clinical image showing violaceous infiltrated nodules and patches located on palms affecting fingertips and both thenar and hypothenar eminences Figure 2. Dermoscopic image showing violaceous patch with blue-grey areas

An 82-year-old man presented with a 2-month history of painful purple lesions located on both palms that worsened with cold. He had been previously diagnosed with chilblains but no response was achieved with the avoidance of cold. No immunosuppressive therapy had been administered to the patient previously. Physical examination revealed violaceous infiltrated nodules and patches located on palms, without any other lesions (Figure 1). Dermoscopy revealed a violaceous patch with blue-grey areas (Figure 2). The clinical diagnosis included atypical KS, chilblains, and chilblain lupus. A skin biopsy was performed. Histological examination and nuclear immunostaining for human herpes virus-8 (HHV-8) confirmed the diagnosis of classic Kaposi sarcoma (Figure 3). An HIV antibody test was performed and was negative. Radiological and endoscopic studies were unremarkable. Treatment with liposomal doxorubicine was started and provided adequate control of disease after 4 months.

Figure 3a	Figure 3b
Figure 3. Histological image revealing a dermis packed by a fusiform cellular proliferation with some hyaline globules (Figures 3a and 3c), with areas of irregular cleft-like vascular channels lined by plump, slightly atypical and hyperchromatic endothelial cells (Figure 3b). Nuclear immunostaining for human herpes virus-8 was positive in the neoplastic cells (Figure 3d).

Figure 3c	Figure 3d

Kaposi sarcoma (KS) is a multicentric neoplasm frequently evident as multiple vascular cutaneous and mucosal nodules. It is linked to HHV-8, which is a necessary, though alone insufficient, condition for the development of the lesions [1]. Four variants have been described: classic, immunosuppression-associated, endemic African, and HIV-related.

Clinically it presents as small, violaceous macules and papules situated particularly on the lower limbs [2] and, less frequently, on the upper arms, forearms, trunk, eyelids, and genital area. Several atypical presentations of KS have been described. Kaposi sarcoma may present as isolated palmoplantar lesions [3]. Lesions mimicking pyogenic granuloma of the hands have also been reported [4]. A case of KS mimicking Stewart-Treves syndrome was described by Salameire et al [5]. Atypical variants of generalized lymphadenopathic, telangiectatic, keloidal, ecchymotic, and cavernous KS have also been described [6].

To the best of our knowledge, no cases of KS mimicking lesions of chilblains on the hands have been described. Chilblains (or perniosis) is an inflammatory skin condition presenting after exposure to cold as pruritic and/or painful erythematous-to-violaceous acral lesions. It usually improves with the warming of acral areas achieved by heat and appropriate clothing. Our case mimicked chilblains because of the exclusive localization of the violaceous nodules and patches on the hands. In addition, the patient had noted worsening of the lesions with cold. Nevertheless, the avoidance of cold not only did not improve the condition, but the eruption gradually became more extensive.

In summary, KS may present with many atypical clinical presentations and physicians should be suspicious when violaceous nodules or patches develop on the palms or soles of elderly patients. A skin biopsy should be performed in these cases.

References

1. Sullivan RJ, Pantanowitz L, Casper C, Stebbing J, Dezube BJ. Epidemiology, pathophysiology, and treatment of Kaposi sarcoma—associated herpesvirus disease: Kaposi sarcoma, primary effusion lymphoma, and multicentric Castleman disease. Clin Infect Dis. 2008; 47: 1209-15. [PubMed]

2. Vano-Galvan S, Alonso-Jimenez T. Classic Kaposi's sarcoma. Isr Med Assoc J. 2007; 9: 896. [PubMed]

3. Al Zolibani AA, Al Robaee AA. Primary palmoplantar Kaposi's sarcoma: an unusual presentation. Skinmed. 2006; 5: 248-9. [PubMed]

4. Cabibi D, Cacciatore M, Viviano E, Guarnotta C, Aragona F. 'Pyogenic granuloma-like Kaposi's sarcoma' on the hands: immunohistochemistry and human herpesvirus-8 detection. J Eur Acad Dermatol Venereol. 2009; 23: 587-9. [PubMed]

5. Salameire D, Templier I, Charles J, Pinel N, Morand P, Leccia MT, Lantuejoul S. An "anaplastic" Kaposi's sarcoma mimicking a Stewart-Treves syndrome. A case report and a review of literature. Am J Dermatopathol. 2008; 30: 265-8. [PubMed]

6. Schwartz RA, Micali G, Nasca MR, Scuderi L. Kaposi sarcoma: a continuing conundrum. Am Acad Dermatol. 2008; 59: 179-206. [PubMed]

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