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Year : 2022  |  Volume : 67  |  Issue : 5  |  Page : 583-586
Pyogenic granuloma-like Kaposi's sarcoma on the ear in an immunocompetent patient

Faculty of Medicine, Department of Dermatology, Sheikh Khalifa Hospital, Mohamed VI University of Health Sciences, Casablanca, Morocco

Date of Web Publication29-Dec-2022

Correspondence Address:
Fatima-Zahra Agharbi
Department of Dermatology, Sheikh Khalifa Hospital, Faculty of Medicine, Mohamed VI University of Health Sciences, Avenue Taieb Naciri - 82403, Casablanca
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijd.ijd_1028_21

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Botriomycome-like Kaposi disease is a rare clinical and pathological variant of Kaposi disease. Showing features of pyogenic granuloma (PG) and of Kaposi's sarcoma (KS), it was initially named KS-like PG and considered benign.[2] It is now considered a true KS and was renamed PG-like KS due to the clinical course and the presence of human herpes virus-8 DNA.[13],[14] This entity has been mostly described on the lower extremities, but other rare locations such as hand, nasal mucosa, and face have been reported in the literature.[1],[3],[4] The location on the ear in an immune-competent field, like in our patient, is a very rare finding, and very few cases have been reported in the literature.[5]

Keywords: Dermoscopy, ear, human herpes virus-8, Kaposi sarcoma, pyogenic granuloma

How to cite this article:
Agharbi FZ, Albouzidi A, Chiheb S. Pyogenic granuloma-like Kaposi's sarcoma on the ear in an immunocompetent patient. Indian J Dermatol 2022;67:583-6

How to cite this URL:
Agharbi FZ, Albouzidi A, Chiheb S. Pyogenic granuloma-like Kaposi's sarcoma on the ear in an immunocompetent patient. Indian J Dermatol [serial online] 2022 [cited 2023 Feb 7];67:583-6. Available from:

   Introduction Top

Kaposi's sarcoma (KS) was first described in 1872 by Moritz Kaposi, as a rare multifocal angioproliferative tumor involving blood and lymphatic vessels in elderly men of Jewish origin. It is the most frequent cancer seen in patients with HIV infection. The tumor has four well-defined clinical presentations that have been categorized as classic, endemic, or African, transplant-associated or iatrogenic, and acquired immunodeficiency syndrome-associated or epidemic KS.[1] KS has different clinic-pathological presentations and there are many kinds of KS variants. Pyogenic granuloma (PG)-like KS is a new variant that protrudes from the skin and resembles a PG. This may make the diagnosis challenging, due to the clinical and histological features of both PG and KS.

The most common location of the non-HIV-associated form is in the lower limbs. The location in the ear, as in our patient, is rarely reported in the literature and increases the risk of misdiagnosis.

   Observation Top

A 50-year-old patient with no significant pathological history consulted for a purplish nodule in the right ear [Figure 1]. On dermoscopic examination, it was noted red and white structure less areas with dotted vessels [Figure 2]. Histological study after resection showed sarcomatous dermal cell proliferation with a double vascular and fibroblastic component with atypia and mitosis and vascular clefts between spindle cells with extravasation of red blood cells. This histological aspect was in favor of Kaposi's disease [Figure 3]. The immune-histochemistry confirmed this diagnosis with positive staining for CD 31 [Figure 4] and human herpes virus-8 (HHV-8) [Figure 5]. Visceral involvement and HIV serology were negative.
Figure 1: Purplish nodule over the right ear

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Figure 2: Red and white structure less areas with dotted vessels

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Figure 3: (HES ×40): proliferation with dual vascular and fibroblast components

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Figure 4: CD 31 positive marking

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Figure 5: Positive HHV 8 marking

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   Discussion Top

PG-like KS is a rare clinic-pathological variant of KS with clinical and histological features of both PG and KS. This condition is characterized by superficially located nodular KS lesions that become protuberant and elicit the development of peripheral epidermal collarets. Traumatized lesions may undergo ulceration and become inflamed such that they may be misdiagnosed as PG. This entity is rare but also often underdiagnosed. A few observations of PG-like KS disease have been reported in the literature in both HIV positive and HIV negative patients.[1],[2],[3],[4],[5],[6],[7],[8],[9] The most frequent location is the lower extremities, but other rare locations have been reported in the literature.[1],[3],[4] In one report, the PG-like KS was initially thought to be a PG of the nasal mucosa.[3] Cabibi et al.[4] reported the PG-like KS on the hands of three patients, which is a common location for PG. Localization in the ear in the form of multiple lesions is common in the HIV-associated form. However, rare cases of Kaposi botriomycoma-like in the ear have been reported by some authors outside the context of immunodepression, as was the case for our patient.[5] The main differential diagnosis of this form of Kaposi disease is botriomycoma. Nodular KS usually shows numerous dome-shaped nodular lesions, unlike PG, which often presents with a single lesion.[10] However, it should be kept in mind that KS may present with a single lesion and PG may show multiple nodules.[11],[12] Histopathological examination and immunohistochemistry are essential for a positive diagnosis.[6] In PG, an anatomopathological study shows the presence of vascular proliferation in a lobular fashion, fibrous septations, inflammation, and edema resembling granulation tissue with no or rare extravasated red blood cells. In KS, it has a sarcomatous appearance, with solid areas made up of atypical spindle cells with mitosis and remarkable nuclear atypia, slit-like vessels, diffuse extravasated red blood cells, and hemosiderin. Fibrous septae and a lobulated growth pattern are absent.[4] Immunohistochemistry is also useful in the differential diagnosis between PG and KS. In PG, smooth muscle actin (SMA) expressed by pericytes and factor VIII-related antigen-marking mature endothelial cells is positive. On the contrary, in the KS, the solid spindle cell areas are typically negative for factor VIII and SMA, due to the absence of both mature endothelial cells and pericytes, and are diffusely positive for endothelial markers CD31 and CD34.[4] In PG-like KS we found histological features of both PG and KS, and it has been reported that immunohistochemistry in such lesions was positive not only for CD31 and CD34 but also for SMA and factor VIII-related antigens, which are typically negative in KS. This profile is typical of PG-like KS. HHV-8 DNA detection is considered both sensitive and specific to KS, so the importance of looking for HHV-8 DNA in PG-like KS has been underlined.[3],[4],[13],[14] Previous studies have shown that polymerase chain reaction-based detection of HHV-8 DNA or RNA has high sensitivity and specificity for diagnosing KS and helps to differentiate KS from its mimickers. Cheuk et al. studied 50 cases of KS and 53 cases of KS mimickers, and findings showed that 100% of KS cases were positive for HHV-8 latent nuclear antigen-1 (LNA-1), while 100% of KS mimickers were negative.[15] This study showed that immunostaining of LNA-1 exhibits high sensitivity and specificity for the diagnosis of KS and is useful for distinguishing it from mimickers.[15] Dermoscopy can also guide the diagnosis. Polychromatic structures, also known as rainbow patterns, are described as the presence of many different colors juxtaposed next to each other on polarized dermoscopy.[16] This rainbow pattern was first described as a specific clue to KS but it has subsequently been observed in many conditions, including blue nevus, angiokeratoma, hypertrophic scars, stasis dermatitis, and acroangiodermatitis.[16],[17] Recently, a study investigating the dermoscopic aspect of PG and KS to demonstrate dermoscopic clues to differential diagnosis has been published.[18] The most common findings detected in nodular KS were polychromatic structures and red and white structureless areas. In PG, the most common dermoscopic sign was red structureless areas, followed by intersecting thick white lines, ulceration, and collarette scale. New dermoscopic signs were reported by this study, which is a widespread vessel composed of a network of nodular KS and a striate surface scaling for PG.[18]

For our patient, the diagnosis of PG-like KS was made based on the clinical appearance, dermoscopy, histology, immunohistochemistry, and positivity of HHV-8. Our report could be interesting because only a few previous reports about PG-like KS in the ear are reported in the literature which increases the diagnostic challenge.

   Conclusion Top

KS can present as rare and atypical variants and may mimic many diseases. PG-like KS is a rare variant that can be misdiagnosed as PG and we think that PG-like KS is an underdiagnosed entity, mainly when localized on uncommon sites like in our case. The differential diagnosis of botriomycome and the entities is essential as both of them differ in prognosis and necessitate different management and follow-up. Positivity of HHV-8 still remains the gold standard in this respect but dermoscopy can be helpful.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Bouzidi H, Gallouj S, Amraoui N, Mernissi FZ, Harmouch T. Maladie de kaposi classique botriomycome-like: Un piège diagnostic. Pan Afr Med J 2014;17:283.  Back to cited text no. 1
Fukunaga M. Kaposi's sarcoma-like pyogenic granuloma. Histopathology 2000;37:192-3.  Back to cited text no. 2
Wyatt ME, Finlayson CJ, Moore-Gillon V. Kaposi's sarcoma masquerading as pyogenic granuloma of the nasal mucosa. J Laryngol Otol 1998;112:280-2.  Back to cited text no. 3
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.  Back to cited text no. 4
Rodríguez LF, Lebróna AB, Ruíz CM, Rocaa JB. Dermatolog non-AIDS Kaposi sarcoma in the external ear. Actas Dermosifiliogr 2016;017:872-84.  Back to cited text no. 5
Scott PL, Motaparthi K, Krishnan B, Hsu S. Pyogenic granuloma-like Kaposi sarcoma: A diagnostic pitfall. Dermatol Online J 2012;18:4.  Back to cited text no. 6
Umar A, Mohammed TT, Ahmed MS, Samaila MO. Pyogenic granuloma-like kaposi's sarcoma. Ann Trop Pathol 2017;8:124-7.  Back to cited text no. 7
  [Full text]  
Kızılyel O, Metin MS, Elmas OF, Bilen H, Akdeniz N, Atasoy M, et al. Pyogenic granuloma-like Kaposi sarcoma: Report of two cases. Cumhur Med J 2015;37:152-5.  Back to cited text no. 8
Megaly M, Boshra N. Pyogenic granuloma like Kaposi's sarcoma. Lancet 2022;399:e38.  Back to cited text no. 9
Curtiss P, Strazzulla LC, Friedman-Kien AE. An update on Kaposi's sarcoma: Epidemiology, pathogenesis and treatment. Dermatol Ther 2016;6:465-70.  Back to cited text no. 10
Schmidt BM, Holmes CM. Classic solitary Kaposi sarcoma of the foot in an immunocompetent patient: A case report. Wounds 2016;28:35-40.  Back to cited text no. 11
Mohanty G, Mohanty R, Satpathy A. Simultaneous occurrence of pyogenic granuloma at multiple sites associated with bone loss: Report of a rare case. J Indian Soc Periodontol 2018;22:174-7.  Back to cited text no. 12
[PUBMED]  [Full text]  
Ryan P, Aarons S, Murray D, Markham T, Markham T, O'Sullivan S, Lyons F, et al. Human herpesvirus 8 (HHV-8) detected in two patients with Kaposi's sarcoma-like pyogenic granuloma. J Clin Pathol 2002;55:619-22.  Back to cited text no. 13
Urquhart JL, Uzieblo A, Kohler S. Detection of HHV-8 in pyogenic granuloma-like Kaposi sarcoma. Am J Dermatopathol 2006;28:317-21.  Back to cited text no. 14
Cheuk W, Wong KO, Wong CS, Dinkel JE, Ben-Dor D, Chan JK, et al. Immunostaining for human herpesvirus 8 latent nuclear antigen-1 helps distinguish Kaposi sarcoma from its mimickers. Am J Clin Pathol 2004;121:335-42.  Back to cited text no. 15
Kelati A, Mernissi FZ. The rainbow pattern in dermoscopy: A zoom on non Kaposi sarcoma skin diseases. Biomed J 2018;41:209-10.  Back to cited text no. 16
Uzunçakmak TK, Ozkanli S, Karadağ AS. Dermoscopic rainbow pattern in blue nevus. Dermatol Pract Concept 2017;7:60-2.  Back to cited text no. 17
Elmas OF, Akdeniz N, Acar EM, Kilitçi A. Pyogenic granuloma and nodular Kaposi's sarcoma: Dermoscopic clues for the differential diagnosis. Turk J Med Sci 2019;49:1471-8.  Back to cited text no. 18


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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