Indian Dermatology Online Journal

: 2017  |  Volume : 8  |  Issue : 1  |  Page : 70--72

SkIndia Quiz 33: Solitary pigmented plaque containing a blue-gray papule

Cody J Connor1, Brian L Swick2,  
1 Carver College of Medicine, University of Iowa, Iowa City Veterans Affairs Medical Center, Iowa, USA
2 Department of Dermatology and Pathology, University of Iowa, Iowa City Veterans Affairs Medical Center, Iowa, USA

Correspondence Address:
Dr. Brian L Swick
Department of Dermatology, University of Iowa, 200 Hawkins Drive, 40025 PFP, Iowa City, Iowa - 52245

How to cite this article:
Connor CJ, Swick BL. SkIndia Quiz 33: Solitary pigmented plaque containing a blue-gray papule.Indian Dermatol Online J 2017;8:70-72

How to cite this URL:
Connor CJ, Swick BL. SkIndia Quiz 33: Solitary pigmented plaque containing a blue-gray papule. Indian Dermatol Online J [serial online] 2017 [cited 2019 Jul 21 ];8:70-72
Available from:

Full Text

A 35-year-old woman presented with a new dark papule that had arose within a previously stable tan plaque on her right thigh. There was no history of itching or bleeding from the lesion. Her past medical and family history were unremarkable. On examination, there was a 1.1 cm sharply demarcated tan plaque on the right anterior thigh containing an eccentric blue-gray papule [Figure 1].{Figure 1}

Low-power histopathologic examination revealed a nested proliferation of cytologically bland appearing melanocytes focally along the dermoepidermal junction and more extensively in the dermis with a second population of pigmented epithelioid appearing melanocytes with surrounding melanophages in the upper reticular dermis [Figure 2]. High-power viewing confirmed the presence of two populations of melanocytes; the first was the small amelanotic population and the second consisted of localized nests of epithelioid melanocytes with conspicuous cytoplasmic melanization. There were prominent pigmented melanophages [Figure 3].{Figure 2}{Figure 3}

 What Is the Diagnosis?

Cellular blue nevusClonal nevus (inverted type A nevus)Common blue nevusDeep penetrating nevusEpithelioid blue nevus

 View Answer


Clonal nevus.


Known by a number of other designations, including inverted type A nevus and melanocytic nevus with focal atypical epithelioid components, clonal nevus is a distinct entity in the differential diagnosis of pigmented, dermal, melanocytic lesions, including malignant melanoma. However, careful histopathologic inspection will reveal distinctive features of clonal nevi that distinguish these benign lesions from malignant melanoma and other pigmented dermal melanocytic lesions.

Clinically, a clonal nevus appears as a biphasic, tan to light brown nevus with a focal macular or papular region of darker pigmentation, which may appear blue-gray to blue-black [Figure 1].[1],[2] This dark area commonly develops amidst a preexisting banal compound or dermal nevus, either centrally or eccentrically, and can be alarming to an affected patient or physician who recognizes the change. Histopathologically, this dark focus corresponds to clusters of similar appearing, atypical, epithelioid-appearing melanocytes surrounded by heavily pigmented melanophages [Figure 2].[1],[2] These melanocytes are characterized by dusty brown cytoplasmic melanization, enlarged nuclei with prominent nucleoli, and (in stark contrast to the mitotically active melanocytes of melanoma) rare, if any, mitotic figures.[1] The nuclei of these melanocyte “clones” are uniform and slightly larger than those of the melanocytes within the surrounding ordinary melanocytic component of the lesion [Figure 3].[1],[2] The pigmented epithelioid melanocytic component demonstrates HMB-45 expression, whereas the surrounding nonpigmented, nonepithelioid dermal melanocytes associated with the lesion typically do not.[3] A histopathologically similar lesion is the deep penetrating nevus. DPN assumes a symmetrical, inverted wedge shape composed of atypical appearing spindled to epithelioid melanocytes with dusty brown cytoplasm and associated heavily pigmented melanophages.[4] In addition, the pigmented dermal melanocytes in DPN are often associated with a background conventional appearing nevus.[4] Although DPN and clonal nevus demonstrate similar cytomorphology, and a background of ordinary appearing melanocytic component, they primarily differ in their respective depth of dermal involvement and presence of periadnexal/neurovascular growth with DPN extending into the reticular dermis in an inverted wedge shape along the adnexal/neural vascular structures.[1],[4] In contrast, clonal nevi remain confined to the superficial dermis.[1] Interestingly, one study identified lesions of intermediate depth, between that of clonal nevi and DPN: A finding that supports the notion that these two lesions may in fact represent different points on a continuum for the same entity.[5] Finally, some authors favor the term combined nevus (monomorphic variant), for those lesions showing features of DPN.[6] Despite their depth of involvement and cytologic atypia, DPNs have never been found to metastasize and, just like clonal nevi, are considered to be benign lesions.[4]

Other pigmented, dermal melanocytic lesions include the common blue nevus, cellular blue nevus, and epithelioid blue nevus. Clinically, common blue nevi are acquired blue or blue-black macules or dome-shaped papules occurring on the backs of the hands and feet, buttocks, face, or scalp.[7] Onset may be in childhood or later in life.[7] Histopathologically, the common blue nevus appears as a symmetric, well-demarcated proliferation of dendritic melanocytes with darkly staining nuclei and elongated cytoplasmic processes. The process is often centered on adnexal structures, and the surrounding stroma can demonstrate varying degrees of fibrosis.[7]

Cellular blue nevi can be biphasic, like clonal nevi, and in that setting are usually paired with a component of common blue nevus.[7] They are larger than common blue nevi, often 1–3 cm or larger, and most commonly present on the buttocks—less often on the head, neck, and extremities—as firm, dermal nodules with a bluish-gray hue. Like DPN, cellular blue nevi show extension along neurovascular and adnexal structures sometimes into the deeper, subcutaneous tissue. Pale-staining oval or spindled melanocytes form fascicles or sheets in “cellular” areas, and “wreath-like” giant cells can be found.[7]

Epithelioid blue nevus was originally described presenting in childhood as a component of the Carney complex, a familial lentiginosis and low-grade, multiorgan neoplasia syndrome consisting of endocrine overactivity, patchy skin pigmentation, myxomas, and psammomatous, melanotic schwannomas.[7],[8] However, since then, sporadic lesions have been described in both children and adults as a dome-shaped darkly pigmented papule on the trunk or extremities.[9] Histopathologically, epithelioid blue nevi present as an oval or wedge-shaped dermal tumor composed of pigmented epithelioid melanocytes and pigmented spindled melanocytes similar to common blue nevi.[7],[8],[9] The epithelioid melanocytic component demonstrates large vesicular nuclei with prominent nucleoli in association with heavily pigmented globular melanophages.[7],[8],[9] Epithelioid blue nevi may occur in association with other blue nevi as part of a combined nevus.[10] Some consider it to represent the same entity as pigmented epithelioid melanocytoma, which is classified as a low-grade melanocytic tumor with metastatic potential.[7]


1Ball NJ, Golitz LE. Melanocytic nevi with focal atypical epithelioid cell components: A review of seventy-three cases. J Am Acad Dermatol 1994;30:724-9.
2Huynh PM, Glusac EJ, Bolognia JL. The clinical appearance of clonal nevi (inverted type A nevi). Int J Dermatol 2004;43:882-5.
3Skelton HG 3rd, Smith KJ, Barrett TL, Lupton GP, Graham JH. HMB-45 staining in benign and malignant melanocytic lesions. A reflection of cellular activation. Am J Dermatopathol 1991;13:543-50.
4Cooper PH. Deep penetrating (plexiform spindle cell) nevus. A frequent participant in combined nevus. J Cutan Pathol 1992;19:172-80.
5High WA, Alanen KW, Golitz LE. Is melanocytic nevus with focal atypical epithelioid components (clonal nevus) a superficial variant of deep penetrating nevus? J Am Acad Dermatol 2006;55:460-6.
6Pulitzer DR, Martin PC, Cohen AP, Reed RJ. Histologic classification of the combined nevus. Analysis of the variable expression of melanocytic nevi. Am J Surg Pathol 1991;15:1111-22.
7Phadke PA, Zembowicz A. Blue nevi and related tumors. Clin Lab Med 2011;31:345-58.
8Carney JA, Ferreiro JA. The epithelioid blue nevus. A multicentric familial tumor with important associations, including cardiac myxoma and psammomatous melanotic schwannoma. Am J Surg Pathol 1996;20:259-72.
9Moreno C, Requena L, Kutzner H, de la Cruz A, Jaqueti G, Yus ES. Epithelioid blue nevus: A rare variant of blue nevus not always associated with the Carney complex. J Cutan Pathol 2000;27:218-23.
10Groben PA, Harvell JD, White WL. Epithelioid blue nevus: Neoplasm Sui generis or variation on a theme? Am J Dermatopathol 2000;22:473-88.