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| CASE REPORT |
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| Year : 2015 | Volume
: 2
| Issue : 2 | Page : 97-99 |
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Giant corymbiform nevus: A rare entity
Aditya Kumar Bubna1, Leena Dennis Joseph2
1 Department of Dermatology, Sri Ramachandra University, Chennai, Tamil Nadu, India
2 Department of Pathology, Sri Ramachandra University, Chennai, Tamil Nadu, India
| Date of Web Publication | 29-Dec-2015 |
Correspondence Address:
Aditya Kumar Bubna
Department of Dermatology, Sri Ramachandra University, Porur, Chennai - 600 116, Tamil Nadu
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/2349-5847.172780
Nevus refers to a maternal impression or birthmark. Various categories of nevi have been described in literature, with newer presentations being continuously reported. At times, gigantic presentations of melanocytic nevi may alarm patients to seek medical attention. Most often, though, these nevi are totally benign, the excision of which is indicated solely for cosmetic indications. We hereby report a case of corymbiform nevi, a morphologic rare subtype of lobulated intradermal nevi, which presented as a huge plaque, and is being reported because of its rarity, and the first of its kind from the Indian subcontinent. Keywords: Corymbiform nevus, giant, lobulated
How to cite this article:
Bubna AK, Joseph LD. Giant corymbiform nevus: A rare entity. Pigment Int 2015;2:97-9 |
| Introduction | |  |
Melanocytic nevi (MN) are benign neoplastic proliferations composed of melanocytes. Clinically, various types of MN have been recognized, namely flat lesions, papillomatous lesions, dome shaped plaques, and pedunculated lesions.[1] When nevi cells occupy the dermis alone, devoid of junctional activity, the term intradermal nevus has been employed.[2] Lobulated intradermal nevus (LID) as suggested by its name, refers to a benign melanocytic growth with nevus cells present in the dermis and phenotypically characterized by a lobulated plaque. Kim et al.[3] have further classified LID based on its clinical appearance into corymbiform nevus (CN), verrucous plaque type and raspberry like LID. Cho et al.[4] were the pioneers in reporting unusual presentations of LID. Following this, there have been five more case reports of LID, one of them being from Indian literature.[5] The case reported from India, however, did not display the corymbiform morphology. We present this case because of its peculiar morphologic presentation, and histologic features which may not necessarily indicate that lobulation is a sign of aging in MN.
| Case Report | | |
A 35-year-old female was referred from the Department of General Surgery for a multilobulated discolored lesion over her right thigh [Figure 1]. The lesion had been there since childhood and had gradually increased in size over the past 5 years to attain the current dimensions, following which there was minimal lesional growth of around a centimeter every year. There were no symptoms associated with this, and the patient continued her daily chores, unhindered. On inspection, a giant pigmented plaque was seen, involving an area of 30 cm × 20 cm, over the right thigh. It was studded with numerous lobules that measured approximately 2 cm × 1 cm × 1 cm and were arranged in an orderly fashion, resembling a bunch of grapes. Some of the lobules were deeply pigmented, whereas others had a flesh colored hue, and few of them demonstrated comedo-like central plugging [Figure 2]. There were areas on the lesion that demonstrated growth of terminal hairs. Palpation revealed these lobules to be fleshy and nontender. Based on these clinical findings a diagnosis of nevus lipomatosus superficialis (NLS) of Hoffmann–Zurhelle and LID was considered. Two biopsy specimens were taken. One from a pigmented lobule and the other from a flesh colored lobule. Histopathology revealed similar findings in both the specimens. The epidermis was normal. A clear grenz zone was appreciated in the papillary dermis. As the section was further explored, it was noticed, in the deeper dermis and extending up to the subcutis, individual nevus cells uniformly occupying the entire section [Figure 3]. The cells in the deeper dermis were rounded and spindle-shaped nevus cells [Figure 4]. There was no adipocyte infiltration in both the specimens examined. With these findings, a diagnosis of giant CN was made, and treatment options for the same explained to the patient. However, the patient refused any surgical intervention. | Figure 1: Clinical picture of the corymbiform nevus on the right thigh of our patient
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 | Figure 2: A close up photograph showing comedo-like plugs in some of the lobules in the nevus
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 | Figure 3: A scanner view of H and E stained skin biopsy specimen, demonstrating the nevus cells occupying the full dermis. Cells in the upper dermis being more deeply pigmented than those in the lower dermis
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 | Figure 4: Dense aggregates of nevus cells, present in the deeper dermis, most of them being round nevus cells and a few of them spindle-shaped. To note, here is the absence of adipocytes (H and E, x20). An inset for the same demonstrating absence of neuronal changes and fibrosis (H and E, x40)
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| Discussion | | |
CN is a type of LID characterized by aggregation of lobules resembling a bunch of grapes. The term CN was first coined by Löffler and Effendy.[6] Till date, only four cases of CN have been reported, by Cho et al.,[4] Kim et al.,[3] Lee et al.,[7] and Löffler and Effendy.[6] Of the four cases described, three have been from Korean literature and one from German literature. The salient features of these cases, including ours, are summarized in [Table 1]. | Table 1: Salient features of various corymbiform nevi described in literature till date including ours
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Our case is the first report of CN, a subtype of LID, from India to the best of our knowledge. Interestingly, all the cases described earlier including ours was seen in females, and whether there appears to be a relationship with regard to female hormones is yet to be clarified. Another feature that resembled our case was the age of presentation which was mainly in the age group ranging from 30 to 37 years, except for the case reported from Germany, which had a later onset of presentation. Our patient differed from the previous four cases morphologically by its giant phenotype, almost covering 75% of the right thigh. Other distinguishing features were presence of flesh colored lobules as well as pigmented lobules that were distributed in varying sizes throughout the extent of the nevi, with some of them demonstrating comedo-like plugging, another unique feature in our patient. Comedo-like plugs have been previously described in literature with reference to NLS as a notable feature in reports by Lynch and Goltz,[8] Abel and Dougherty [9] and Jones et al.[10] In our case, this unusual feature of comedo-like plugs in CN is an extremely rare feature and is being reported for the 1st time. NLS definitely poses to be an important differential diagnosis for the all cases of CN because of their close resemblance to each other, and with certain features overlapping, clinical examination alone may not suffice at arriving a conclusive diagnosis, with histopathologic examination being mandatory. Biopsy from our patient revealed features consistent with intradermal nevi. However, certain differences as compared to the other cases described earlier were striking. In general, dermal fatty infiltration is a hallmark finding. However, our slide did not show evidence of adipocyte infiltration from both biopsy specimens examined. This feature was, however also reported by Lee et al.[7] in their patient. In the papillary dermis nevus cells with extensive melanization, arranged in organized nests and cords were visualized. At the level of reticular dermis and extending to the subcutis, numerous round and spindle-shaped nevus cells were seen, which diffusely occupied all fields was the characteristic finding observed. Other findings such as neuronal changes and fibrosis that indicate aging of MN were not a feature in our case as compared to the previous four cases. It has been previously suggested that lobulation in an intradermal nevi is a feature indicative of aging in MN. However, our patient did not show any of the histologic features of aging MN. Rather there was no evidence suggestive of regression in the nevi. Based on these features, we concluded that the lobulation may not necessarily indicate aging of MN. The other new findings discovered here were:
- CN may present as a gigantic growth
- Comedo-like plugs, a notable feature of NLS may also be seen in CN
- The absence of adipocyte infiltration may be a possibility in corymbiform nevi.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
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| 2. | Weedon D, editor. Lentigenes, nevi and melanomas. In: Weedon's Skin Pathology. 3 rd ed. London: Churchill Livingstone Elsevier; 2010. p. 710-56. |
| 3. | Kim DH, Park HS, Paik SH, Jeon HC, Cho KH. Four cases of lobulated intradermal nevus: A sign of aging melanocytic nevus. Ann Dermatol 2011;23:115-8. |
| 4. | Cho KH, Lee AY, Suh DH, Lee YS, Koh JK. Lobulated intradermal nevus. Report of three cases. J Am Acad Dermatol 1991;24:74-7. |
| 5. | Mhatre MA, Mysore VN. Lobulated intradermal nevus: A rare entity. Indian J Dermatopathol Diagn Dermatol 2014;1:83-5.  |
| 6. | Löffler H, Effendy I. Corymbiform nevus cell nevus. Hautarzt 1998;49:730-2. |
| 7. | Lee ES, Min KS, Min HG, Kim JM. A case of lobulated intradermal nevus with satellite lesions. Korean J Dermatol 1992;30:418-21. |
| 8. | Lynch FW, Goltz RW. Nevus lipomatosus cutaneous superficialis (Hoffmann-Zurhelle). Arch Dermatol 1958;78:479-82. |
| 9. | Abel R, Dougherty JW. Nevus lipomatosus cutaneus superficialis (Hoffman-Zurhelle); report of two cases. Arch Dermatol 1962;85:524-6. [ PUBMED] |
| 10. | Jones EW, Marks R, Pongsehirun D. Naevus superficialis lipomatosus. A clinicopathological report of twenty cases. Br J Dermatol 1975;93:121-33. [ PUBMED] |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1]
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