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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.79 no.6 Rio de Janeiro Nov./Dec. 2004
Sutton's Nevus simulating cockade nevus: report of a case*
Maurício ZaniniI; Carlos D'Apparecida Machado FilhoII; Francisco Macedo PaschoalIII; Francisco Le VocciIII
IDermatologist, active member of the
Brazilian Dermatology Society, Assistant Lecturer in Dermatological Surgery
IIPhD in Dermatology, Acting Head of Dermatology Course
IIIMSc, Dermatologist, Assistant Professor - Dermatology Department
Authors relate a case of Sutton's nevus clinically simulating the cockade nevus. Cockade nevus is a rare form of acquired nevomelanocytic nevus that presents characteristic aspect of target-like lesion. It may determine difficulty diagnostic with melanoma, dysplastic and Sutton's nevus.
Key words: diagnosis; nevus; nevus, pigmented; pathology.
Nevus (Latin: naevus, mole, birthmark) is a circumscribed lesion of the skin and/or of the mucosa with a long or permanent clinical course, probably reflecting genetic mosaicism.1,2 A melanocytic nevus is a grouping of nevomelanocytes in the epidermis and/or in the dermis.3 The nevomelanocytic cell is similar to the melanocyte, but it can be differentiated by the fact that it does not present dendritic processes, besides having a rounder aspect, with more abundant cytoplasm and more prominent nuclei and nucleoli. The melanosomes in a nevomelanocytic cell are identical to those found in melanocytes, except for their size and number.3 Eighty percent or more of the population has or has had at least one melanocytic nevus. These nevi tend to appear during the first year of life and grow with corporal development then regress with aging.2,3
Sutton's halo nevus is characterized by the presence of a leucodermic halo around a melanocytic nevus.2,3 Cocardiform nevus (in cockade) is a rare variant of acquired melanocytic nevus4 that characteristically presents a lesion with a target-like aspect.3,4 In this work, the authors report a case in which a Sutton's nevus closely resembled a cockade nevus.
The patient, white, female, aged 35 years, complained of a stain on her back, present for more than a year. The course of growth of the lesion had been slow, occasionally associated with pruritus and burning sensation. In the examination, a well-delineated target-like lesion on the patient's back was observed, measuring 2 x 2 cm. It consisted of an erythematous central papule discreetly raised, normochromic circular intervening zone, and a peripheral brownish macular halo, with a diameter of 0.5 cm (Figures 1 and 2). The patient also presented multiple stains and brownish papules of various shades on her back, compatible with a diagnosis of melanocytic nevi and solar melanoses. She denied having a family history of similar disease. She was submitted to histopathological evaluation with an excision biopsy. The clinical hypothesis included Sutton's nevus, melanoma, cockade nevus and dysplastic nevus. The histopathology revealed nevomelanocytic cells in the epidermis and in the superficial dermis, and intense inflammatory infiltrate, predominantly lymphocytic, in the halo (Figure 3). The histopathological diagnosis was Sutton's nevus.
Melanocytic nevus is a grouping of nevomelanocytes in the epidermis and/or dermis. It is considered to be a benign tumor or hamartoma of nevomelanocytic cells. It originates from an abnormal melanoblast (nevomelanoblast) derived from the neural crest, which then migrates with the melanoblasts to the dermis and subsequently to the epidermis before the eighth week of gestation.3 Nevus cells present sequential maturation, beginning in the epidermis and later migrating to the dermis in the form of cellular nests. Depending on the maturation phase, there is a clinicohistological variability in the acquired melanocytic nevi occurring in the junctional (epidermis), connective (epidermic and dermic) and intradermic tissues.5 They may also be found in other areas, such as in the deep dermis, panniculus and adnexial structures.1,2,3,5
Sutton's halo nevus, also known as acquired centrifugal leucoderma, is characterized by the presence of a leucodermic halo around a melanocytic nevus. In 1874, Hebra and Kaposi described the condition inadequately as vitiligo. It was in 1916 that Sutton determined that this nevus was a dermatologically distinct entity. It affects 1% of the general population, and is unrelated to gender, race or genetics. Its peak incidence is in the second decade of life. Up to 50% of these patients present more than one halo nevus, which predominately occur in the trunk.3 The development of Sutton's halo nevus seems to be the result of a phenomenon of self-destruction of the nevus cells due to an immunological mechanism; in which the nevus progressively disappears, leaving a leucodermic stain that eventually repigments completely. Normally it is an alteration without clinical expression, however, it may be the first manifestation of vitiligo, in which case treatment of the patient would be necessary.6 The halo phenomenon, similar to Sutton's nevus, can occur with melanoma.3
Cocardiform nevus (in cockade) is a rare variation of acquired melanocytic nevus, initially described by Mehregan and King, in 1972.4 The term 'cockade', of French origin, was introduced by Happle in 1974. It is used because the lesion reminds one of a rosette, a badge of coiled ribbon in the form of a rose, used in hats and buttonholes.4,7 The adjective cocardiform is also used for other dermatological disturbances, such as vitiligo and purpura.8,9 It refers to a central melanocytic nevus in the junctional complex (usually the nevus is junctional) or in the connective tissue. It is surrounded by a pigmented halo, and between the center and the halo, a normochromic area, giving the lesion a target-like aspect.3,4,5,7,10,11 There are little more than ten cases reported in the literature. Perhaps this low number is due to confusion regarding this affliction, with consequent diagnostic error (Sutton's nevus, dysplastic nevus, common melanocytic nevus).3,10 All of the reported cases affected young patients. Some cases may be associated with spinal dysraphism or juvenile diabetes mellitus.3,4,10 The mechanism responsible for the development of this clinical form of melanocytic nevus is unknown.11 Its histology does not present inflammatory infiltrate, and immunofluorescence is negative. These data rule out the possibility of an immunological mechanism being responsible for the development of cockade nevus; this is contrary to what occurs with Sutton's nevus.7,11 The management of cockade nevus is the same as with common melanocytic nevus. Concerning cockade nevus, however, there is eventual difficulty in the differential clinical diagnosis with dysplastic nevus, Sutton's nevus and melanoma.
To the authors' knowledge, this study is the first description of the way in which Sutton's nevus simulates cockade nevus. It was surprising that the presentation of Sutton's nevus revealed itself to be identical to that of cockade nevus, since usually in Sutton's nevus, the halo phenomenon begins in the periphery of the nevus lesion. Unlike the usual situation, in the patient reported here, the halo began intermediately, between the periphery and the center of the melanocytic nevus. The clinicopathological correlation revealed that the central papula, predominantly erythematous, was composed of nevus cells surrounded by inflammatory infiltrate, and the circular pigmented area contained epidermal nevus cells.
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Received on January 02, 2003
Approved by the Consultive Council and accepted for publication on December 05, 2003
* Work done at "Instituto da Pele Prof. Dr. Luiz Henrique Camargo Paschoal" - ABC Medical School, Santo Andre, SP, Brazil.