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Healthy 5-year-old male presents with lesion on his abdomen
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A healthy 5-year-old male presents to your clinic for a well-child visit. His mother brings to your attention the large café-au-lait lesion on his abdomen that has developed some areas of pigmentation during the past few years. The birthmark presented shortly after birth is asymptomatic and has grown with him. He does not have any other café-au-lait macules or other stigmata of neurofibromatosis.
On physical examination, you notice a few well-demarcated, evenly round, smooth, dark brown macules within a several centimeter tan patch on the abdomen. The remainder of his physical examination is unremarkable.
Can you spot the rash?
Diagnosis: Nevus spilus or speckled lentiginous nevus
A nevus spilus is believed to be a type of congenital melanocytic nevus that may be confused with a café-au-lait macule, especially early in life. A larger nevus spilus may also be referred to as a speckled lentiginous nevus (SLN), or in many cases, these names are used interchangeably.
Figure. A tan patch speckled with several variably sized dark brown macules and papules on the abdomen.
Source: Perman M
Nevi spili (NS) are usually present at birth or in early life but may evolve over time. The most common presentation is an asymptomatic, well-defined, linear, block-like or irregularly shaped tan patch studded with several darker brown or black macules or papules. NS often obey the mid-line, usually evolve over time, and grow with the patient similar to congenital melanocytic nevi. Any skin surface may be involved, although NS often favor the trunk or extremities. There is no known association with sun exposure.
NS can be divided into several clinical subtypes. Often, NS are classified by size similar to congenital melanocytic nevi, including small, intermediate and giant. Zosteriform presentations are also described. In addition, linear NS may follow Blaschko’s lines (the proposed embryologic pattern of skin cells). Schaffer and colleagues proposed that NS may evolve into more characteristic congenital melanocytic nevi or exist as a hybrid, further supporting the link between these two melanocytic nevi.
The macules and papules within NS may show several benign histopathologic findings, including lentigines, junctional, compound or dermal nevi. In addition, there are many reports of Spitz nevi occurring within NS. Other less commonly reported nevi found upon biopsy of the macules and papules include blue nevi, neural nevi and, rarely, melanoma.
Vidaurri-de la Cruz and Happle proposed that NS with macules tends to have a uniform speckled pattern with all of the macules being similar in size, whereas NS with papules tend to be more irregularly spaced and of varying sizes. Additionally, NS with macules may be associated with phakomatosis pigmentovascularis (specifically, phacomatosis spilorosea: a nevus spilus co-existing with a pale pink telangiectatic nevus), whereas NS with papules is found in association with phacomatosis pigmentokeratotica (a variant of the epidermal nevus syndrome with a co-existing epidermal nevus and speckled lentiginous nevus) and SLN syndrome — a segmental SLN in association with ipsilateral neuromuscular abnormalities and/or other central nervous system disorders — (Torchia and Schachner, 2011) .
Patients should be followed over time, with serial exams and photography for the evolution of the lesions, with a particular focus on the developing macules and papules. Concerning lesions should be biopsied. Elective prophylactic excision is not recommended in most cases, as the entirety of the lesion requires removal, and the sheer size of many NS would make complete excision and closure difficult.
References:
Fernandez-Flores A. Am J Dermatopathol. 2012;published online ahead of print June 12.
Happle R. Clin Exp Dermatol. 2009;34:133-135.
Schaffer JV. Arch Dermatol. 2001;137:172-178.
Torchia D. Pediatr Dermatol. 2011;28:749.
Vidaurri-de la Cruz H. Dermatology. 2006;212:53-58.