The patient is a 30 month old girl with a 15 month history of a papule on the left ala nasi. Her father has Hereditary Leiomyoma Renal Cell Carcinoma syndrome (HLRCC) and the patient is positive for the gene. The question is whether this papule is a possible sign of the HLRCC syndrome or an unrelated entity.
The tumor is 3-4 mm in diameter and has been growing very slowly. The child is in good general health otherwise. The lesion is red in color, regular in outline. Clinical and dermoscopic pictures are presented. (I am new to dermascopic photography and it was difficult to focus on this area in a cooperative but somewhat squirmy child.)
Your thoughts will be appreciated.
Dear David,
ReplyDeleteI am more confident about diagnosing a Spitz nevus when it is pigmented rather than with a pink lesion like this! It could be a Spitz nevus but it could also be a vascular lesion. It looks benign though.
I agree with Ian about Spitz nevus.
ReplyDelete“A dermatologist can approach to skin lesions through different ways; approach to the shape, pattern, location, color and... are among them. In a color-based approach Spitz nevus could be the most likely diagnosis for this kid. Juvenile xanthogranuloma is in my DDx list. I'm not aware of the importance of that gene, but i think biopsy is a need (at least for quenching of our thirst to know the diagnosis).”
ReplyDelete1. JXG
ReplyDelete2. angiomatous lesion
3. Spitz tumor
4. I don't know, but it looks benign.
I have seen a Spitz naevus with very similar dermoscopy.
ReplyDeleteI would think JXG, Spitz nevus or angiofibroma. Ben Barankin
ReplyDeleteJXG
ReplyDelete