Cerebriform intradermal nevus presenting as cutis verticis gyrata with multiple cellular blue nevus over the body: A rare occurrence
A 28-year-old male patient presented to the skin out-patient department with history of a huge progressively increasing swelling over the back of the scalp [] and multiple black nodules at various parts of the body. According to patient same kind of lesions were present over the trunk at birth, which were surgically removed when he was 4-year-old. The lesion over the scalp started at the age of 6-7 years and the truncal lesions developed gradually since then. On examination, there was a single bosselated irregular non-tender skin colored swelling of 15 cm × 20 cm with deep folds over the occipital region extending toward the nape of the neck. There were certain areas of depigmentation [ along with blue streaks over the swelling. The surface of the skin was hairless, the skin over the swelling was non-pinchable and the swelling was immobile. There was no skin erosion, dermatitis, infection, odor or tenderness.\nThere were multiple hyperpigmented nodules distributed over different areas of the body such as mandible, flanks, abdomen [], front and back of both thighs, right knee joint, lower leg [], right sole and over volar aspect of first and second finger of the left hand []. The size of the nodules ranged from 0.5 cm × 1 cm to 1.5 cm × 2.5 cm. The nodules were blackish brown in color surface was smooth and hairless. They were asymptomatic and non-tender on palpation. Nails and mucous membrane examination were within normal limits. He had normal intelligence (the full-scale intelligence quotient was 97) and he had no psychiatric problem. Examination of the neurological, ophthalmological and other systems was normal. Laboratory results, including venereal disease research laboratory test, serology for human immunodeficiency virus, growth hormone and thyroid function, were all negative or normal. A punch biopsy from the scalp lesion and an excision biopsy from the nodule over the body was performed. Section from the specimen showed multiple nests and cords of nevus cells in the upper dermis, which contained a moderate amount of melanin []. There was no cellular atypia. Section from the body lesion showed deeply pigmented dendritic melanocytes in addition to nests and fascicles of spindle shaped cells with abundant pale cytoplasm containing little melanin [Figures and ]. The spindle cells were arranged in a storiform pattern. Based on the clinical feature and histology it was diagnosed as a case of congenital melanocytic nevus with cellular blue nevi.