Insight into evolution of a giant congenital nevomelanocytic nevus over 14 years.

Giant congenital nevomelanocytic nevus (GCNN) is a rare variant of congenital melanocytic nevus measuring >20 cm in size that often has a garment-like distribution. Regular follow up is recommended because of a risk of melanoma transformation of 4.6%. We report a 14-year-old boy with gradual regression of giant congenital melanocytic nevus over the left upper limb, chest, back and axilla, whom we have followed-up since birth. At birth, a hyperpigmented jet-black patch without hair was present over the left side of torso and upper limb including palms and nails. Follow up at the ages of 1, 5, 11 and 14 years showed progressive spontaneous regression of the nevus resulting in shiny atrophic skin, diffuse hypopigmentation, lentigo-like macules, nodules and arthrogryphosis of affected areas. Histopathology of the lesions on follow-up revealed absence of pigmented nevus cells in the regressing areas and thickened sclerotic collagen bundles.

Childhood onset dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow growing, locally aggressive dermal tumor of disputed histogenesis with a tendency for local recurrence. A 16-year-old female presented with hyperpigmented macules, papules, plaques, erythematous nodules and atrophic, indurated lesions ranging from a few millimeters to a few centimeters. Biopsy revealed thinned out epidermis and a diffuse dermal infiltration by fascicles of spindle cells arranged in storiform pattern. The pseudoseptae formed by the tumor cells interdigitating between the subcutaneous adipocytes resulted in a honeycomb pattern. Histochemical findings are helpful, but the chronicity, size, indolent nature, recurrence after surgery and histopathology are more important to arrive at a diagnosis. Wide and deep surgical excision is the treatment of choice.