Tumor of the follicular infundibulum with its characteristic histopathologic manifestations is a well-recognized entity nowadays. Our case further expands the constellation of the clinical presentation of the multiple variant. Although the possibility of malignant basocellular degeneration seems remote, the multiplicity of the lesions, the possibility of clinical overlook, and the impracticality of complete treatment makes regular follow-up rational.

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Background: Pigmented dermatofibrosarcoma is a rare tumor of the skin and constitutes 1–5% of all dermatofibrosarcoma. Most cases present as polypoid multinodular growth. Occasional cases may be atrophic. We report an extraordinary case with progressive infraorbital atrophy. Observation: A 24-year-old woman came to us for a diffuse bluish atrophic lesion over the left infraorbital area. The lesion progressed gradually over 2 years. Histologic examination revealed mature spindle cell proliferation in the lower dermis and hypodermis. Interspersed were some heavily pigmented melanocytes. Conclusion: We report an unusual case of progressive bluish discoloration and atrophy of the infraorbital area. This is a rare manifestation of dermatofibrosarcoma.

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