Clinically, ML-DDH presents as a solitary, several centimeter-sized, round or oval, erythematous to yellow-brown, atrophic plaque on the neck or upper trunk. Patients may complain of pain and itching within the rash. The skin lesion is congenital and can enlarge over many years with normal growth of the patient.

Diagnosis is based on clinical and histological examination. Histopathological examination showed a band-like CD34-positive hypocellular fibrotic area in the superficial reticular dermis, which did not spread to the deep reticular dermis and subcutaneous tissue. Orcein stain showed normal elastic fibers. S-100 and CD56 were negative.

• Congenital atrophic dermatofibrosarcoma protuberans
• Keloid
• Scar
• Fibrosarcoma

Treatment involves surgical excision of the lesion with subsequent biopsy to rule out a diagnosis of Congenital atrophic dermatofibrosarcoma protuberans. Recurrence is possible after excision.