Diagnostic Considerations

Few other conditions present with ulcerations or scars in the newborn period. Aplasia cutis congenita (ACC) must be differentiated from the erosions of epidermolysis bullosa; this differentiation is especially important because aplasia cutis congenita may be diagnosed alone before new lesions from epidermolysis bullosa become apparent.

Neonatal herpes infection may present with an erosion, often on the scalp, but rarely at delivery. Some lesions have also been attributed to fetal varicella infection.

Lesions are sometimes mistakenly attributed to birth trauma secondary to vacuum extraction, forceps, or fetal scalp monitor electrodes.

Setleis syndrome includes bitemporal aplasia cutis congenita with other facial cutaneous abnormalities and has been termed congenital ectodermal dysplasia of the face.

Transient bullous dermolysis of the newborn is a rare, benign, blistering disease evident at birth or shortly thereafter; this condition resolves during the first months of life leaving no dystrophic scars.

Differential Diagnoses

Workup

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Media Gallery

Aplasia cutis congenita on the scalp (most common location) shortly after birth.
Triplet areas of aplasia cutis congenita are common in infants with trisomy 13.
This area of healed aplasia cutis congenita is located in an area of nevus flammeus. Note the collarette of coarser hair at the margin of the defect.
Extensive aplasia cutis congenita on the scalp, extending down to the skull.
Bilateral involvement of the lower extremities in aplasia cutis congenita associated with fetus papyraceous.

of 5

Author

Chris G Adigun, MD, FAAD Director and Physician, Dermatology and Laser Center of Chapel Hill; Senior Medical Advisor, Klara

Chris G Adigun, MD, FAAD is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, Council for Nail Disorders, North Carolina Dermatology Association, North Carolina Medical Society

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Sciton; OrthoDermatologics; Lumenis; PhotonMD; Galderma<br/>Received income in an amount equal to or greater than $250 from: Sciton.

Coauthor(s)

Megan E Freeze MD Candidate, The Brody School of Medicine at East Carolina University

Megan E Freeze is a member of the following medical societies: American Medical Student Association/Foundation

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Van Perry, MD Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas School of Medicine at San Antonio

Van Perry, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Additional Contributors

Joy Wan, MD, MSCE Assistant Professor, Johns Hopkins University School of Medicine

Joy Wan, MD, MSCE is a member of the following medical societies: American Academy of Dermatology, Society for Pediatric Dermatology

Disclosure: Received research grant from: Pfizer .

Acknowledgements

Mark A Crowe, MD Assistant Clinical Instructor, Department of Medicine, Division of Dermatology, University of Washington School of Medicine

Mark A Crowe, MD is a member of the following medical societies: American Academy of Dermatology and North American Clinical Dermatologic Society

Disclosure: Nothing to disclose.