Laboratory Studies

Histopathologic examination of skin changes can reveal acanthosis nigricans.

Imaging Studies

Imaging studies are described below.^{[1, 3, 4, 6, 38]}

Skull, spine, and hand radiography is usually necessary to confirm the diagnosis.

Skull radiography reveals the following:

Oxycephaly - Acrocranium (most characteristic), obliterated sutures (mostly coronal, sagittal)
Deep digitate impression, shallow eye sockets (exophthalmos)
Turkish saddle deepened with osseous sternum connecting anterior and posterior clinoid processes
Shortened anterior cranial fossa
Cranial base deformity; develops sequentially from anterior to posterior ^[40]
Underdeveloped lateral nasal sinuses
Tympanic membranes fixed oblique, narrowed external auditory canals, absence of mastoid processes pneumatization, and small pyramids with symptoms of sclerosis

On spine radiography, lumbarization and the presence of bifid spinous process are possible, and slight symptoms of achondroplasia may be visible.

Radiographic examination of the metacarpal bones and fingers reveals slight achondroplasia.

Two- and three-dimensional ultrasonography have been used to screen for symptoms associated with the disorder prenatally, but genetic testing would be required to confirm the results.

Other Tests

Other tests may include the following:

Ophthalmologic examination with ophthalmoscopy
Laryngologic examinations with audiography
General physical examination with ECG
Psychiatric examinations and psychological testing
Stomatologic examination
EEG - Low-voltage, increased convulsive excitability
Genetic testing

Histologic Findings

Histologic features of acanthosis nigricans include hyperkeratosis, acanthosis, and papillomatosis. In the basal layer, the amount of pigment cells is also increased in the upper dermis. No difference exists in histologic features of acanthosis nigricans among symptomatic, benign, and malignant forms.

Treatment & Management

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Media Gallery

Acrocephaly, beaked nose, "frog face," and pseudoprognathism of Crouzon syndrome.
Malposed teeth and acanthosis nigricans around the mouth in Crouzon syndrome.
No physiological spinal curvature is observed in Crouzon syndrome.
Acanthosis nigricans of the axillary fossa in Crouzon syndrome.
Short stature and dark skin of Crouzon syndrome.

of 5

Author

Amarateedha Prak LeCourt, MD Resident Physician, Department of Anesthesiology, Naval Medical Center San Diego; Naval Flight Surgeon/Fleet Marine Force Qualified Officer, US Navy

Amarateedha Prak LeCourt, MD is a member of the following medical societies: Aerospace Medical Association, Association of Military Surgeons of the US

Disclosure: Nothing to disclose.

Coauthor(s)

Kristina Marie Dela Rosa, MD Dermatologist, Insight Dermatology, San Diego, CA

Kristina Marie Dela Rosa, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association

Disclosure: Nothing to disclose.

Homavirak Prak, BA Emergency Medical Technician, Basic AirCare Ambulance; Medical Scribe, Progeny Psychiatric Group

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.

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Jacek C Szepietowski, MD, PhD Professor, Vice-Head, Department of Dermatology, Venereology and Allergology, Wroclaw Medical University; Director of the Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Poland

Disclosure: Received consulting fee from Orfagen for consulting; Received consulting fee from Maruho for consulting; Received consulting fee from Astellas for consulting; Received consulting fee from Abbott for consulting; Received consulting fee from Leo Pharma for consulting; Received consulting fee from Biogenoma for consulting; Received honoraria from Janssen for speaking and teaching; Received honoraria from Medac for speaking and teaching; Received consulting fee from Dignity Sciences for consulting; .

Grazyna Szybejko-Machaj, MD, PhD Assistant Professor, Department of Dermatology, Wroclaw Medical University

Disclosure: Nothing to disclose.

Lukasz Matusiak, MD, PhD Assistant, Department and Clinic of Dermatology, Venereology and Allergology, Medical University of Wroclaw

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Mieczyslawa Miklaszewska, MD, to the development and writing of this article.

Crouzon Syndrome Workup

Laboratory Studies

Imaging Studies

Other Tests

Histologic Findings

References

Tables

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