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Perifolliculitis Capitis Abscedens et Suffodiens

  • Author: Malgorzata D Skibinska, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Apr 12, 2016
 

Background

Perifolliculitis capitis abscedens et suffodiens (PCAS, or dissecting cellulitis of the scalp) is a therapeutically challenging suppurative scalp disease of unknown etiology. Spitzer first described the disease in 1903, and Hoffman named it descriptively in 1907 (suffodiens is from the Latin suffodio, meaning to dig under). (See Etiology and Pathophysiology, Treatment, and Medication.)[1, 2]

PCAS is an uncommon disease. It occurs predominantly in black males (see the images below), in their second to fourth decade of life, but the condition also has been reported in other races and in women. Clinically, patients develop perifollicular pustules, nodules, and abscesses, with interconnecting sinus tracts that drain pus or blood. PCAS usually runs a chronic course with unpredictable relapses, although spontaneous resolution may occur. (See Prognosis and Presentation.)[3, 4, 5, 6]

Perifolliculitis capitis abscedens et suffodiens iPerifolliculitis capitis abscedens et suffodiens in a black man. Painful cutaneous nodules and patchy alopecia.
Side view of a black man with painful cutaneous noSide view of a black man with painful cutaneous nodules and patchy alopecia, characteristic of perifolliculitis capitis abscedens et suffodiens.
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Etiology and Pathophysiology

The cause of PCAS has not been clearly defined. The disease is thought to result from occlusion of the pilosebaceous unit. Acne conglobata, hidradenitis suppurativa, and pilonidal cysts are frequent concomitant diseases, which, along with PCAS, are together referred to as the follicular occlusion triad (without pilonidal cysts) or tetrad.[5, 6]

With follicular occlusion, retention of material dilates the follicle, leading to rupture, with exposure of keratin to the skin and organisms. This, in turn, causes inflammation with a neutrophilic and granulomatous response.[3, 7, 8]

Bacterial infection likely develops secondarily, as most bacteriologic cultures are negative. The most frequently isolated pathogens are Staphylococcus aureus, S epidermidis, and S albus.

In three patients, keratosis-ichthyosis-deafness (KID) syndrome has been reported to be associated with the follicular occlusion triad.[9, 10, 11]

A single report describes the follicular occlusion triad and pyoderma gangrenosum in a 16-year-old patient.[12]

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Prognosis

The prognosis for complete recovery from PCAS is poor. The disease is not life threatening, but it is chronic and relapsing. Complications can include the following:

  • Squamous cell carcinoma - Development is a possibility in chronic, relapsing lesions[7, 13]
  • Permanent alopecia - Occurs in chronically inflamed, scarred areas
  • Marginal keratitis - Has been described as a possible clinical association in patients PCAS[14]
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Contributor Information and Disclosures
Author

Malgorzata D Skibinska, MD, PhD Locum Consultant Dermatologist, Department of Dermatology, Basildon University Hospital, UK

Malgorzata D Skibinska, MD, PhD is a member of the following medical societies: British Medical Association, Royal College of Physicians, Royal Society of Medicine, British Association of Dermatologists, British Society of Allergy and Clinical Immunology

Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

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.

Acknowledgements

Jeffrey J Miller, MD Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Joshua A Zeichner, MD Assistant Professor, Director of Cosmetic and Clinical Research,

Mount Sinai School of Medicine; Chief of Dermatology, Institute for Family Health at North General

Joshua A Zeichner, MD is a member of the following medical societies: American Academy of Dermatology, American Acne and Rosacea Society, and National Psoriasis Foundation

Disclosure: Valeant Consulting fee Consulting; Medicis Grant/research funds Other; Galderma Consulting fee Consulting; Promius Consulting fee Consulting; Pharmaderm Consulting fee Consulting; Onset Consulting fee Consulting

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Perifolliculitis capitis abscedens et suffodiens in a black man. Painful cutaneous nodules and patchy alopecia.
Side view of a black man with painful cutaneous nodules and patchy alopecia, characteristic of perifolliculitis capitis abscedens et suffodiens.
A white patient with painful nodules. Image used with permission from Medical Science Monitor, 2000, 6(3): 602-4.
A white patient with painful nodules after 3 months of isotretinoin treatment. Image used with permission from Medical Science Monitor, 2000, 6(3): 602-4.
Histopathologic picture of biopsy taken from a white patient with perifolliculitis capitis abscedens et suffodiens. Hematoxylin and eosin stain, original magnification 400X. Image used with permission from Medical Science Monitor, 2000, 6(3): 602-4.
 
 
 
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