Clinical Challenge: Crusted Papules and Scarring on Scalp

Dissecting cellulitis of the scalp (DCS) is a chronic and recurrent inflammatory disease of the hair follicles, which is also referred to as Hoffman disease or perifolliculitis capitis abscedens et suffodiens. It is a component of the follicular occlusion tetrad...

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Dissecting cellulitis of the scalp (DCS) is a chronic and recurrent inflammatory disease of the hair follicles, which is also referred to as Hoffman disease or perifolliculitis capitis abscedens et suffodiens. It is a component of the follicular occlusion tetrad that includes hidradenitis suppurativa, acne conglobata, and pilonidal disease.¹ These conditions are believed to result from loss of immune tolerance to alloantigens in the hair follicle, which leads to an inflammatory reaction.²

In the United States, DCS predominantly occurs in Black men between the ages of 20 to 40 years; the condition is rare in other races and in women.³ The etiology is unknown; however, both genetic predisposition and environmental factors are thought to play roles in susceptibilty.⁴ The pathogenesis of DCS is linked to occlusion of the pilosebaceous unit and follicular hyperkeratosis with subsequent follicular dilatation and rupture resulting in a neutrophilic and granulomatous inflammatory response.⁵

The incidence of DCS is underreported, likely because of diagnostic confusion with other dermatological conditions.⁶ Diseases that mimic DCS include acne keloidalis nuchae, pseudopelade, tinea capitis, and folliculitis decalvans.⁷ Dissecting cellulitis of the scalp may be associated with sternoclavicular hyperostosis, polyarticular arthritis, and human leukocyte antigen B27 (HLA-B27) seronegative spondyloarthropathies.⁸

Dissecting cellulitis of the scalp presents as firm or fluctuant tender papules and pustules arising on the vertex and occipital regions. Formation of interconnecting draining sinus abscesses with scarring alopecia and keloids mark disease progression.⁵ Although the abscesses are sterile, secondary bacterial infection with organisms such as Pseudomonas species, Staphylococcus epidermidis, Propionibacterium acnes, and Prevotella intermedia may occur.⁶

Treatment of DCS is often challenging and is usually not curative. No treatment option has been demonstrated superior to the others.⁹ Oral antibiotics are commonly used as first-line treatment and include doxycycline, azithromycin, ciprofloxacin, and the combination of rifampin and clindamycin.⁵ Improvement with isotretinoin is often noted; however, cases frequently relapse when therapy is discontinued.¹⁰ Tumor necrosis factor (TNF)-α inhibitors such as adalimumab have resulted in good outcome.¹¹

In severe cases refractory to medical therapy, targeted surgical interventions or a broad approach that includes scalpectomy with split-thickness skin grafting have produced favorable results.¹ Because DCS can lead to disfigurement and significant psychological distress, early resection has been advocated by some clinicians; long-term outcome requires more study.¹²

Alexandra Stroia, BS, is a medical student at the Lake Erie College of Osteopathic Medicine in Erie, Pennsylvania. Stephen Schleicher, MD, is director of the DermDox Dermatology Centers, associate professor of medicine at Geisinger Commonwealth Medical College, and clinical instructor of dermatology at Arcadia University and Kings College.

References

1. Cuellar TA, Roh DS, Sampson CE. Dissecting Cellulitis of the Scalp: A Review and Case Studies of Surgical Reconstruction. Plast Reconstr Surg Glob Open. 2020;8(8):e3015. Published 2020 Aug 18. doi:10.1097/GOX.0000000000003015

2. Segurado-Miravalles G, Camacho-Martínez FM, Arias-Santiago S, et al. Epidemiology, clinical presentation and therapeutic approach in a multicentre series of dissecting cellulitis of the scalp. J Eur Acad Dermatol Venereol. 2017; 31:e199–e200 

3. Scheinfeld N. Dissecting cellulitis (Perifolliculitis Capitis Abscedens et Suffodiens): a comprehensive review focusing on new treatments and findings of the last decade with commentary comparing the therapies and causes of dissecting cellulitis to hidradenitis suppurativa. Dermatol Online J. 2014;20(5):22692. Published 2014 May 16.

4. Melo DF, Lemes LR, Pirmez R, Duque-Estrada B. Trichoscopic stages of dissecting cellulitis: a potential complementary tool to clinical assessment. An Bras Dermatol. 2020;95(4):514-517. doi:10.1016/j.abd.2019.10.008

5. Alsantali A, Almalki B, Alharbi A. Recalcitrant Dissecting Cellulitis of the Scalp Treated Successfully with Adalimumab with Hair Regrowth: A Case Report. Clin Cosmet Investig Dermatol. 2021;14:455-458. Published 2021 May 10. doi:10.2147/CCID.S301451

6. Syed TA, Ul Abideen Asad Z, Salem G, Garg K, Rubin E, Agudelo N. Dissecting Cellulitis of the Scalp: A Rare Dermatological Manifestation of Crohn’s Disease. ACG Case Rep J. 2018;5:e8. Published 2018 Jan 31. doi:10.14309/crj.2018.8

7. Skibinska M. Perifolliculitis Capitis Abscedens et Suffodiens: Background, Etiology and Pathophysiology, Prognosis. Emedicine.medscape.com. https://emedicine.medscape.com/article/1072603-overview. Published 2018. Accessed April 6, 2022.

8. Jerome MA, Laub DR. Dissecting cellulitis of the scalp: case discussion, unique considerations, and treatment options. Eplasty. 2014;14:ic17.

9. Thomas J, Aguh C. Approach to treatment of refractory dissecting cellulitis of the scalp: a systematic review. J Dermatolog Treat. 2021; 32:144–149

10. Guo W, Zhu C, Stevens G, Silverstein D. Analyzing the Efficacy of Isotretinoin in Treating Dissecting Cellulitis: A Literature Review and Meta-Analysis. Drugs R D. 2021;21(1):29-37. doi:10.1007/s40268-020-00335-y

11. Takahashi T, Yamasaki K, Terui H, et al. Perifolliculitis capitis abscedens et suffodiens treatment with tumor necrosis factor inhibitors: a case report and review of published cases. J Dermatol. 2019;46(9):802–807. doi:10.1111/1346-8138.14998

12. Hintze JM, Howard BE, Donald CB, Hayden RE. Surgical Management and Reconstruction of Hoffman’s Disease (Dissecting Cellulitis of the Scalp). Case Rep Surg. 2016;2016:2123037. doi:10.1155/2016/2123037