accepted: marsupialization: a review published: copyright

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Journal of Dermatology and Clinical Research Cite this article: Meunier N, Zaleski L, Bloom D, Steger J (2014) Treatment of Dissecting Cellulitis of the Scalp and the Use of Marsupialization: A Review. J Dermatolog Clin Res 2(2): 1015. Central *Corresponding author Dr. Nicole Meunier, General Medical Officer for the 3rd Radio Battalion, Marine Corps Base Hawaii, USA, Tel: 808-397-7587; Email: Submitted: 22 January 2014 Accepted: 13 February 2014 Published: 15 February 2014 Copyright © 2014 Meunier et al. OPEN ACCESS Keywords • Cellulitis • Scalp • Marsupialization • Follicular occlusion triad Case Report Treatment of Dissecting Cellulitis of the Scalp and the Use of Marsupialization: A Review Nicole Meunier 1 *, Lisa Zaleski 2 , David Bloom 3 and James Steger 4 1 General Medical Officer for the 3rd Radio Battalion, Marine Corps Base Hawaii, USA 2 Department of Dermatology, Naval Hospital Okinawa, Japan 3 Department of Ear Nose and Throat, Naval Hospital Okinawa, Japan 4 Department of Dermatology, Naval Medical Center San Diego, USA Abstract Dissecting cellulitis of the scalp (DCS), or perifolliculitis capitis abscedens et suffodiens, is a rare, chronic, relapsing and remitting inflammatory disease of the scalp. We present a case of a patient with severe DCS refractory to medical management on chronic high-dose prednisone. We describe the procedure of marsupialization under general anesthesia which may be an effective treatment option for severe cases of DCS. ABBREVIATIONS DCS: Dissecting Cellulitis of the Scalp INTRODUCTION Dissecting cellulitis of the scalp (DCS), or perifolliculitis capitis abscedens et suffodiens, is a rare, chronic, relapsing and remitting inflammatory disease of the scalp. DCS was first described by Spitzer in 1903 and named perifolliculitis capitis abscedens et suffodiens by Eric Hoffman in 1908. The condition is often referred to in the European literature as Hoffman’s disease and was renamed DCS by Barney in 1931 [1,2]. DCS is described as one component of the follicular occlusion triad, along with hidradenitis suppurativa and acne conglobata [3]. DCS most often occurs on the vertex and occipital scalp of African American males between 20 and 40 years of age [4]. The disease is characterized by firm or fluctuant subcutaneous nodules and burrowing abscesses that initially cause a potentially reversible overlying alopecia but eventually result in dense dermal fibrosis, chronic sinus tract formation, hypertrophic scarring and scarring alopecia [5]. Multiple therapeutic modalities have been described with often disappointing outcomes. We describe a case of severe DCS refractory to medical management with symptom flares while on high-dose prednisone. Marsupialization was performed under general anesthesia on selected areas of the occipital scalp with significant symptom relief. CASE PRESENTATION Patient history A 29 year old African American male with hidradenitis suppurativa and cystic acne presented with a five year history of painful scalp cysts. Of his follicular occlusion triad, the dissecting cellulitis of the scalp was his primary complaint and the worst of all of his symptoms. The patient completed multiple courses of antibiotics including trials of doxycycline, minocycline, trimethoprim/sulfamethoxazole DS, and clindamycin orally twice daily. These medications were used in conjunction with topical clindamycin solution and benzoyl peroxide 10% wash with no significant change in the patient’s symptoms. Isotretinoin (13-cis-retinoic acid) 80 mg daily was then attempted but the patient reported a severe worsening of symptoms and declined further treatment. Given the lack of success with multiple antibiotic regimens and isotretinoin, the patient was started on a trial of the anti-tumor- necrosis factor-alfa inhibitor etanercept 25 mg biweekly. There was mild improvement in the first three weeks, but no sustained response was observed over a two month period. The patient was unable to complete a 4 month trial period due to military travel and the inability to keep the etanercept refrigerated. The patient refused 1064 nm laser hair removal treatments. High dose prednisone at 80 mg daily was subsequently initiated due to constant 8/10 scalp pain. The first sign of dramatic change occurred after four weeks of prednisone therapy, but this improvement was transient. Despite high-dose prednisone of at least 60 mg orally daily over a year timeframe, the patient continued to experience flares. Combination therapy was attempted with oral clindamycin and etanercept for 3 months, clindamycin and rifampin for 6 weeks, and dapsone for 3 months. No sustained benefit with any of the above regimens was observed. The patient desired a more permanent solution to his symptoms and consented to a marsupialization of the scalp.

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Journal of Dermatology and Clinical Research

Cite this article: Meunier N, Zaleski L, Bloom D, Steger J (2014) Treatment of Dissecting Cellulitis of the Scalp and the Use of Marsupialization: A Review. J Dermatolog Clin Res 2(2): 1015.

Central

*Corresponding authorDr. Nicole Meunier, General Medical Officer for the 3rd Radio Battalion, Marine Corps Base Hawaii, USA, Tel: 808-397-7587; Email:

Submitted: 22 January 2014

Accepted: 13 February 2014

Published: 15 February 2014

Copyright© 2014 Meunier et al.

OPEN ACCESS

Keywords• Cellulitis• Scalp• Marsupialization• Follicular occlusion triad

Case Report

Treatment of Dissecting Cellulitis of the Scalp and the Use of Marsupialization: A ReviewNicole Meunier1*, Lisa Zaleski2, David Bloom3 and James Steger4

1General Medical Officer for the 3rd Radio Battalion, Marine Corps Base Hawaii, USA2Department of Dermatology, Naval Hospital Okinawa, Japan3Department of Ear Nose and Throat, Naval Hospital Okinawa, Japan4Department of Dermatology, Naval Medical Center San Diego, USA

Abstract

Dissecting cellulitis of the scalp (DCS), or perifolliculitis capitis abscedens et suffodiens, is a rare, chronic, relapsing and remitting inflammatory disease of the scalp. We present a case of a patient with severe DCS refractory to medical management on chronic high-dose prednisone. We describe the procedure of marsupialization under general anesthesia which may be an effective treatment option for severe cases of DCS.

ABBREVIATIONSDCS: Dissecting Cellulitis of the Scalp

INTRODUCTIONDissecting cellulitis of the scalp (DCS), or perifolliculitis

capitis abscedens et suffodiens, is a rare, chronic, relapsing and remitting inflammatory disease of the scalp. DCS was first described by Spitzer in 1903 and named perifolliculitis capitis abscedens et suffodiens by Eric Hoffman in 1908. The condition is often referred to in the European literature as Hoffman’s disease and was renamed DCS by Barney in 1931 [1,2]. DCS is described as one component of the follicular occlusion triad, along with hidradenitis suppurativa and acne conglobata [3]. DCS most often occurs on the vertex and occipital scalp of African American males between 20 and 40 years of age [4]. The disease is characterized by firm or fluctuant subcutaneous nodules and burrowing abscesses that initially cause a potentially reversible overlying alopecia but eventually result in dense dermal fibrosis, chronic sinus tract formation, hypertrophic scarring and scarring alopecia [5]. Multiple therapeutic modalities have been described with often disappointing outcomes. We describe a case of severe DCS refractory to medical management with symptom flares while on high-dose prednisone. Marsupialization was performed under general anesthesia on selected areas of the occipital scalp with significant symptom relief.

CASE PRESENTATIONPatient history

A 29 year old African American male with hidradenitis suppurativa and cystic acne presented with a five year history of

painful scalp cysts. Of his follicular occlusion triad, the dissecting cellulitis of the scalp was his primary complaint and the worst of all of his symptoms. The patient completed multiple courses of antibiotics including trials of doxycycline, minocycline, trimethoprim/sulfamethoxazole DS, and clindamycin orally twice daily. These medications were used in conjunction with topical clindamycin solution and benzoyl peroxide 10% wash with no significant change in the patient’s symptoms. Isotretinoin (13-cis-retinoic acid) 80 mg daily was then attempted but the patient reported a severe worsening of symptoms and declined further treatment.

Given the lack of success with multiple antibiotic regimens and isotretinoin, the patient was started on a trial of the anti-tumor-necrosis factor-alfa inhibitor etanercept 25 mg biweekly. There was mild improvement in the first three weeks, but no sustained response was observed over a two month period. The patient was unable to complete a 4 month trial period due to military travel and the inability to keep the etanercept refrigerated. The patient refused 1064 nm laser hair removal treatments.

High dose prednisone at 80 mg daily was subsequently initiated due to constant 8/10 scalp pain. The first sign of dramatic change occurred after four weeks of prednisone therapy, but this improvement was transient. Despite high-dose prednisone of at least 60 mg orally daily over a year timeframe, the patient continued to experience flares. Combination therapy was attempted with oral clindamycin and etanercept for 3 months, clindamycin and rifampin for 6 weeks, and dapsone for 3 months. No sustained benefit with any of the above regimens was observed.

The patient desired a more permanent solution to his symptoms and consented to a marsupialization of the scalp.

Meunier et al. (2014)Email:

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Telemedicine consultation with one of the authors (Steger) who had extensive successful experience using marsupialization of the scalp resulted in reassurance that this technique had great promise for this patient. After further discussion with the patient, he consented to marsupialization of the involved areas of the scalp.

Procedure

Preoperative pictures were obtained shown in Figure 1. The patient was placed under general anesthesia in the operating room. The affected occipital scalp was injected with 10 ml of 1% lidocaine with 1: 100, 000 epinephrine. An open sinus was identified and a metal eyelet probe was inserted. The Bovie was used on cutting mode at a setting of 15 to open the entire length of the sinus tract in both directions. The Bovie was also used to remove any free skin on the entire roof of the sinus tract. The base of the tract was curetted to remove the granulation tissue.

A probe was then used to explore the valley created and identify connections to other areas. These sinus tracts were marsupialized in the same manner. This method was repeated until the majority of the most frequently flared regions had been treated. Approximately 20% of his scalp was marsupialized and the rest was left for a subsequent marsupialization. Hemostasis was achieved with electrocautery. Each marsupialized area was then packed with gelfoam and a compressive dressing was applied overnight.

Our patient followed up on post operative day 1 appearing as shown in Figure 2A, B, and C and then followed up weekly thereafter. The wounds were cleansed with normal saline daily and then a mixture of aquaphor and silver sulfadiazine was placed into the wounds. A telfa pad was placed over the wounds and then wrapped with curlex gauze. Percocet was prescribed for pain as needed. The patient’s wife is a nurse and was able to help the patient with bandage changes daily at home.

By post operative day 34 of the first marsupialization procedure, the wounds were mostly healed as shown in Figures 3A, B, and C. The wounds were completely healed by post operative day 60 as shown in Figures 4A, B, and C. The patient has not had any DCS flares in the marsupialized regions that were widely excised since his surgery. There were 5 areas on the left scalp where smaller areas of the scalp had been marsupialized. These regions returned with flares and were treated with a larger excision during his second marsupialization.

The second marsupialization procedure was completed on post operative day 90 with the same procedures followed as the

Figure 1 Preoperative view of occipital scalp.

Figure 2 Post operative day 1 from the first marsupialization procedure.

Figure 3 Post operative day 34 from the first marsupialization procedure.

Figure 4 Post operative day 60 from the first marsupialization procedure. In figure 4A note the recurrence of pus in the regions previously treated with smaller resections.

Figure 5 Post operative day 4 from the second marsupialization procedure.

prior marsupialization. Figures 5A, B, and C show the patient on post operative day 4 from the second marsupialization procedure where larger areas were marsupialized. Figures 6A, B, and C demonstrate the filling of the surgical defects by post operative day 34. The same recovery period was seen with the second procedure with excellent patient satisfaction as shown in Figures 7A, B, and C. Figures 8A, B, and C show continued wound healing and no disease recurrence in the treated areas 7 months after the second marsupialization procedure.

DISCUSSIONDCS is a rare, chronic, relapsing and remitting inflammatory

disease. The etiology remains unclear, however the mechanism appears to be related to follicular hyperkeratosis and occlusion.

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Multiple treatment modalities have been described with varying degrees of success [6]. Nonsurgical therapies include topical and systemic antibiotics, systemic steroids, and systemic retinoids.

Monotherapy with antibiotics has not been reported with sustained success. Doxycycline, erythromycin, minocycline and flucoxacillin are among other antibiotics that have been unsuccessful in achieving consistent symptom control [7,8]. A case of systemic steroids initiated at a high dose with rapid transition to alternate-day dosing was described. The patient remained free of disease activity after 12 months, however remained on the alternate-day dosing of prednisone [9].

Isotretinoin appears to be the most efficacious among nonsurgical treatment modalities, with several successful cases described after prolonged treatment courses of 9 to 11 months. Two-thirds of the cases reported in one study demonstrated sustained therapeutic benefit after a nine month course of isotretinoin at 1 mg/kg/day dosing. One patient had no scalp disease and complete hair re-growth two and a half years after treatment [7].

Isotretnoin has also been used in conjunction with rifampin. In 2 of 4 reported cases, there was no recurrence noted 10 months after treatment. However, biopsies taken along the edge of the active lesions demonstrated persistent histological features consistent with dissecting cellulitis [10].

Several cases have also been reported on the use of anti-

TNF alpha therapy. With infliximab, one successful case was reported with complete remission that remained stable for at least 12 months [11]. Another case demonstrated near complete remission at a 3 month follow up, but the authors described their results as palliative rather than curative. In two of three patients treated with adalimumab, histological evidence of residual structures including subcutaneous sinus tracts remained. Symptoms relapsed in one case after the therapeutic agent was discontinued [12].

X-ray therapy was used in the 1960s, but due to increased risk of skin cancer it is no longer a primary treatment option [13]. More recently, hair follicle epilation with various lasers including the 800-nm pulsed-diode laser has been reported with good results in regards to symptom control. However, the absence of or minimal hair regrowth is a common result of laser therapy [14,15].

In one case study, the patient underwent four treatment sessions at four week intervals using an 800-nm diode laser. At a six month follow-up the patient’s scalp condition improved but there was no hair regrowth [14]. The largest study to date using the pulsed-diode laser included 38 men and women. Patients were given up to four treatments. There were no reported cases of infection, textural changes, dyschromia, or scarring, however fifty-nine percent reported sparse regrowth with hair that tended to be thinner and lighter in color [15].

The long-pulsed Nd:YAG laser has more recently been utilized without significant adverse cutaneous side effects. Four patients were treated with three to seven monthly treatments over one year with the 1,064-nm Nd:YAG laser. Three patients had at least partial hair regrowth at treatment sites. Further, three of the patients were able to stop or decrease the need for systemic medications [8].

The most effective surgical interventions to date in the treatment of DCS is excisional surgery or scalpectomy followed by split-thickness grafting. Local incision and drainage has been employed, but at most provides temporary symptom relief. Treatment with scalpectomy was first reported in 1986 with subsequent publication of successful cases. One case was reported to have no recurrence at nine months post-operatively [16-18].

The use of marsupialization in DCS cases has not been widely published, with only two cases printed of discussions that occurred during regional dermatology society meetings in 1943 and 1960 [19,20]. In our case the patient failed multiple trials of topical and oral antibiotics, was unable to tolerate isotretinoin, failed anti-TNF alpha therapy and multiple combination therapies. The only significant improvement was transient with the initiation of high-dose prednisone, but recurrent and severe symptom flares significantly delayed attempts at a prednisone taper. The patient declined laser therapy because he was concerned about the potential for permanent hair loss. Marsupialization compares favorably to existing treatment options and was the leading option in this case as it offered a more permanent outcome for the patient’s severe and recalcitrant symptoms. It is a significantly less invasive surgery than complete scalpectomy that can heal remarkably well by secondary intention without the

Figure 6 Post operative day 34 from the second marsupialization procedure.

Figure 7 Post operative day 63 from the second marsupialization procedure.

Figure 8 7 months after the second marsupialization procedure.

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requirement for split-thickness grafting. While marsupialization scars continue to contract and remodel over a year’s time, diffusely affected areas of the scalp may be difficult to conceal. In areas of the scalp with less extensive disease, scarring can be hidden by hair styling.

Our patient tolerated the procedure well with early symptom relief. He remains symptom free in the treated regions 7 months post surgery. Despite the patient’s initial concern about hair loss, he is very satisfied with the significant improvement in his symptoms and has noticed gradual improvement in his scarring over the past several months. New lesions on the frontal scalp have since occurred in areas that have not been treated. Our patient continues to be on clindamycin 300 mg twice daily and oral hydrocortisone 40 mg daily due to his groin hidradenitis. The prednisone had been changed to hydrocortisone due to endocrinology recommendations for ease of weaning off of oral steroids. A course of isotretinoin is planned as the next course of action but has been delayed due to the desire to have another child. Lessons learned from the case are summarized in Table 1.

In summary, DCS is often difficult and frustrating to treat. However, marsupialization can be a powerful tool in controlling and clearing it. It is our hope that this report will increase awareness of marsupialization as a significant treatment option for this disfiguring and discouraging scalp affliction.

DISCLOSUREThe views expressed in this article are those of the authors

and do not reflect the official policy of the Department of the Navy, Department of Defense, or U.S. Government. This work has not been published elsewhere.

REFERENCES1. Spitzer L. Dermatitis follicularis et perifollicularis conglobata.

Dermatol Ztschr. 1903; 10: 109.

2. Barney RE. Dissecting cellulitis of the scalp. Arch Dermatol Syph. 1931; 23: 503.

3. Chicarilli ZN. Follicular occlusion triad: hidradenitis suppurativa, acne

Gel Foam

-A small amount is enough to provide post-operative hemostasis if the scalp hemorrhage is well controlled with electro-cauterization during surgery. -A single layer of gel foam cut to the exact size and shape of the surgical wound is optimum. -If slightly larger, the gel foam can be compacted into the wound.

Sinus tract removal

-it is of tant amount importance to remove all of the sinus tracts connecting into each unroofed abscess or the DCS will reform in that area and expand to other areas. -Although larger marsupializations of the scalp may take longer to heal, if performed correctly, application of 1% silver nitrate (for antimicrobial activity) + 5% balsam of Peru (for accelerated reepithelialization) compounded in Aquaphor provides an excellent dressing.-If unavailable, the use of silver containing wound dressing is highly recommended.

Recurrence of symptoms

-Recurrence in completely marsupialized areas is quite rare [personal experience of Dr. Steger]. -However, new unconnected adjacent tracts may form in these patients because of their personal susceptibility to this condition, but these new areas respond to subsequent marsupializations. -The reason standard surgical approaches fail, short of a more radical scalpectomy, is their inability to identify incoming sinus tracts intraoperatively.

Wound healing Despite the extensive wounds seen immediately postoperatively, marsupialization with secondary healing is in reality a tissue sparing procedure via finding, unroofing, and removing any diseased tissue.

Comparison with medical management

Although perhaps beneficial at times, traditional combination therapies with antibiotics, intralesional or systemic steroids, retinoids, and biologics typically fail sooner or later, but can provide temporary benefit while awaiting more definitive surgical therapies.

Table 1: Lessons Learned.

conglobata, and dissecting cellulitis of the scalp. Ann Plast Surg. 1987; 18: 230-237.

4. Moyer DG, Williams RM. Perifolliculitis capitis abscedens et suffodiens. A report of six cases. Arch Dermatol. 1962; 85: 378-384.

5. Sperling LC. Scarring alopecia and the dermatopathologist. J Cutan Pathol. 2001; 28: 333-342.

6. Mundi JP, Marmon S, Fischer M, Kamino H, Patel R, Shapiro J. Dissecting cellulitis of the scalp. Dermatol Online J. 2012; 18: 8.

7. Scerri L, Williams HC, Allen BR. Dissecting cellulitis of the scalp: response to isotretinoin. Br J Dermatol. 1996; 134: 1105-1108.

8. Krasner BD, Hamzavi FH, Murakawa GJ, Hamzavi IH. Dissecting cellulitis treated with the long-pulsed Nd:YAG laser. Dermatol Surg. 2006; 32: 1039-1044.

9. Adrian RM, Arndt KA. Perifolliculitis capitis: successful control with alternate-day corticosteroids. Ann Plast Surg. 1980; 4: 166-169.

10. Georgala S, Korfitis C, Ioannidou D, Alestas T, Kylafis G, Georgala C. Dissecting cellulitis of the scalp treated with rifampicin and isotretinoin: case reports. Cutis. 2008; 82: 195-198.

11. Wollina U, Gemmeke A, Koch A. Dissecting Cellulitis of the Scalp Responding to Intravenous Tumor Necrosis Factor-alpha Antagonist. J Clin Aesthet Dermatol. 2012; 5: 36-39.

12. Navarini AA, Trüeb RM. 3 cases of dissecting cellulitis of the scalp treated with adalimumab: control of inflammation within residual structural disease. Arch Dermatol. 2010; 146: 517-520.

13. McMullan FH, Zeligman I. Perifolliculitis capitis abscedens et suffodiens; its successful treatment with x-ray epilation. AMA Arch Derm. 1956; 73: 256-263.

14. Boyd AS, Binhlam JQ. Use of an 800-nm pulsed-diode laser in the treatment of recalcitrant dissecting cellulitis of the scalp. Arch Dermatol. 2002; 138: 1291-1293.

15. Campos VB, Dierickx CC, Farinelli WA, Lin TY, Manuskiatti W, Anderson RR. Hair removal with an 800-nm pulsed diode laser. J Am Acad Dermatol. 2000; 43: 442-447.

16. Williams CN, Cohen M, Ronan SG, Lewandowski CA. Dissecting cellulitis of the scalp. Plast Reconstr Surg. 1986; 77: 378-382.

17. Housewright CD, Rensvold E, Tidwell J, Lynch D, Butler DF. Excisional

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Meunier N, Zaleski L, Bloom D, Steger J (2014) Treatment of Dissecting Cellulitis of the Scalp and the Use of Marsupialization: A Review. J Dermatolog Clin Res 2(2): 1015.

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surgery (scalpectomy) for dissecting cellulitis of the scalp. Dermatol Surg. 2011; 37: 1189-1191.

18. Arneja JS, Vashi CN, Gursel E, Lelli JL. Management of fulminant dissecting cellulitis of the scalp in the pediatric population: Case report and literature review. Can J Plast Surg. 2007; 15: 211-214.

19. Cannon AB. Perifolliculitis capitis abscedens et suffodiens. Arch Dermatol. 1944; 49: 67-68.

20. Wolf M. Diagnosis: Perifolliculitis capitis abscedens and suffodiens. Arch Dermatol. 1960; 81: 1016-1017.