Carbon Dioxide Laser Perforation and Extirpation of Steatocystoma Multiplex Waseem Bakkour, MD, MSc, MRCP, and Vishal Madan, MD, FRCP*

BACKGROUND Steatocystoma multiplex (SM) is a rare condition that presents as multiple dermal cysts, the appearance of which can have a significant impact on the patients’ quality of life. Treatment options for SM are limited to surgical excision. OBJECTIVE To present our experience of treating 8 SM cases using a novel approach that uses the carbon dioxide (CO2) laser and to explore patients’ views about the treatment. METHODS Patients were identified from our records. All patients had multiple lesions treated using the CO2 laser in the super pulse mode that punctured the cyst. This was followed by extirpating the cyst wall and its contents using a small Volkmann’s spoon. Treatment efficacy was assessed clinically and feedback from the patients was sought through a telephone interview. RESULTS All patients showed significant clinical improvement with minimal scarring and low recurrence rates. High levels of patient satisfaction, which helped to significantly improve their quality of life, were reported with the CO2 laser treatment. CONCLUSION CO2 laser perforation and extirpation is a successful approach for managing SM that results in high patient satisfaction. The authors have indicated no significant interest with commercial supporters.

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teatocystoma multiplex (SM) is a rare autosomal dominant condition characterized by multiple dermal cysts that originate from the pilosebaceous unit. It results from mutation in the keratin 17 gene; however, it can also be sporadic. It can be associated with eruptive vellus hair cysts and pachyonychia congenita Type 2.1,2 SM appears clinically as multiple, yellow to skin-color, dermal cysts, which can range from few millimeters to 20 mm or more in size. It commonly affects the trunk, proximal limbs, and axillae, but can be located anywhere on the skin including the face and genital area. The onset is usually in adolescence or in early adult life, but occasionally it can appear earlier to worsen at puberty. Histology shows dermal cysts lined by a thin stratified squamous epithelium with sebaceous lobules within the cyst wall.3,4

Although the lesions are usually asymptomatic, their appearance can have a significant adverse impact on patients’ quality of life. Furthermore, SM frequently becomes inflamed and painful. Treatment options for SM are limited, especially because of the impracticalities of treating a large number of lesions and consequent scarring. We present our experience in using a novel approach that uses the carbon dioxide (CO2) laser to treat SM in 8 patients. Materials and Methods We performed a retrospective case note review of all patients with SM treated at our tertiary laser unit over a period of 11 years, between 2002 and 2013. Treatment outcome was assessed clinically at follow-up.

*Both the authors are affiliated with the Manchester Laser Unit, Dermatology Centre, Salford Royal NHS Foundation Trust, Manchester, United Kingdom

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© 2014 by the American Society for Dermatologic Surgery, Inc. Published by Lippincott Williams & Wilkins ISSN: 1076-0512 Dermatol Surg 2014;40:658–662 DOI: 10.1111/dsu.0000000000000013

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BAKKOUR AND MADAN

Laser Treatment All patients were treated using a CO2 laser (Sharplan 40C or KLS Martin MCO 50plus). Individual cysts were identified and marked with a surgical marker. A test patch was treated after infiltration with local anesthetic (LA) using 2% lignocaine solution containing adrenaline (1:200,000) (Xylocaine Astra Zeneca UK Limited). If patients were satisfied with the result, further treatments were performed either under LA or general anesthetic (GA). The CO2 laser was used in the super pulse mode (0.8–1.5 W, repetition mode) 2 to 4 pulses in focus to create a tiny perforation in the cyst. A small Volkmann’s/Chalazion spoon (Figure 1) was then inserted through the perforation to extirpate the cyst contents, and rotational movement allowed removal of the cyst wall. The resultant wound was left to heal secondarily. Topical mupirocin ointment (Bactroban) was applied to all treated areas immediately postoperatively, but no dressing was used. Oral antibiotics were not used in any patients. Telephone Survey All patients were contacted for a post-treatment telephone interview (Table 1). A questionnaire was used

TABLE 1. Telephone Survey Questionnaire 1. What impact did the condition have on your quality of life and did it stop you from doing anything? 2. How successful do you feel the treatment was? A. Very successful 7–10 B. Fairly successful 4–6 C. Not very successful 1–3 3. How much improvement did the treatment bring to your quality of life (if any) and how? 4. How would you rate the post-treatment scarring (mild/minimal, moderate, or severe)? 5. Did you experience any recurrence in any of the treated areas?

to explore the impact of the condition on patients’ quality of life and their perception of the treatment. Table 1 shows the telephone survey questionnaire.

Results Eight patients (4 males and 4 females; age: range, 20–41 years; mean, 28 years) with SM treated with the above method were indentified (Table 2). All patients were of Caucasian origin and had skin phototypes II-III. The chest, back, and axillae were the main sites of SM in most patients, and 1 patient had vulval SM as well. One patient had isolated facial SM, and another patient had coexisting eruptive vellus hair cysts. The diagnosis was established clinically and histologically in all patients. All patients underwent test treatments, where up to 5 lesions were extirpated under local anesthetic. After satisfactory review at 3 months, 5 patients continued to receive treatments under local anesthetic and 3 patients underwent treatment under general anesthetic. No postoperative complications were noted. Patients were followed up for an average of 48.5 months (range, 12–137 months). Good clinical improvement was noted in all treated areas with minimal scarring, and recurrence of SM at the treatment sites was uncommon. Examples of the improvement after treatment are shown in Figures 2 and 3. Telephone Survey Results

Figure 1. (A) Volkmann’s/Chalazion spoon (B) with close-up of the tip.

Six patients agreed to take part in the telephone interview (Table 3). All 6 responders felt that SM had significant impact on their quality of life. Cosmetic

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STEATOCYSTOMA MULTIPLEX TREATMENT WITH THE CO2 LASER

TABLE 2. Summary of Patient Characteristics

Patient Age Gender

Areas Treated

1

41

F

Abdomen, chest, back, neck, vulva

LA

16

None

137 months

2

35

M

Chest, back, axillae

LA

1

None

14 months

GA

1

LA

2

None

88 months

GA

1

LA LA

3 5

None None

12 months 16 months

3

27

F

Hip, axillae, neck, chest, back

4 5

27 26

M F

Chest Face

6

25

M

Back, chest

LA

3

None

72 months

7

25

F

Neck, axillae, chest, thighs

LA

4

None

37 months

8

20

M

Chest, back

LA

1

None

12 months

GA

1

appearance of the cysts limiting social activities and frequent infections causing pain and discomfort were listed as the main concerns. All patients felt that the treatment helped to significantly improve their quality of life and enabled them from overcoming the above-described difficulties. The treatment was very successful for 4 of the 6 patients, with 2 of them rating it at 10 of 10 and 2 patients considered it to be fairly successful. Five patients viewed the scarring as minimal, and 1 patient reported no scarring. Four patients had no recurrence of SM at the treated areas, and 2 reported minimal recurrence.

Figure 2. SM (A) before treatment showing multiple yellow cysts on the lower chest and abdomen and (B) 3 months after treatment showing clearance of the cysts with minimal scarring.

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Number of Treatment Type of Anesthetic Sessions Complications Follow-up

Discussion Steatocystoma multiplex is a rare condition that can have a significant negative impact on patients’ quality of life. Treatment is often difficult because the cysts are located deeply in the dermis and their effective destruction is critical to prevent recurrence while minimizing the risk of scarring. Furthermore, patients usually have multiple lesions, which makes some treatment modalities impractical. Isotretinoin has been used with variable results and may slow the progression of the disease. It has little effect on preexisting lesions, and recurrence is the norm on treatment cessation.5,6 Cryotherapy has been described, but it is painful, yields poor cosmetic result, and is impractical for multiple lesions.6 Surgical excision

Figure 3. Steatocystoma multiplex on the chest (A) before treatment and (B) 2 months after treatment showing clearance of the cysts with residual erythema that completely cleared by 4 months.

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No recurrence Minimal Very successful (8/10) 6

Self-conscious of the condition, avoided social interaction

A lot of improvement to quality of life, increased confidence

No recurrence No scarring Very successful (10/10) 5

Conscious of appearance, stopped patient from doing activities such as swimming

Great improvement to quality of life. Now enjoys swimming regularly

Minimal Minimal Fairly successful (6/10) 4

Painful lesions in groin area that affected walking

Yes, it helped improve quality of life

No recurrence Minimal Very successful (10/10) 3

Patient was conscious of the appearance, affected his confidence

Significantly improved quality of life

No recurrence Minimal Fairly successful (6/10) 2

Stopped patient from swimming, frequently painful inflamed lesions

Significant improvement to quality of life

Minimal Minimal A lot of improvement to quality of life, now able to wear clothes she was not able to before treatment Very successful (9/10) Felt very embarrassed by the appearance, unable to wear certain types of clothes 1

Treatment Efficacy Impact on Quality of Life Patient

TABLE 3. Summary of Telephone Survey Results

Improvement to Quality of Life

Scarring

Recurrence

BAKKOUR AND MADAN

risks scarring and is impractical for multiple lesions. A number of alternative surgical approaches have been reported in single case reports. Puncturing the cyst with sharp-tipped cautery point followed by squeezing the cyst content was reported in one case with good clearance but left some hypopigmentation.7 Another technique using incision with a surgical blade and hooking the cyst content with a phlebotomy hook followed by Steri-Strips to close the wound was tried in 5 patients. The results were reasonable, but only 2 of their patients decided to have further treatment.8 Surgical incision with a blade is invasive and likely to cause bleeding and increase the risk of complications. Radiofrequency incision followed by expressing the cyst content has also been reported in 2 cases. Antibiotics were prescribed for both patients for a week after surgery.9 Laser treatment of SM has also been described in a number of single case reports. Rossi and coworkers used the CO2 laser in a patient with predominantly facial lesions. They vaporized smaller cysts and opened larger ones with the CO2 laser followed by emptying the content with a forceps.4 The cysts sit deep in the dermis and vaporizing them with the CO2 laser is more invasive than our technique and can lead to scarring. The erbium:yttrium aluminium garnet laser has also been used in 1 patient, but the technique only involved puncturing the cyst with no mention of destroying its wall. The patient was only followed up for 3 months, and we would expect high recurrence rate with puncture only technique.10 A combination of 2 nonablative lasers were used in 1 patient with lesions on the trunk. Moody and colleagues hypothesized that the 1,450-nm diode laser would target the abnormal sebaceous glands and the 1,550-nm fractionated erbiumdoped fiber laser would target the dermal cysts.11 Two sessions were required for the same areas, and they noticed more than 75% improvement at 8 months.11 The perforation and extirpation method for the treatment of SM was first reported by the senior author (VM) in 2009.12 Since then, we have used this technique successfully in 8 patients. On exploring patients’ perspective, it is clear that the CO2 laser treatment is highly acceptable and brings

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STEATOCYSTOMA MULTIPLEX TREATMENT WITH THE CO2 LASER

a significant improvement to their quality of life. Other advantages of this technique include minimal scarring and low recurrence of SM at the treatment sites despite prolonged follow-up. The treatment is also practical allowing for a large number of lesions to be treated in one session, especially if performed under general anesthetic. Study limitations include a small sample size and its retrospective nature. For a rare condition such as SM, it is unlikely that a prospective, head-to-head comparison of treatment modalities can be achieved. To the best of our knowledge, this is the largest therapeutic review of any treatment modality of SM to date and the only one to formally explore patients’ views of the treatment. In summary, the minimally invasive nature of the treatment, the ability to treat multiple lesions in a single session, excellent cosmesis, and low recurrence coupled with encouraging patient feedback has made CO2 laser perforation and extirpation our treatment of choice for SM. Acknowledgment We acknowledge Dr. Paul J August who developed the technique and treated some of the patients in the study. References 1. Covello SP, Smith FJ, Sillevis Smitt JH, Paller AS, et al. Keratin 17 mutations cause either steatocystoma multiplex or pachyonychia congenita type 2. Br J Dermatol 1998;139:475–80.

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2. Torchia D, Vega J, Schachner LA. Eruptive vellus hair cysts: a systematic review. Am J Clin Dermatol 2012;13:19–28. 3. Cho S, Chang SE, Choi JH, Sung KJ, et al. Clinical and histologic features of 64 cases of steatocystoma multiplex. J Dermatol 2002;29:152–6. 4. Rossi R, Cappugi P, Battini M, Mavilia L, et al. CO2 laser therapy in a case of steatocystoma multiplex with prominent nodules on the face and neck. Int J Dermatol 2003;42:302–4. 5. Schwartz JL, Goldsmith LA. Steatocystoma multiplex suppurativum: treatment with isotretinoin. Cutis 1984;34:149–50, 153. 6. Apaydin R, Bilen N, Bayramgürler D, Bas¸das¸ F, et al. Steatocystoma multiplex suppurativum: oral isotretinoin treatment combined with cryotherapy. Australas J Dermatol 2000;41:98–100 7. Kaya TI, Ikizoglu G, Kokturk A, Tursen U. A simple surgical technique for the treatment of steatocystoma multiplex. Int J Dermatol 2001;40: 785–8 8. Lee SJ, Choe YS, Park BC, Lee WJ, et al. The vein hook successfully used for eradication of steatocystoma multiplex. Dermatol Surg 2007; 33:82–4. 9. Choudhary S, Koley S, Salodkar A. A modified surgical technique for steatocystoma multiplex. J Cutan Aesthet Surg 2010;3:25–8 10. Mumcuoglu CT, Gurel MS, Kiremitci U, Erdemir AV, et al. Er: yag laser therapy for steatocystoma multiplex. Indian J Dermatol 2010;55:300–1. 11. Moody MN, Landau JM, Goldberg LH, Friedman PM. 1,450-nm diode laser in combination with the 1550-nm fractionated erbium-doped fiber laser for the treatment of steatocystoma multiplex: a case report. Dermatol Surg 2012;38:1104–6 12. Madan V, August PJ. Perforation and extirpation of steatocystoma multiplex. Int J Dermatol 2009;48:329–30.

Address correspondence and reprint requests to: Waseem Bakkour, MSc, MRCP, MD, Dermatology Department, Salford Royal NHS Foundation Trust, Manchester, Stott lane, M6 8HD, or e-mail: [email protected]

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Carbon dioxide laser perforation and extirpation of steatocystoma multiplex.

Steatocystoma multiplex (SM) is a rare condition that presents as multiple dermal cysts, the appearance of which can have a significant impact on the ...
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