2386 East Heritage Way, Suite B, Salt Lake City, Utah 84109 USA Phone +1-877-628-7300 • Email—Info@pachyonychia.org

www.pachyonychia.org

15 March 2005

Use of Articles in the Pachyonychia Congenita Bibliography

The articles in the PC Bibliography may be restricted by copyright laws. These have been made available to you by PC Project for the exclusive use in teaching, scholar-ship or research regarding Pachyonychia Congenita. To the best of our understanding, in supplying this material to you we have followed the guidelines of Sec 107 regarding fair use of copyright materials. That section reads as follows:

Sec. 107. - Limitations on exclusive rights: Fair use Notwithstanding the provisions of sections 106 and 106A, the fair use of a copyrighted work, including such use by reproduction in copies or phonorecords or by any other means specified by that section, for purposes such as criticism, comment, news reporting, teaching (including multiple copies for classroom use), scholarship, or research, is not an infringement of copyright. In determining whether the use made of a work in any particular case is a fair use the factors to be considered shall include - (1) the purpose and character of the use, including whether such use is of a commercial nature or is for nonprofit educational purposes; (2) the nature of the copyrighted work; (3) the amount and substantiality of the portion used in relation to the copyrighted work as a whole; and (4) the effect of the use upon the potential market for or value of the copyrighted work. The fact that a work is unpublished shall not itself bar a finding of fair use if such finding is made upon consideration of all the above factors.

We hope that making available the relevant information on Pachyonychia Congenita will be a means of furthering research to find effective therapies and a cure for PC.

The Vein Hook Successfully Used for Eradication ofSteatocystoma Multiplex

SEOK-JONG LEE, MD, YOON SEOK CHOE, MD, BYUNG CHUL PARK, MD, WOEN JU LEE, MD, AND

DO WON KIM, MD�

BACKGROUND Steatocystoma multiplex is characterized by the formation of the numerous cutaneouscysts in the exposed area leaving some cosmetic problems for the patients. Only surgical excisionhas been effective, and its several variations were done with limited success. Because thepatients usually have many cysts, excision of cysts was tedious for the doctors and left scars onthe patients.

METHOD Five patients agreeing to participate in this experiments were selected. The vein hookused for ambulatory phlebectomy was employed to eradicate the cysts. The skin was incised appro-ximately 2 to 3 mm in length. Then the mosquito forceps removed the cysts by gently squeezingor hooking the inner or outer cyst wall. By completely removing tissue around the cyst, recurrence wasable to be prevented.

RESULT It took approximately 1 minute to excise one cyst completely, it left no hypertrophic scarsexcept for transient postinflammatory hyperpigmentation, and it had no recurrences for 14 to 30 monthson five patients.

CONCLUSION The use of this instrument is very simple and time-saving, providing excellent successrate with favorable cosmetic results. It can be a good alternative for eradication of the cysts in steat-ocystma multiplex.

The authors have indicated no significant interest with commercial supporters.

Steatocystoma multiplex is a disorder clinically

characterized by numerous cutaneous cysts

with nonkeratinizing walls. The walls are lined

by thin epidermis and sebaceous glands within or

adjacent to the cyst walls histopathologically.

There is no known medical treatment for this

disease, and surgical excision is the only effective

treatment. Patients usually have so many cysts,

however, that it takes a long time for surgeons

to excise them all, which can leave hypertrophic

scars in spite of high success rate.

Numerous surgical procedures have been tried to

overcome these limitations; for example, 18-gauge

needle aspiration,1 3-mm punch excision,2 laser

therapy,3 cryotherapy,4 incision, and artery forceps

extraction5 have not been satisfactory considering

time consumed, scar formation, and more import-

antly, success rate.

We employed a vein hook used for ambulatory

phlebectomy for patients with varicose vein of the

lower extremities. Its tip is properly bent twice,

so after being inserted through the narrow incision

and twisted, it can hook the outer or inner

surface of the cyst or its capsule (Figure 1). When

the cyst is pulled, the hook easily separates the cyst

from the surrounding soft tissue. The procedure

with the vein hook was anticipated to decrease the

size of the incision, reducing the time needed for

removing cysts from surrounding soft tissues and

for the excision of the cysts. This also diminishes

the possibility of scars for good cosmetic results

to the patients.

& 2007 by the American Society for Dermatologic Surgery, Inc. � Published by Blackwell Publishing �ISSN: 1076-0512 � Dermatol Surg 2007;33:82–84 � DOI: 10.1111/j.1524-4725.2007.33013.x

8 2

�All authors are affiliated with the Department of Dermatology, Kyung Pook National University Hospital, Daegu,Korea

The practical technique is to mark each cyst with pen

and to inject 1:100,000 epinephrine-premixed lido-

caine under each cyst. By injecting under the cysts,

the cysts then are pushed up just under the epidermis

for easy separation from the surrounding tissue in

addition to patients’ comfort. Next, the skin is in-

cised about 2 to 3 mm using a No. 11 blade at a right

angle (Figure 2A), and the cysts are removed by vein

hook without puncture of the walls if possible (Fig-

ure 2B). In contrast, if the wall is punctured, the cyst

should be squeezed to cause the contents to come out

first. The hook then inserts to grasp either the inner

or the outer wall for the cyst and pulls it out gently.

When the cyst is exposed, the baby mosquito forceps

grasp the portion of the cyst and pull it out gently

(Figure 2C). Other grasps are frequently needed to

remove the complete lining of the cysts adhering to

the surrounding tissues (Figure 2D). The wounds are

cleaned with gauze soaked in alcohol, and then the

margins are approximated by steri-strips.

Five typical cases of steatocystoma multiplex are re-

ported, which were treated by the above-mentioned

simple surgical technique (Table 1). On these five

cases, our method proved successful. They had a mild

transient hyperpigmentation for about 2 to 4 weeks,

and all resulted in satisfactory cosmetic work. Two

were eager to remove the remainder of the cysts

(Figures 3A and 3B).

We expect ‘‘simple incision or puncture for hook-

ing out the cyst by phlebectomy hook’’ to be very

successful in steatocystoma multiplex patients. It is

possible to work by making small incisions,

Figure 1. The vein hook used for the removal of the cystsand tip of hook bent twice (inset).

Figure 2. The sequence: incision by No. 11 blade (A); inser-tion of vein hook through the incision (B); pulling thehooked cyst out (C); and complete removal of cyst and sur-rounding soft tissue with help of mosquito forceps (D).

3 3 : 1 : J A N U A RY 2 0 0 7 8 3

L E E E T A L

approximately 2 to 3 mm, to minimize the risk of

tissue injury, infection, the resultant hyperpigmen-

tation, scarring, and operation time. Accordingly,

this method might be the first line of treatment of the

steatocystoma multiplex instead of conventional

excisional surgery.

References

1. Sato K, Shibuya K, Taguchi H, et al. Aspiration therapy in stea-

tocystoma multiplex. Arch Dermatol 1993;129:35–7.

2. Takuro K, Kyoko K, Masayuki N, Tamotsu K. A case of steato-

cystoma multiplex with prominent cysts on the scalp treated suc-

cessfully using a simple surgical technique. J Dermatol

1995;22:438–40.

3. Krahenbuhl A, Eichmann A, Pfaltz M. CO2 laser therapy for

steatocystoma multiplex. Dermatologica 1991;183:294–6.

4. Notowicz A. Treatment of lesion of steatocystoma multiplex and

other epidermal cysts by cryosurgery. J Dermatol Surg Oncol

1980;6:98–9.

5. Keefe M, Leppard BJ, Royle G. Successful treatment of steatocysto-

ma multiplex by simple surgery. Br J Dermatol 1992;127:41–4.

Address correspondence and reprint requests to: Seok-jongLee, MD, Department of Dermatology, Kynugpook NationalUniversity Hospital, 50, Samduck-2-ga, Chung-gu, Daegu,Korea (South) 700-721, or e-mail: oblongata73@hanmail.net.

TABLE 1. Vein Hook Operation 5 Cases

No. Sex

Age

(years) Location

Duration

(years)

Size

(mm) Number

Follow-up

(months)

Side

effect Recurrence

1 Male 53 Neck 30 5–10 12 22 (–) (–)

2 Male 28 Arm 12 3–5 14 15 (–) (–)

3 Male 24 Forearm 10 4–6 11 30 (–) (–)

4 Female 31 Neck 6 5–7 8 19 (–) (–)

5 Male 46 Face 20 7–10 5 14 (–) (–)

Figure 3. Preoperative feature (A) and 4 weeks post-operative feature (B) in Case 1.

D E R M AT O L O G I C S U R G E RY8 4

V E I N H O O K F O R T H E E R A D I C AT I O N O F S T E AT O C Y S T O M A M U LT I P L E X