benign familial pemphigus (hailey-hailey disease)

4
CASE REPORT Benign Familial Pemphigus (Hailey-Hailey Disease) Treatment with the Pulsed Carbon Dioxide Laser DANY J. TOUMA, MD MADELINE KRAUSS, MD DAVID S. FEINGOLD, MD MICHAEL S. KAMINER, MD background . Benign familial pemphigus (BFP) is a chronic blistering disease with significant morbidity. Surgical methods are often needed to control flares in difficult cases. objective . To describe the response of BFP to vaporization with a pulsed carbon dioxide (C 02) laser. methods . A 38-year-old woman with chest and axillary in- volvement unresponsive to conventional therapy was treated with the UltraPulse 5000 Laser (Coherent Medical Group, Palo Alto, CA). After active sites of BFP showed good response to treatment, we treated uninvolved skin of the left axilla to assess the efficacy of prophylactic therapy. results . Treatment of affected areas, except biopsy sites, re- sulted in clearing of active lesions after 1-2 weeks. We noted striking sparing of the treated areas from developing subsequent disease. The region that was later treated prophylactically has shown minor, asymptomatic recurrence of BFP in less than 5% of the area treated over an 18-month follow-up period. conclusion . The pulsed carbon dioxide laser is a useful mo- dality in treatment of BFP. In our patient, prophylactic treat- ment led to near complete eradication of disease in the treated area. A controlled, larger study is needed to confirm our results, and to determine optimal laser parameters. Long-term effects and duration of remission remain to be determined. © 1998 by the American Society for Dermatologic Surgery, Inc. Dermatol Surg 1998;24:1411-1414. B enign familial pemphigus (BFP) (Hailey-Hailey disease) is a rare, recurrent blistering disease, transmitted as an autosomal dominant trait. The disease presents in the second or third decade of life, and is usually triggered by friction and/or excessive sweating. Flares are associated with significant morbid- ity, and can be partially responsive to topical cortico- steroids. As superinfection with staphylococcus species and Candida is common, treatment often includes oral and topical antibiotics and/or topical antifungal thera- py.1 Unfortunately, many patients have severe unre- sponsive disease. Excision of involved areas followed by grafting has been performed successfully, but with considerable as- sociated morbidity, including infection, bleeding, thromboembolic disease, contractures and poor cos- metic results.2,3 Treatment with the continuous wave From the Departments of Dermatology at Harvard Medical School and Beth Israel Deaconess Medical Center (MK, MSK); Boston University School of Medicine (DJT); and Tufts University School of Medicine (DSF), Boston, Massachusetts. Address correspondence and reprint requests to: Michael Kaminer, MD, Beth Israel Deaconess Medical Center, Department of Dermatology, 330 Brookline Avenue, Boston, MA 02215. carbon dioxide (C02) laser has been associated with lasting remissions of disease.4-6 Fibrosis resulting from ablation of the epidermis and papillary dermis was thought to be an important element for improvement. Unfortunately, unacceptable scarring can follow poorly controlled deep ablation. Dermabrasion has been asso- ciated with similar results, but with less frequent scar- ring.7,8 We describe the use of the UltraPulse 5000 C 02 laser (Coherent Medical Group, Palo Alto, CA) as a safe, adjunctive treatment in the management of BFP. Case Report In July 1996, a 38-year-old white female with chest and axillary involvement of BFP who was unresponsive to conventional therapy was treated with the UltraPulse C02 laser. A pulse energy of 500 mj was used at 2-3 W with the 3-mm collimated Truespot hand piece. (Coher- ent Medical Group, Palo Alto, CA) Test sites were treated with one to three passes on the chest, and one to four passes in the axilla. Biopsies were obtained from untreated and treated skin, and the patient was exam- ined at weekly intervals to assess results. © 1998 by the American Society for Dermatologic Surgery, Inc. Published by Elsevier Science Inc. 1076-0512/98/$ 19.00 • PII S1076-0512(98)00164-2 1411

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Page 1: Benign Familial Pemphigus (Hailey-Hailey Disease)

CASE REPORT

Benign Familial Pemphigus (Hailey-Hailey Disease)Treatm ent w ith the Pulsed Carbon D ioxide Laser

DANY J. TOUMA, M D M ADELINE KRAUSS, M D D A VID S. FEINGOLD, M D M ICHAEL S. KAMINER, M D

b a c k g r o u n d . Benign familial pemphigus (BFP) is a chronic blistering disease with significant morbidity. Surgical methods are often needed to control flares in difficult cases. o b j e c t iv e . To describe the response of BFP to vaporization with a pulsed carbon dioxide (C 02) laser.m e t h o d s . A 38-year-old woman with chest and axillary in­volvement unresponsive to conventional therapy was treated with the UltraPulse 5000 Laser (Coherent Medical Group, Palo Alto, CA). After active sites of BFP showed good response to treatment, we treated uninvolved skin of the left axilla to assess the efficacy of prophylactic therapy.r e s u l t s . Treatment of affected areas, except biopsy sites, re­sulted in clearing of active lesions after 1-2 weeks. We noted

striking sparing of the treated areas from developing subsequent disease. The region that was later treated prophylactically has shown minor, asymptomatic recurrence of BFP in less than 5% of the area treated over an 18-month follow-up period. c o n c l u s io n . The pulsed carbon dioxide laser is a useful mo­dality in treatment of BFP. In our patient, prophylactic treat­ment led to near complete eradication of disease in the treated area. A controlled, larger study is needed to confirm our results, and to determine optimal laser parameters. Long-term effects and duration of remission remain to be determined. © 1998 by the American Society for Dermatologic Surgery, Inc. Dermatol Surg 1998;24:1411-1414.

Benign familial pemphigus (BFP) (Hailey-Hailey disease) is a rare, recurrent blistering disease, transmitted as an autosomal dominant trait. The

disease presents in the second or third decade of life, and is usually triggered by friction and/or excessive sweating. Flares are associated with significant morbid­ity, and can be partially responsive to topical cortico­steroids. As superinfection with staphylococcus species and Candida is common, treatment often includes oral and topical antibiotics and/or topical antifungal thera­py.1 Unfortunately, many patients have severe unre­sponsive disease.

Excision of involved areas followed by grafting has been performed successfully, but with considerable as­sociated morbidity, including infection, bleeding, thromboembolic disease, contractures and poor cos­metic results.2,3 Treatment with the continuous wave

From the Departments of Dermatology at Harvard Medical School and Beth Israel Deaconess Medical Center (MK, MSK); Boston University School of Medicine (DJT); and Tufts University School of Medicine (DSF), Boston, Massachusetts.

Address correspondence and reprint requests to: Michael Kaminer, MD, Beth Israel Deaconess Medical Center, Department of Dermatology, 330 Brookline Avenue, Boston, MA 02215.

carbon dioxide (C02) laser has been associated with lasting remissions of disease.4-6 Fibrosis resulting from ablation of the epidermis and papillary dermis was thought to be an important element for improvement. Unfortunately, unacceptable scarring can follow poorly controlled deep ablation. Dermabrasion has been asso­ciated with similar results, but with less frequent scar­ring.7,8

We describe the use of the UltraPulse 5000 C 02 laser (Coherent Medical Group, Palo Alto, CA) as a safe, adjunctive treatment in the management of BFP.

Case Report

In July 1996, a 38-year-old white female with chest and axillary involvement of BFP who was unresponsive to conventional therapy was treated with the UltraPulse C 02 laser. A pulse energy of 500 mj was used at 2-3 W with the 3-mm collimated Truespot hand piece. (Coher­ent Medical Group, Palo Alto, CA) Test sites were treated with one to three passes on the chest, and one to four passes in the axilla. Biopsies were obtained from untreated and treated skin, and the patient was exam­ined at weekly intervals to assess results.

© 1998 by the American Society for Dermatologic Surgery, Inc. • Published by Elsevier Science Inc.1076-0512/98/$ 19.00 • PII S1076-0512(98)00164-2

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O R IG IN A L A R T IC L E S

Dermatol Surg1998;24;14ll-1414

A ll treated areas (except biopsy sites) cleared o f any blistering, crusting, or significant erythema after 1-2 weeks. Six weeks later, the patient had a flare o f disease invo lv ing most o f her le ft ax illa ry skin. However, treated areas were s trik ing ly spared (Figure 2). M inor scarring was noted on the chest in areas treated w ith three passes w ith the C 0 2 laser.

Encouraged by these results, we investigated whether resurfacing has a role in preventing disease. U ninvolved skin o f the entire le ft axilla was treated w ith one pass at 300 m j and a second pass at 200 m j using a 2.25-mm scanner device at a density setting o f 5 (Figure 3A). Local anesthesia was achieved w ith the tumescent technique. W ith in 3 weeks, w h ile in the heal­ing phase, the patient developed active disease affect­ing 5%-10% of the area treated (Figure 3B). U nlike her usual disease, these lesions were largely asymptomatic.

Over the last 18 months, a ll o f the treated areas have shown resistance to involvem ent w ith Hailey-H ailey disease. There is rare asymptomatic activ ity in less than 5% o f the treated axilla. She has continued to get re­peated flares, however, in the contralateral axilla. Scar­ring has been absent, except in areas treated w ith more than tw o laser passes on the chest.

D i s c u s s i o n

O ur patient has clearly benefitted from laser resurfacing o f an area prone to involvem ent w ith BFP. In the small area where sparing was not complete, skin involvem ent w ith BFP was m inor and almost asymptomatic when compared w ith untreated surrounding skin.

Epiderm al acantholysis in BFP is caused by abnor­m al keratinocyte cohesion, w hich is the result o f a breakdown in the desmosome-keratin filam ent com­plexes. This defect appears to invo lve in te rfo llicu la r epiderm al cells, whereas the adnexal keratinocytes are usually spared.7 In fact, histopathology o f affected skin typ ica lly shows the acantholysis sparing the follicles.

Benefits o f treatment o f BFP w ith vaporization are like ly derived from ablation o f the affected keratino­cytes and re-epithelia lization from the uninvolved ad- nexae. The advantages o f th is destructive m odality over those previously reported are the reproducible preci­sion o f epiderm al ablation and the avoidance o f blood splattering seen w ith dermabrasion. The precision of the laser is clearly o f value in standardizing treatment between physicians, and thus may decrease the risk of scarring seen w ith other treatments. In addition, heal­ing from C 0 2 laser treatment in our patient was com-

H istopathology o f treated areas (Figure 1) revealed plete in 2-3 weeks,progressive ablation o f tissue w ith each pass sim ilar to O ur p re lim inary results indicate that the pulsed C 0 2results seen w ith other studies.9 We note that complete laser may be a safe alternative method fo r the treatmentvaporization o f the epiderm is was achieved in areas o f BFP. Complete epiderm al ablation appears necessarytreated w ith tw o or more passes. fo r a favorable outcome, and is achieved w ith tw o

Figure 1. A) Histopathology of intact lesionai skin, B) after one pass at 500 m j with 3-mm hand piece, C) after two passes, and, D) after three passes. Note incomplete ablation of epidermis after one pass. H & E, X 200.

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T O U M A E T A L . 1413

T R E A T M E N T O F B F P W IT H C 0 2 L A S E R

Figure 2. A) Lesionai skin of left axilla before treatment with 1 to 4 passes. B) BFP flare o f the left axilla 6 weeks after treatment. Note sparing of the treated site (arrow). A biopsy scar is present inferiorly.

Figure 3. A ) Left axilla immediately after prophylactic treatment with two passes. B) Three weeks after resurfacing, a small area o f active BFP (boxed) is present in the center o f the axilla. Residual erythema faded over the next several weeks.

passes at 300 m j (w ith scanner) o r 500 m j (w ith the Truespot hand piece). A dd itiona l passes can lead to scarring (as seen in our patient). Prophylactic treatment in areas o f predilection for the disease, such as the axillae, may have a d istinct role in preventing or alle­v ia ting fu ture flares o f the disease.

In th is case report we treated on ly one patient, and our fo llow up was re lative ly short (18 months). A l­though long-term effects and duration o f remission re­main to be determ ined, our patient clearly benefitted

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Dermatol Surg1998;24;1411-1414

from laser resurfacing of BFP. W ith the in troduction of the Erbium :YAG laser, we now have more options fo r w ell-contro lled tissue ablation. Further studies are needed to confirm our results and to determ ine optim al laser parameters.

References

1. Burge S. Hailey-Hailey disease: the clinical features, response to treatment and prognosis. Br J Dermatol 1992;126:275-82.

2. Berger RS, Lynch PJ. Familial benign chronic pemphigus: surgical treatment and pathogenesis. Arch Dermatol 1971;104:380-4.

3. Crotty CP, Scheen SR III, Masson JK, W inkelmann RK. Surgical treatment of familial benign chronic pem phigus. Arch Dermatol 1981;117:540-2.

4. Don PC, Carney PS, Lynch WS, Zaim MT, Hassan MO. Carbon dioxide laserabrasion: a new approach to management of familial benign chronic pem phigus (Hailey-Hailey disease). J Dermatol Surg Oncol 1987;13:1187-94.

5. McElroy JA, Mehregan DA, Roenigk RK. Carbon dioxide vapor­ization of recalcitrant symptomatic plaques of H ailey-Hailey dis­ease. J Am Acad Dermatol 1990;23:893-7.

6. Kartamaa M, Reitamo S. Familial benign chronic pem phigus. Treatment w ith C 0 2 laser vaporization. Arch Dermatol 1992;128: 646-8 .

7. Hamm H, M etze D, Brocker EB. Hailey-Hailey disease. Eradication by dermabrasion. Arch Dermatol 1994;130:1143-9.

8. Kirtschig G, Gieler U, Happle R. Treatment of H ailey-Hailey dis­ease by dermabrasion. J Am Acad Dermatol 1993;28:784-6.

9. Kauvar AB, Waldorf HA, Geronemus RG. A histopathological comparison of "char-free" carbon dioxide lasers. Dermatol Surg 1996;22:343-8.