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Familial nevus sebaceus of Jadassohn: Occurrence in three generations
Volume 22 Number 5, Part I May 1990
extent of involvement also decreased progressively during treatment. Complete remission or marked improvement was obtained in six of eight patients who completed the study. No significant changes in laboratory values were detected. During the second treatment phase (weeks 9 to 16) the dosage of 30 tiig] day was maintained in four patients and decreased to 20 or 10 mg/day in the other four. Comment The results obtained in this open study demonstrate that acitretin is effective in the treatment of severe lichen planus. The rapid improvement suggests that acitretin may be slightly more effective than etretinate or isotretinoin. An initial dosage of acitretin, 30 mg/ day, gave good results, similar to those observed after 1 mg/kg/day (50 to 100 tag] day) of either etretinate8. 1! or isotretinoin.P The side effects were those commonly seen with etretinate.P The main advantage of acitretin over etretinate is its more rapid elimination. Thus the teratogenic risk is essentially limited to the treatment period.
Brief communications 853 Familial nevus sebaceus of J adassolm: Occurrence in three generations William J. SaW, Jr., MD Oklahoma City, Oklahoma Until recently the appearance of nevus sebaceus was thought to be sporadic and not inherited. 1 This is the second reported case of inherited nevus sebaceus and the first to occur in three generations. Case report. A 65-year-old black man had a lifelong history of a 4 X6 em bald area on the crown of the scalp. Within the bald spot a 2.5 X 3.5 em tumor had grown From the Department of Dermatology, University of Oklahoma Health Sciences Center. Reprint requests: William J. Sahl, Jr., MD, Chief, Dermatologic Surgery, Department of Dermatology, University of Oklahoma Health Sciences Center, Oklahoma City, OK 73104.
16/4/16080
REFERENCES 1. Haenni R. Pharmacokinetic and metabolic pathways of
2.
3.
4.
5.
6.
7.
8. 9.
10.
11.
12.
systematically applied retinoids. Dermatologica 1978; 157(suppl 1):5-10. Paravicini V, Stoeckel K, McNamara PJ, et al. On metabolism and pharmacokinetics of an aromatic retinoid. Ann NY Acad Sci 1981;359:54-67. Paravicini V, Camenzind M, Gower M, et al. Multiple dose pharmacokinetics of Ro 10-1670, the main metabolite of etretinate (Tigason). In: Saurat H-H, ed. Retinoids: new trends in research and therapy. Basel, Switzerland; Karger, 1985:289-92. Brindley C. An overview of recent clinical pharmacokinetic studies with acitretin (Ro 10-1670, etretin). Dermatologica 1989;178:79-87. Geiger J-M, Ott F, Bollag W. Clinical evaluation of an aromatic retinoid, Ro 10-1670, in severe psoriasis. Curr Ther Res 1984;35:735-40. Geiger J-M, Czarnetzki B-M. Acitretin (Ro 10-1670, etretin): overall evaluation of clinical studies. Dermatologica 1988;176:182-90. Goldfarb M, Ellis C, Gupta A, et al. Acitretin improves psoriasis in a dose-dependent fashion. JAM ACAD DERMATOL 1988;18:655-62. Schuppli R. The efficacy of a new retinoid (Ro 10-9359) in lichen planus. Dermatologica 1978;157(suppll):60-3. Ebner H. Erfahrungen mit der systemischen Retinoidbehandlung (Ro 10-9359) bei Lichen rubber planus. Wien Klin Wochenschr 1979;91:161-4. Viglioglia PA. Therapeutic evaluation of the oral retinoid Ro 10-9359 in several non-psoriatic dermatoses. Br J Dermatol 1980;103:483-7. Cordero AA, Allevato MAJ, Barclay CA, et al. Treatment of lichen planus and leukoplakia with the oral retinoid Ro 10-9359. In: Orfanos CE, Braun-Falco 0, Farber EM, et al., eds. Retinoids: advances in basic research and therapy. Berlin: Springer, 1981:273-8. Ellis CN, Voorhees JJ. Etretinate therapy. J AM ACAD DERMATOL 1987;16:267-91.
2 Fig. 1. Photomicrograph of basal cell carcinoma arising within index patient's nevus sebaceus. (Hematoxylineosin stain; X32.) Fig. 2. Photomicrograph of index patient's nevus sebaceus after excision of basal cell carcinoma. (Hematoxylin-eosin stain; X28.)
Journal of the American Academy of Dermatology
854 Brief communications
Valium in 1982. 2 The mother also had a syringocystadenoma papilliferum within the nevus. As in my patients, transmission was most compatible with an autosomal dominant inheritance pattern. Two interesting features of familial nevus sebaceus are exclusive confinement to the scalp and an additional type of tumor in each index case.
REFERENCES I. MehreganAH, PinkusH. Life historyoforganoid nevi. Areh Derrnatol 1965;91 :574-88. 2. Monk BE, Vollum OJ. Familial naevus sebaceous. J R Soc Med 1982;75:660-1.
Fig. 3. Tan-colored verrucous plaque on scalp of index patient's daughter.
The use of dolls as a teaching aid for children undergoing treatment with the flashlamp-pulsed tunable dye laser Karen F. Rothman, MD, Alberta Nutile, RN, and Carolyn Appel, LPN Worcester, Massachusetts
Fig. 4. Linear verrucous plaque on scalp of index patient's granddaughter. during the preceding month. A biopsy specimen disclosed a basal cell carcinoma (Fig. 1) arising in a nevus sebaceus (Fig. 2). Mohs surgery was successfully performed. Thereafter, complete excision of the underlying nevus was accomplished. Further history uncovered two other family members with similar bald spots. Examination of the daughter's scalp showed a nevus sebaceus (Fig. 3) on the left frontal aspect of the scalp and a nevus sebaceus on the right partial aspect of the scalp of his granddaughter (Fig. 4). Neither ofthese lesions was histologically examined or excised. The cutaneous lesions in this family were not associated with neurologic abnormalities. Discussion. Familial nevus sebaceus of Jadassohn in a mother and daughter was first described by Monk and
The tunable dye laser is becoming the treatment of choice for vascular lesionsof the skin 1,2 and is particularly useful in children. Unlike the carbon dioxide and argon lasers, the tunable dye laser can be used in children with a low risk of scarring. Pain felt by the patient is minimal and little or no postoperative wound care is required. Despite the minimal to moderate pain associated with treatment, children may find the treatment frightening. The psychological trauma of the procedure may be lessened by demonstrating the laser on a familiar object. At the beginning of the initial laser visit, we give each child a Raggedy Ann or Andy doll (Playskool). The child, nurse, or physician draws the "vascular anomaly" on the doll with a red crayon (Fig. 1). Everyone, including the doll, then puts on their laser goggles. The energy is set at 7 to 10 joules/ern', depending on how darkly the crayon is drawn on. On the child's count of 3, the foot pedal is depressed and clear circles are produced (Fig. 2). If the child wishes, he or she can hold the "wand" as the physician depresses the foot pedal. When the laser is activated, a small puff of smoke appears at the treated spot, From the Division of Dermatology, University of Massachusetts Medical Center. Presented in part at a meeting of the American Society for Plastic and Reconstructive Surgeons, San Francisco, Calif., Oct. 30, 1989. Reprint requests: Karen F. Rothman, MD, Division of Dermatology, University of Massachusetts Medical Center, 55 Lake Ave. North, Worcester, MA 01655.
16/4/16421
extent of involvement also decreased progressively during treatment. Complete remission or marked improvement was obtained in six of eight patients who completed the study. No significant changes in laboratory values were detected. During the second treatment phase (weeks 9 to 16) the dosage of 30 tiig] day was maintained in four patients and decreased to 20 or 10 mg/day in the other four. Comment The results obtained in this open study demonstrate that acitretin is effective in the treatment of severe lichen planus. The rapid improvement suggests that acitretin may be slightly more effective than etretinate or isotretinoin. An initial dosage of acitretin, 30 mg/ day, gave good results, similar to those observed after 1 mg/kg/day (50 to 100 tag] day) of either etretinate8. 1! or isotretinoin.P The side effects were those commonly seen with etretinate.P The main advantage of acitretin over etretinate is its more rapid elimination. Thus the teratogenic risk is essentially limited to the treatment period.
Brief communications 853 Familial nevus sebaceus of J adassolm: Occurrence in three generations William J. SaW, Jr., MD Oklahoma City, Oklahoma Until recently the appearance of nevus sebaceus was thought to be sporadic and not inherited. 1 This is the second reported case of inherited nevus sebaceus and the first to occur in three generations. Case report. A 65-year-old black man had a lifelong history of a 4 X6 em bald area on the crown of the scalp. Within the bald spot a 2.5 X 3.5 em tumor had grown From the Department of Dermatology, University of Oklahoma Health Sciences Center. Reprint requests: William J. Sahl, Jr., MD, Chief, Dermatologic Surgery, Department of Dermatology, University of Oklahoma Health Sciences Center, Oklahoma City, OK 73104.
16/4/16080
REFERENCES 1. Haenni R. Pharmacokinetic and metabolic pathways of
2.
3.
4.
5.
6.
7.
8. 9.
10.
11.
12.
systematically applied retinoids. Dermatologica 1978; 157(suppl 1):5-10. Paravicini V, Stoeckel K, McNamara PJ, et al. On metabolism and pharmacokinetics of an aromatic retinoid. Ann NY Acad Sci 1981;359:54-67. Paravicini V, Camenzind M, Gower M, et al. Multiple dose pharmacokinetics of Ro 10-1670, the main metabolite of etretinate (Tigason). In: Saurat H-H, ed. Retinoids: new trends in research and therapy. Basel, Switzerland; Karger, 1985:289-92. Brindley C. An overview of recent clinical pharmacokinetic studies with acitretin (Ro 10-1670, etretin). Dermatologica 1989;178:79-87. Geiger J-M, Ott F, Bollag W. Clinical evaluation of an aromatic retinoid, Ro 10-1670, in severe psoriasis. Curr Ther Res 1984;35:735-40. Geiger J-M, Czarnetzki B-M. Acitretin (Ro 10-1670, etretin): overall evaluation of clinical studies. Dermatologica 1988;176:182-90. Goldfarb M, Ellis C, Gupta A, et al. Acitretin improves psoriasis in a dose-dependent fashion. JAM ACAD DERMATOL 1988;18:655-62. Schuppli R. The efficacy of a new retinoid (Ro 10-9359) in lichen planus. Dermatologica 1978;157(suppll):60-3. Ebner H. Erfahrungen mit der systemischen Retinoidbehandlung (Ro 10-9359) bei Lichen rubber planus. Wien Klin Wochenschr 1979;91:161-4. Viglioglia PA. Therapeutic evaluation of the oral retinoid Ro 10-9359 in several non-psoriatic dermatoses. Br J Dermatol 1980;103:483-7. Cordero AA, Allevato MAJ, Barclay CA, et al. Treatment of lichen planus and leukoplakia with the oral retinoid Ro 10-9359. In: Orfanos CE, Braun-Falco 0, Farber EM, et al., eds. Retinoids: advances in basic research and therapy. Berlin: Springer, 1981:273-8. Ellis CN, Voorhees JJ. Etretinate therapy. J AM ACAD DERMATOL 1987;16:267-91.
2 Fig. 1. Photomicrograph of basal cell carcinoma arising within index patient's nevus sebaceus. (Hematoxylineosin stain; X32.) Fig. 2. Photomicrograph of index patient's nevus sebaceus after excision of basal cell carcinoma. (Hematoxylin-eosin stain; X28.)
Journal of the American Academy of Dermatology
854 Brief communications
Valium in 1982. 2 The mother also had a syringocystadenoma papilliferum within the nevus. As in my patients, transmission was most compatible with an autosomal dominant inheritance pattern. Two interesting features of familial nevus sebaceus are exclusive confinement to the scalp and an additional type of tumor in each index case.
REFERENCES I. MehreganAH, PinkusH. Life historyoforganoid nevi. Areh Derrnatol 1965;91 :574-88. 2. Monk BE, Vollum OJ. Familial naevus sebaceous. J R Soc Med 1982;75:660-1.
Fig. 3. Tan-colored verrucous plaque on scalp of index patient's daughter.
The use of dolls as a teaching aid for children undergoing treatment with the flashlamp-pulsed tunable dye laser Karen F. Rothman, MD, Alberta Nutile, RN, and Carolyn Appel, LPN Worcester, Massachusetts
Fig. 4. Linear verrucous plaque on scalp of index patient's granddaughter. during the preceding month. A biopsy specimen disclosed a basal cell carcinoma (Fig. 1) arising in a nevus sebaceus (Fig. 2). Mohs surgery was successfully performed. Thereafter, complete excision of the underlying nevus was accomplished. Further history uncovered two other family members with similar bald spots. Examination of the daughter's scalp showed a nevus sebaceus (Fig. 3) on the left frontal aspect of the scalp and a nevus sebaceus on the right partial aspect of the scalp of his granddaughter (Fig. 4). Neither ofthese lesions was histologically examined or excised. The cutaneous lesions in this family were not associated with neurologic abnormalities. Discussion. Familial nevus sebaceus of Jadassohn in a mother and daughter was first described by Monk and
The tunable dye laser is becoming the treatment of choice for vascular lesionsof the skin 1,2 and is particularly useful in children. Unlike the carbon dioxide and argon lasers, the tunable dye laser can be used in children with a low risk of scarring. Pain felt by the patient is minimal and little or no postoperative wound care is required. Despite the minimal to moderate pain associated with treatment, children may find the treatment frightening. The psychological trauma of the procedure may be lessened by demonstrating the laser on a familiar object. At the beginning of the initial laser visit, we give each child a Raggedy Ann or Andy doll (Playskool). The child, nurse, or physician draws the "vascular anomaly" on the doll with a red crayon (Fig. 1). Everyone, including the doll, then puts on their laser goggles. The energy is set at 7 to 10 joules/ern', depending on how darkly the crayon is drawn on. On the child's count of 3, the foot pedal is depressed and clear circles are produced (Fig. 2). If the child wishes, he or she can hold the "wand" as the physician depresses the foot pedal. When the laser is activated, a small puff of smoke appears at the treated spot, From the Division of Dermatology, University of Massachusetts Medical Center. Presented in part at a meeting of the American Society for Plastic and Reconstructive Surgeons, San Francisco, Calif., Oct. 30, 1989. Reprint requests: Karen F. Rothman, MD, Division of Dermatology, University of Massachusetts Medical Center, 55 Lake Ave. North, Worcester, MA 01655.
16/4/16421