- Email: [email protected]
Melanomas arising in married couples
400
Correspondence
called pseudotumors, that are ordinarily located over the elbows and knees. In addition, patients with EhlersDanlos syndrome may have small, hard, calcified lumps under the skin that are probably related to injury and subsequent repair. These processes are in no way dangerous and are not related to cancer. Bruising. Patients with Ehlers-Danlos syndrome usually bruise easily. Sometimes the resulting bruise is extensive, but clotting of blood is ordinarily normal. Since the bruising results from a problem in the structure of the underlying connective tissue, it is difficult for blood vessels to close themselves after they have been damaged. Moreover, they are more easily damaged because the connective tissue holding them together is abnormal. In a sense the processs resembles poking a pin-sized hole in a rubber sheet. Before long the hole becomes much larger than pin-sized. In a simple sense, this is what happens to the blood vessels when they are injured, Varicose veins. Since connective tissue in the entire body is involved, as well as the skin, the condition may lead to other manifestations. Varicose veins, particularly on the lower extremities, are common in patients with Ehlers-Danlos syndrome. In some forms of EhlersDanlos syndrome, the varicosities can be extreme.
Management Ascorbic acid (vitamin C). I recommend to my patients with Ehlers-Danlos syndrome that they take 2 to 4 gm of ascorbic acid daily. Children, of course, are given appropriately lower doses. This medication should be taken with a full glass of water or other fluid. Asdorbic acid is ordinarily safe, although it is metabolized b y the body to oxalic acid. Since this compound may be found as a part of some kidney stones, it is important to ensure that the patient has not had previous kidney stones and does not have a history of kidney stones in the family. Under these circumstances the advice of a physician is imperative. Doses larger than those recommended are not appropriate and will not be of additional value. All ascorbic acid, regardless of source, is the same, and comparison shopping is encouraged. We find that ascorbic acid is a big help with bruising and in many cases has improved wound healing and muscular strength. Recently we have learned in the laboratory that vitamin C instructs the body to make collagen. Wound repair. In families afflicted with poor wound healing, one of the most disappointing results is the cosmetic disfigurement that m a y result. This ordinarily occurs in the first 3 or 4 years of life and is a result of the thinness and fragility of the skin and poor wound healing, Injury is especially risky as the child attempts
Journal of the American Academy of Dermatology
to learn how to walk on loose, unstable joints. During this period, extra care is necessary to prevent falls. Furniture should be appropriately padded or avoided, and any obstacles such as loose carpets should be removed. An ounce of prevention is extremely important here. When lacerations occur, appropriate care is important. Such families should enlist the services of a plastic surgeon who knows about this disorder so that optimal results can be anticipated. Because of fragility of the skin and underlying connective tissue, it is often necessary to utilize special closure technics to achieve good results. The procedure includes taping as well as suturing. In some cases, joints should be partially immobilized to maximize the results. Prevention of wrinkles. Because the skin is already thin in Ehlers-Danlos syndrome, premature aging of the skin is an added risk. Since the sun markedly accelerates this process,.it is prudent to protect exposed skin from the sun. Sunscreen should be used daily but especially when sun exposure is contemplated. Several high-quality sunscreens are available on the market, and from the many available, patients should choose one with a sun protection factor of 15. Sunscreens can be obtained in cream, lotion, or clear gel formulations. In addition, some formulations are water resistant. Since patient acceptance usually dictates choice, the best approach is to try several of the products and judge for yourself. Wearing a wide-brimmed hat is a simple and effective measure. Care should especially be taken with infants and children to protect them when they are in the sun.
Sheldon R. Pinnell, M.D. Duke University Medical Center Durham, NC 27710
Melanomas arising in married couples To the Editor: This is to report a phenomenon of melanomas arising in each partner of a married couple, an extremely rare occurrence.
Case reports. Reports of Cases 1 and 2 follow. Case 1. The patient was a 67-year-old white woman who had a history of a nevus removed from the right tibia in 1971. She was told to go elsewhere for further treatment; however, the lesion seemed to heal and she did not see a physician until Aug. 1, 1984, when she came to us with an 8.5 X 6-cm brown and red plaque with an irregular border and a 2-cm~ central area consisting of an entrusted nodule. This malignant melanoma, type undetermined, with a fiat area and a depth of invasion of 0.8 ram, was widely excised and grafted. A lymph node dissection was done and the nodes were found to be benign. To the time of writing she had done nicely. Case 2. A 72-year-old white man, seen on Jan. 13, 1986,
Volume 16 Number 2, Part 1 February 1987
gave a history of a change in a congenital nevus on his right shoulder. On physical examination there was a 3 x 3.2-cm tan, slightly raised nodule with red, white, and blue discolorations and an irregular border. This was a melanoma in situ and wide excision was the treatment used.
Review of literature. To date there are very few reports of melanoma in conjugal pairs in the literature. Robertson J reported a case in 197l. The woman, 41 years of age, had a malignant melanoma on the right lower leg and her husband had had one on the back 1V~ years previously. In this instance, a 41-year-old woman was diagnosed as having a malignant melanoma on the right lower leg and her husband's lesion on his back was also diagnosed 1V2 years earlier as malignant melanoma. In 1978, three cases of married couples with melanoma were reported by Mintzes et al) Russ and Scanlon 3reported the largest group, consisting of eleven married couples with malignancies; however, only two had melanomas. Discussion. Familial incidences of melanoma and dysplastic nevi have been documented. The possibility of genetic influence in conjugal pairs exists because certain types of individuals tend to intermarry because of heritage, tradition, or religious considerations. Environmental factors also play a role. Connubial pairs live and play in similar regions and share similar lifestyles. Chemical exposure in the home, for instance, may be implicated, as well as the family's recreational patterns and sun exposure, for example, boating and tennis. According to one estimation by Mintzes et al, 2 melanoma in both spouses could have been expected to occur in about 132 couples between Jan. I, 1950, and Feb. 1, 1976 in the United States. However, in the New York study "more than a fair share" of melanomas were reported in married partners. The possibility must be considered that this geographic area, with its heavy concentration of industry, may represent a conducive environment for the occurrence of melanoma. Further studies are needed to evaluate this phenomenon over the entire population. There is an apparent need for dermatologists to be aware of this potential in order that a more accurate epidemiologic assessment of the situation can be made. LindaSusan Marcus, M.D. 260 Godwin Ave., Wyckoff, NJ 07481
REFERENCES 1. Robertson MG, Malignant melanoma in husband andwife. JAMA 1971;217:1553.
Correspondence
401
2. Mintzes MJ, Berger AP, Greenwald E, et al. Malignant melanoma in spouses. Cancer 1978;42:804-7. 3. Russ JE, Scanlon EF. Identical cancers in husband and wife. Surg Gynecol Obstet 1980;150:664-6.
Retired doctors club To the Editor: If you are retired, lonesome, and bored, why not organize a retired doctors club? I organized one 3 years ago. We have fifty members and meet once a month for lunch and a program. The retired doctors really enjoy these meetings and fellowship. I would be glad to provide information to anyone interested in beginning such a club. Hervey A. Foerster, M.D. J503 Camden Way, Oklahoma City, OK 73116
Topical (bathwater) PUVA therapy To the Editor: The contribution of Lowe et al regarding topical bathwater delivery of 8-methoxypsoralen is of great interest (Lowe N J, Weingarten D, Bourget T, Moy LS. PUVA therapy for psoriasis: Comparison of oral and bathwater delivery of 8-methoxypsoralen. J AM ACAD DERMATOLt986;14:754-60). The observation of a declining minimal phototoxic dose threshold with consecutive treatments is important. The authors propose that " a n accumulation of psoralen in the skin" occurs. We have previously demonstrated ~ that topical application of aqueous 8-methoxypsoralen solution (0.003%) to human skin causes quite short-lived photosensitization, with undetectable sensitivity after 24 hours. If, however, topical application is followed immediately by exposure to as little as 1/16th of the minimal phototoxic dose of ultraviolet A (UVA) radiation, the area remains photosensitive for at least 72 hours (the phenomenon was much smaller in magnitude following oral methoxsalen). This suggests that radiation is necessary for the persistence of photosensitivity, and perhaps for the effect observed by Lowe et al. It appears that psoralen may be photobound in the skin by subphototoxic exposures. A hypothesis is that topical psoralen application immediately followed by exposure by subphototoxic doses of radiation generates a population of monoadducts that are removed rather slowly. Subsequent exposure, up to 72 hours later, results in cross-linking and cutaneous phototoxicity. This phenomenon may explain some of the "unpredictable" bullous phototoxicity reactions observed after topical treatment: while the initial therapeutic exposure
Correspondence
called pseudotumors, that are ordinarily located over the elbows and knees. In addition, patients with EhlersDanlos syndrome may have small, hard, calcified lumps under the skin that are probably related to injury and subsequent repair. These processes are in no way dangerous and are not related to cancer. Bruising. Patients with Ehlers-Danlos syndrome usually bruise easily. Sometimes the resulting bruise is extensive, but clotting of blood is ordinarily normal. Since the bruising results from a problem in the structure of the underlying connective tissue, it is difficult for blood vessels to close themselves after they have been damaged. Moreover, they are more easily damaged because the connective tissue holding them together is abnormal. In a sense the processs resembles poking a pin-sized hole in a rubber sheet. Before long the hole becomes much larger than pin-sized. In a simple sense, this is what happens to the blood vessels when they are injured, Varicose veins. Since connective tissue in the entire body is involved, as well as the skin, the condition may lead to other manifestations. Varicose veins, particularly on the lower extremities, are common in patients with Ehlers-Danlos syndrome. In some forms of EhlersDanlos syndrome, the varicosities can be extreme.
Management Ascorbic acid (vitamin C). I recommend to my patients with Ehlers-Danlos syndrome that they take 2 to 4 gm of ascorbic acid daily. Children, of course, are given appropriately lower doses. This medication should be taken with a full glass of water or other fluid. Asdorbic acid is ordinarily safe, although it is metabolized b y the body to oxalic acid. Since this compound may be found as a part of some kidney stones, it is important to ensure that the patient has not had previous kidney stones and does not have a history of kidney stones in the family. Under these circumstances the advice of a physician is imperative. Doses larger than those recommended are not appropriate and will not be of additional value. All ascorbic acid, regardless of source, is the same, and comparison shopping is encouraged. We find that ascorbic acid is a big help with bruising and in many cases has improved wound healing and muscular strength. Recently we have learned in the laboratory that vitamin C instructs the body to make collagen. Wound repair. In families afflicted with poor wound healing, one of the most disappointing results is the cosmetic disfigurement that m a y result. This ordinarily occurs in the first 3 or 4 years of life and is a result of the thinness and fragility of the skin and poor wound healing, Injury is especially risky as the child attempts
Journal of the American Academy of Dermatology
to learn how to walk on loose, unstable joints. During this period, extra care is necessary to prevent falls. Furniture should be appropriately padded or avoided, and any obstacles such as loose carpets should be removed. An ounce of prevention is extremely important here. When lacerations occur, appropriate care is important. Such families should enlist the services of a plastic surgeon who knows about this disorder so that optimal results can be anticipated. Because of fragility of the skin and underlying connective tissue, it is often necessary to utilize special closure technics to achieve good results. The procedure includes taping as well as suturing. In some cases, joints should be partially immobilized to maximize the results. Prevention of wrinkles. Because the skin is already thin in Ehlers-Danlos syndrome, premature aging of the skin is an added risk. Since the sun markedly accelerates this process,.it is prudent to protect exposed skin from the sun. Sunscreen should be used daily but especially when sun exposure is contemplated. Several high-quality sunscreens are available on the market, and from the many available, patients should choose one with a sun protection factor of 15. Sunscreens can be obtained in cream, lotion, or clear gel formulations. In addition, some formulations are water resistant. Since patient acceptance usually dictates choice, the best approach is to try several of the products and judge for yourself. Wearing a wide-brimmed hat is a simple and effective measure. Care should especially be taken with infants and children to protect them when they are in the sun.
Sheldon R. Pinnell, M.D. Duke University Medical Center Durham, NC 27710
Melanomas arising in married couples To the Editor: This is to report a phenomenon of melanomas arising in each partner of a married couple, an extremely rare occurrence.
Case reports. Reports of Cases 1 and 2 follow. Case 1. The patient was a 67-year-old white woman who had a history of a nevus removed from the right tibia in 1971. She was told to go elsewhere for further treatment; however, the lesion seemed to heal and she did not see a physician until Aug. 1, 1984, when she came to us with an 8.5 X 6-cm brown and red plaque with an irregular border and a 2-cm~ central area consisting of an entrusted nodule. This malignant melanoma, type undetermined, with a fiat area and a depth of invasion of 0.8 ram, was widely excised and grafted. A lymph node dissection was done and the nodes were found to be benign. To the time of writing she had done nicely. Case 2. A 72-year-old white man, seen on Jan. 13, 1986,
Volume 16 Number 2, Part 1 February 1987
gave a history of a change in a congenital nevus on his right shoulder. On physical examination there was a 3 x 3.2-cm tan, slightly raised nodule with red, white, and blue discolorations and an irregular border. This was a melanoma in situ and wide excision was the treatment used.
Review of literature. To date there are very few reports of melanoma in conjugal pairs in the literature. Robertson J reported a case in 197l. The woman, 41 years of age, had a malignant melanoma on the right lower leg and her husband had had one on the back 1V~ years previously. In this instance, a 41-year-old woman was diagnosed as having a malignant melanoma on the right lower leg and her husband's lesion on his back was also diagnosed 1V2 years earlier as malignant melanoma. In 1978, three cases of married couples with melanoma were reported by Mintzes et al) Russ and Scanlon 3reported the largest group, consisting of eleven married couples with malignancies; however, only two had melanomas. Discussion. Familial incidences of melanoma and dysplastic nevi have been documented. The possibility of genetic influence in conjugal pairs exists because certain types of individuals tend to intermarry because of heritage, tradition, or religious considerations. Environmental factors also play a role. Connubial pairs live and play in similar regions and share similar lifestyles. Chemical exposure in the home, for instance, may be implicated, as well as the family's recreational patterns and sun exposure, for example, boating and tennis. According to one estimation by Mintzes et al, 2 melanoma in both spouses could have been expected to occur in about 132 couples between Jan. I, 1950, and Feb. 1, 1976 in the United States. However, in the New York study "more than a fair share" of melanomas were reported in married partners. The possibility must be considered that this geographic area, with its heavy concentration of industry, may represent a conducive environment for the occurrence of melanoma. Further studies are needed to evaluate this phenomenon over the entire population. There is an apparent need for dermatologists to be aware of this potential in order that a more accurate epidemiologic assessment of the situation can be made. LindaSusan Marcus, M.D. 260 Godwin Ave., Wyckoff, NJ 07481
REFERENCES 1. Robertson MG, Malignant melanoma in husband andwife. JAMA 1971;217:1553.
Correspondence
401
2. Mintzes MJ, Berger AP, Greenwald E, et al. Malignant melanoma in spouses. Cancer 1978;42:804-7. 3. Russ JE, Scanlon EF. Identical cancers in husband and wife. Surg Gynecol Obstet 1980;150:664-6.
Retired doctors club To the Editor: If you are retired, lonesome, and bored, why not organize a retired doctors club? I organized one 3 years ago. We have fifty members and meet once a month for lunch and a program. The retired doctors really enjoy these meetings and fellowship. I would be glad to provide information to anyone interested in beginning such a club. Hervey A. Foerster, M.D. J503 Camden Way, Oklahoma City, OK 73116
Topical (bathwater) PUVA therapy To the Editor: The contribution of Lowe et al regarding topical bathwater delivery of 8-methoxypsoralen is of great interest (Lowe N J, Weingarten D, Bourget T, Moy LS. PUVA therapy for psoriasis: Comparison of oral and bathwater delivery of 8-methoxypsoralen. J AM ACAD DERMATOLt986;14:754-60). The observation of a declining minimal phototoxic dose threshold with consecutive treatments is important. The authors propose that " a n accumulation of psoralen in the skin" occurs. We have previously demonstrated ~ that topical application of aqueous 8-methoxypsoralen solution (0.003%) to human skin causes quite short-lived photosensitization, with undetectable sensitivity after 24 hours. If, however, topical application is followed immediately by exposure to as little as 1/16th of the minimal phototoxic dose of ultraviolet A (UVA) radiation, the area remains photosensitive for at least 72 hours (the phenomenon was much smaller in magnitude following oral methoxsalen). This suggests that radiation is necessary for the persistence of photosensitivity, and perhaps for the effect observed by Lowe et al. It appears that psoralen may be photobound in the skin by subphototoxic exposures. A hypothesis is that topical psoralen application immediately followed by exposure by subphototoxic doses of radiation generates a population of monoadducts that are removed rather slowly. Subsequent exposure, up to 72 hours later, results in cross-linking and cutaneous phototoxicity. This phenomenon may explain some of the "unpredictable" bullous phototoxicity reactions observed after topical treatment: while the initial therapeutic exposure