CAMEO
OCCULT SYRINGOMA ASSOCIATED WITH ALOPECIA THOMAS N. HEEM, M.D., JOAN CUITART, M.D., WIEMA E. BERCEEED, M.D., AND ERNEST BENEDETTO, M.D.
A 50-year-old white woman presented for evaluation of thinning hair (Fig. 1). She had noted diffuse thinning of her scalp hair over the past 20 years and had been placed on minoxidil 2% solution by an outside dermatologist 7 months previously. The patient had used the minoxidil 2% solution twice daily, but noted no improvement. Physical examination revealed only diffuse alopecia of the scalp. Routine laboratory studies, luteinizing hormone, prolactin, follicle stimulating hormone, antinuclear factor studies, and thyroid hormone levels were all found to be normal. She was postmenopausal and was taking conjugated estrogens and medroxyprogesterone acetate. Her total testosterone and dehydroepiandrosterone-sulfate levels were lower than normal, and her sex hormone binding globulin level was increased. The elevation in sex hormone binding globulin was attributed to her estrogen therapy. She was otherwise in good health. Her cutaneous examination was otherwise unremarkable. She had
no history of malignancy and was without complaints of scalp pruritus. She had been instructed to continue minoxidil topically, but after noting no improvement after 12 months of treatment, gradually discontinued therapy on her own. She presented for re-evaluation of hair loss IV2 years later, approximately 8 months after discontinuing the minoxidil. Again, other than for diffuse thinning, the cutaneous examination of the scalp was unremarkable. To rule out other possible etiologies of diffuse hair loss, two scalp biopsies were performed in random areas of diffuse thinning. The biopsies revealed glandular structures embedded in dense collagen tissue in the mid and deep dermis (Fig. 2). The lining of these structures was composed of a double layer of epithelial cells. Large glandular cells with abundant clear cytoplasm were present on the luminal surface, and basaloid cells were located in the peripheral layer. Some of the cystic structures contained periodic acid-Schiff (PAS) positive material. Others were compressed by the dense fibrous matrix acquiring a "comma-like" shape. Atypia or mitoses were not identified. Hair follicles were decreased in number and some were miniaturized. The glandular structures impinged upon, but preserved adjacent epithelial adnexae. Collagen bundles were haphazardly arranged (Fig. 3), but no primary inflammatory or scarring process was identified. The epidermis was unremarkable.
DISCUSSION
We present this as a case of diffuse, female-pattern alopecia secondary to an occult syringoma. Although inflammatory and scarring processes may be accompanied
Figure 1.
Diffuse alopecia in a female androgenic pattern.
From the Department of Dermatology, Cleveland Clinic Foundation, Cleveland, Ohio. This paper was submitted for publication in 1989. Address for correspondence: Thomas N. Helm, M.D., Department of Dermatology, Cleveland Clinic Foundation, Cleveland, OH 44195-0338.
Figure 2. Glandular structures embedded in dense collagen (hematoxylin and eosin, original magnification x 40). 437
International Journal of Dermatology Vol. 31, No. 6, June 1992
liferation is not clearly established. In our case, the slow progression of the alopecic process over 20 years without signs of a systemic disease was evidence enough for us to conclude that this was a benign process. To our knowledge, this is the fourth such case.^^ The finding of occult syringoma in patients with diffuse hair loss underscores the shortcomings of clinical examination as the sole means of diagnosis and highlights the importance of histologic examination of progressive hair loss.
DRUC NAMES
conjugated estrogens: Premarin medroxyprogesterone acetate: Provera
Figure 3. Haphazard arrangement of collagen is noted around the glandular structures (hematoxylin and eosin, original magnification x 200).
REEERENCES
by occlusion and cystic changes of the eccrine gland,' eccrine proliferation was the primary process in our case. The haphazard distribution of collagen and differentiated epithelial cells seen in our case are suggestive of an adenomatous process. The mechanism by which syringomas may cause hair loss is unclear, but some have suggested that stromal changes may inhibit the development of hair follicles.^'^ The possibility of a metastatic carcinoma or a sclerosing eccrine carcinoma should be entertained when the benign nature of the epithelial pro-
1. 2. 3. 4.
Mehregan AH. Proliferation of sweat ducts in certain diseases of the skin. Am J Dermatopathol 1981; 3:27-31. Shelley WB, Wood MC. Occult syringomas of scalp associated with progressive hair loss. Arch Dermatol 1980; 116:843-844. Neuman KM, Burnett JW. Alopecia associated with syringomas. J Am Acad Dermatol 1985; 13:528-529. Trozak DJ, Wood C. Occult eccrine sweat duct hamartoma and cicatricial scalp alopecia. Cutis 1984; 34: 475-477.
Mercury and the Origin of Syphilis An interesting sidelight is thrown upon the origin of syphilis by the fact that mercury had been in use for more than three centuries against a variety of diseases of the skin; when, therefore, syphilis assumed the alleged epidemic proportions, physicians were not unfamiliar with its treatment. It is not an unreasonable assumption that some of the conditions in which mercury, since its introduction by the Arabs, had proved efficacious, may have been of syphilitic nature. It is customary to ascribe the first preparation of mercurial ointment to Giovanni da Vigo, but Gilbertus Anglicus preceded him; Jacopo Berengario da Carpi seems to have been the first to use it extensively in the treatment of syphilis, but, if we may believe Benvenuto Cellini, Berengario's cures were not lasting. Mercury was also used internally and in fumigations. Since the treatment whether by inunctions or by fumigations was not readily carried out at home, patients were sent to hospitals, which, up to this time, had been places of refuge or of isolation to prevent the spread of contagious diseases. Owing to the enormous quantities of mercury administered, salivation ^¥as common, in fact, it was considered essential to successful treatment. The result was that mercury fell into disfavor with many. Casparad Torella in 1497 wrote: Avoid, like the plague, these murderous ointments of charlatans who have already made so many victims. It is they who killed Cardinal Segorbe. Alphonse Borgia and his brother owe their premature death to these charlatans, who if they escape punishment on earth, will find it in Eternity. Erom Riesman D. The story of medicine in the Middle Ages. New York: Paul B. Hoeber Inc., 1935:299. 438
OCCULT SYRINGOMA ASSOCIATED WITH ALOPECIA THOMAS N. HEEM, M.D., JOAN CUITART, M.D., WIEMA E. BERCEEED, M.D., AND ERNEST BENEDETTO, M.D.
A 50-year-old white woman presented for evaluation of thinning hair (Fig. 1). She had noted diffuse thinning of her scalp hair over the past 20 years and had been placed on minoxidil 2% solution by an outside dermatologist 7 months previously. The patient had used the minoxidil 2% solution twice daily, but noted no improvement. Physical examination revealed only diffuse alopecia of the scalp. Routine laboratory studies, luteinizing hormone, prolactin, follicle stimulating hormone, antinuclear factor studies, and thyroid hormone levels were all found to be normal. She was postmenopausal and was taking conjugated estrogens and medroxyprogesterone acetate. Her total testosterone and dehydroepiandrosterone-sulfate levels were lower than normal, and her sex hormone binding globulin level was increased. The elevation in sex hormone binding globulin was attributed to her estrogen therapy. She was otherwise in good health. Her cutaneous examination was otherwise unremarkable. She had
no history of malignancy and was without complaints of scalp pruritus. She had been instructed to continue minoxidil topically, but after noting no improvement after 12 months of treatment, gradually discontinued therapy on her own. She presented for re-evaluation of hair loss IV2 years later, approximately 8 months after discontinuing the minoxidil. Again, other than for diffuse thinning, the cutaneous examination of the scalp was unremarkable. To rule out other possible etiologies of diffuse hair loss, two scalp biopsies were performed in random areas of diffuse thinning. The biopsies revealed glandular structures embedded in dense collagen tissue in the mid and deep dermis (Fig. 2). The lining of these structures was composed of a double layer of epithelial cells. Large glandular cells with abundant clear cytoplasm were present on the luminal surface, and basaloid cells were located in the peripheral layer. Some of the cystic structures contained periodic acid-Schiff (PAS) positive material. Others were compressed by the dense fibrous matrix acquiring a "comma-like" shape. Atypia or mitoses were not identified. Hair follicles were decreased in number and some were miniaturized. The glandular structures impinged upon, but preserved adjacent epithelial adnexae. Collagen bundles were haphazardly arranged (Fig. 3), but no primary inflammatory or scarring process was identified. The epidermis was unremarkable.
DISCUSSION
We present this as a case of diffuse, female-pattern alopecia secondary to an occult syringoma. Although inflammatory and scarring processes may be accompanied
Figure 1.
Diffuse alopecia in a female androgenic pattern.
From the Department of Dermatology, Cleveland Clinic Foundation, Cleveland, Ohio. This paper was submitted for publication in 1989. Address for correspondence: Thomas N. Helm, M.D., Department of Dermatology, Cleveland Clinic Foundation, Cleveland, OH 44195-0338.
Figure 2. Glandular structures embedded in dense collagen (hematoxylin and eosin, original magnification x 40). 437
International Journal of Dermatology Vol. 31, No. 6, June 1992
liferation is not clearly established. In our case, the slow progression of the alopecic process over 20 years without signs of a systemic disease was evidence enough for us to conclude that this was a benign process. To our knowledge, this is the fourth such case.^^ The finding of occult syringoma in patients with diffuse hair loss underscores the shortcomings of clinical examination as the sole means of diagnosis and highlights the importance of histologic examination of progressive hair loss.
DRUC NAMES
conjugated estrogens: Premarin medroxyprogesterone acetate: Provera
Figure 3. Haphazard arrangement of collagen is noted around the glandular structures (hematoxylin and eosin, original magnification x 200).
REEERENCES
by occlusion and cystic changes of the eccrine gland,' eccrine proliferation was the primary process in our case. The haphazard distribution of collagen and differentiated epithelial cells seen in our case are suggestive of an adenomatous process. The mechanism by which syringomas may cause hair loss is unclear, but some have suggested that stromal changes may inhibit the development of hair follicles.^'^ The possibility of a metastatic carcinoma or a sclerosing eccrine carcinoma should be entertained when the benign nature of the epithelial pro-
1. 2. 3. 4.
Mehregan AH. Proliferation of sweat ducts in certain diseases of the skin. Am J Dermatopathol 1981; 3:27-31. Shelley WB, Wood MC. Occult syringomas of scalp associated with progressive hair loss. Arch Dermatol 1980; 116:843-844. Neuman KM, Burnett JW. Alopecia associated with syringomas. J Am Acad Dermatol 1985; 13:528-529. Trozak DJ, Wood C. Occult eccrine sweat duct hamartoma and cicatricial scalp alopecia. Cutis 1984; 34: 475-477.
Mercury and the Origin of Syphilis An interesting sidelight is thrown upon the origin of syphilis by the fact that mercury had been in use for more than three centuries against a variety of diseases of the skin; when, therefore, syphilis assumed the alleged epidemic proportions, physicians were not unfamiliar with its treatment. It is not an unreasonable assumption that some of the conditions in which mercury, since its introduction by the Arabs, had proved efficacious, may have been of syphilitic nature. It is customary to ascribe the first preparation of mercurial ointment to Giovanni da Vigo, but Gilbertus Anglicus preceded him; Jacopo Berengario da Carpi seems to have been the first to use it extensively in the treatment of syphilis, but, if we may believe Benvenuto Cellini, Berengario's cures were not lasting. Mercury was also used internally and in fumigations. Since the treatment whether by inunctions or by fumigations was not readily carried out at home, patients were sent to hospitals, which, up to this time, had been places of refuge or of isolation to prevent the spread of contagious diseases. Owing to the enormous quantities of mercury administered, salivation ^¥as common, in fact, it was considered essential to successful treatment. The result was that mercury fell into disfavor with many. Casparad Torella in 1497 wrote: Avoid, like the plague, these murderous ointments of charlatans who have already made so many victims. It is they who killed Cardinal Segorbe. Alphonse Borgia and his brother owe their premature death to these charlatans, who if they escape punishment on earth, will find it in Eternity. Erom Riesman D. The story of medicine in the Middle Ages. New York: Paul B. Hoeber Inc., 1935:299. 438
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