Syringoma presenting as milia

Syringoma presenting as milia

Journal of the American Academy of Dermatology Thornfeldt and Menkes sponsive to minocycline hydrochloride. Cutis 1978; 21:535-8. 35. Davies MG, Pip...

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Journal of the American Academy of Dermatology

Thornfeldt and Menkes

sponsive to minocycline hydrochloride. Cutis 1978; 21:535-8. 35. Davies MG, Piper S. Pyoderma gangrenosum: successful therapy with minocycline. Clin Exp Dermatol 1980; 6:219-23. 36. Shelley WB. Demethylchlortetraeycline and griseofulvin as examples of specific treatment for mycosis fungoides. Br J Dermatol 1981;104:477.

37. Thomsen K. Mycosis fungoides responding to demethylchlortetracycline and griseofulvin. Br J Dermatol 1981; 105:483-4. 38. Berk MA, Lorinez AL. The treatment of bullous pemphigoid with tetracycline and niacinamide. Arch Dermatol t986;122:670-4.

Syringoma presenting as milia* Stephen J. Friedman, Major, MC, USA, and David F. Butler, Major, MC, USA

El Paso, TX We present two unrelated patients with numerous infraocular milium-like lesions that histologically revealed syringoma with many overlying keratin cysts in the papillary dermis. A Fontana-Masson stain revealed no melanincontaining cells in the keratin cyst walls, suggesting that they originated from eccrine ductal structures. A classification of the clinical variants of syringoma is presented. (J AM ACAD DERMATOL 1987; 16:310-4.)

Syringomas are common benign appendageal tumors derived from the intraepidermal eccrine ducts. ~'2 Although sometimes solitary, 3 syringomas usually are multiple and localized, 4.'a although they m a y be generalized. ~4,~5 Usually the lesions are small, firm, dermal papules ranging in size f r o m a few millimeters to 1 cm. Ordinarily, they are flesh-colored but may be in shades o f red, tan, or brown. Multiple lesions may simulate multiple basal cell carcinomas, pigmented nevi, trichoepitheliomas, angiofibromas, cylindromas, and lichen p l a n u s - l i k e lesions? '7'~4 W e found no mention in the literature of syringoma presenting as milium-like lesions. We report two patients with multiple clustered infraorbital milium-like lesions with typical histologic findings o f syringoma. From the Department of Internal Medicine DermatologyService, William BeaumontArmy Medical Center. Accepted for publicationJuly 17, 1986. Reprint requests to: Dr. StephenJ. Friedman, DermatologyService, William BeaumontArmy MedicalCenter, E1 Paso, TX. *The opinions or assertions containedherein are the private views of the authors and are not to be construedas officialor as reflecting the views of the Departmentof Defense. 310

CASE REPORTS Case 1 A 23-year-old white woman presented to the William Beaumont Army Medical Center Dermatology Clinic in August 1985, complaining of the gradual appearance of grouped white papules on the lower eyelids and upper aspect of the cheeks for 3 years. The lesions were asymptomatic but were of cosmetic concern. There was no history of local trauma. The patient was in excellent health. No other family members had a similar problem. Examination revealed multiple white, firm, globoid papules measuring 1-3 mm in diameter on the lower eyelids and upper aspect of the cheeks bilaterally (Fig. 1). No similar lesions were present elsewhere.

Case 2 A 20-year-old Latin-American man presented to the dermatology clinic in April 1986, complaining of the gradual appearance of grouped white papules on the lower eyelids and upper aspect of the cheeks for 3 years. The lesions were asymptomatie but were of cosmetic concern. There was no history of local trauma. The patient was in excellent health. No other family members had a similar problem. Physical examination revealed multiple white, firm, globoid papules measuring 1-3 mm in diameter on the

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Syringoma presenting as milia

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Fig. 1. Solitary and clustered milium-like lesions on the lower eyelid and cheek regions. lower eyelids and upper aspect of the cheeks bilaterally. No similar lesions were present elsewhere. HISTOLOGIC RESULTS Punch biopsy specimens, 4 mm and 3 ram, were obtained from a milium-like lesion on the left lower eyelid from Patients 1 and 2, respectively, and were essentially identical histologically. There were numerous cystic structures containing concentric lamellae of keratin (Fig. 2). They were lined by a few cell layers of stratified epithelium with a thin or single-cell layer of stratum granulosum. The papillary and mid dermis contained many small ductal structures lined by two rows of epithelial cells embedded iri a dense, fibrous strorna (Fig. 3). The lumina were filled with amorphous hyaline material. Some ductal structures showed a commalike tail of epithelial cells; serial sections revealed an extension of the keratin cyst toward these structures. A Fontana-Masson stain revealed melanin-containing cells in the epidermis but none in the keratin-filled cyst walls (Fig. 4). DISCUSSION Syringomas usually appear on the infraocular skin of otherwise healthy individuals. Lesions may develop at any age, and they occur predominantly in women. The initial appearance may be during adolescence 15-17 or as late as the sixth or seventh decades, 3,6,n,~8 but it is most frequent during the third and fourth decades. Familial occurrence has been reported, 17~9 and syringomas are found with

Fig. 2. Keratin-filled cysts are concentrated in the pap-

illary and upper reticular dermis. Below are numerous cystic ducts and epithelial strands within a fibrous stroma. (Hematoxylin-eosin stain; x 40.) increased frequency in Japanese women 2° and in patients with Down's syndrome. ~6.20 The histogenesis of the syringoma was debated for many years. However, histochemical,J.21 electron microscopic,21 and monoclonal antikeratin an-

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Journal of the American Academy of Dermatology

Fig. 3. Numerous solid strands of epithelial cells and small cystic ducts containing colloidal material. (Hematoxylin-eosin stain; x 100.)

Fig. 4. Numerous melanin-containing cells within the epidermis; however, there is no melanin within the cells lining the cystic structure. (Fontana-Masson stain; × 120.) tibody studies 19 support the theory that syringomas are of eccrine duct differentiation. There are many clinical variants of syringoma, and there are many reports of the occurrence of this tumor in unusual distributions '2.'3 and locations. 4'6-tt A rare variety, eruptive hidradenoma, 14-~6generally occurs on the anterior half of the body of young adults; histologic, histochemical, electron microscopic,'4 and monoclonal antibody technics ~8 have shown that it is of eccrine

differentiation and that it is identical to "ordinary" syringoma. No previous attempts at classifying syfingoma according to the clinical features of individual lesions have been made. We present a classification of syringoma according to clinical features and associations (Table I). Noncicatricial alopecia associated with occult syringomas 4'6 and a lichen planus-like lesion of syringoma have been describedT'14; there were no histologic features of

Volume 16 Number 2, Part 1 February 1987

these clinical variants that would distinguish them from an "ordinary" syfingoma. Syringomas have a characteristic histologic presentation. ~.2,z4,2~As illustrated in our patients, however, the presence of solid strands of basaloid cells embedded in a fibrous stroma and of horn cysts near the epidermis resembles desmoplastic trichoepithelioma or sclerosing basal cell epithelioma. = The existence of ductal structures containing amorphous material suggests syfingoma. Certain histologic variations of syringoma may occur. Clear cell syringoma has been reported in which ductal cells develop a large amount of clear cytoplasm that is rich in glycogen. ~s.t8 Cystic ductal lumina filled with keratin and lined by cells containing keratohyaline granules were noted in the papillary dermis of some of the patients, 3'14"~9'= including ours. The keratin cysts may become large and sometimes rupture, producing a foreignbody reaction, perhaps with calcification or actual bone formation. ~'= Excluding our patients, there has been no mention of distinctive clinical and morphologic features of these histologic variants. The clinical and histologic features of the keratin-filled cystic lesions from our patients were typical of milia. ~3 To our knowledge, there have been no previous cases of syringoma presenting as milia. Despite the presence of keratin-filled cysts histologically, there was no mention of clinical evidence of milia associated with the syringoma. 3,~4a9,22 Milia are small epidermal cysts that are thought to arise from pluripotential cells of the cutaneous epithelial system, including aberrant epidermis, hair follicles, sebaceous glands, and eccrine sweat ducts. They are classified as primary and secondary types. Primary milia (common "whiteheads") arise spontaneously in the skin in predisposed individuals, and secondary milia develop in the setting of underlying factors, such as bullous disease or trauma. Primary milia grow from the undifferentiated sebaceous collar, and secondary milia arise from either hair follicles or eccrine ducts. 23 In our patients the milia arose as part of the tumor, without antecedent trauma, and should be considered secondary milia. Fontana-Masson staining of a lesion from each of our patients revealed no melanin-containing

Syringoma presenting as milia

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Table I, Clinical variants of syringoma

I.

Localized A. SolitaryTM B. Multiple-unifocal 1. Papular a. Infraocular~s,2° b. Genital~a c. AcraPT M d. UnilateraP 2 and unilateral linear nevoidaP a 2. Occult a. Scalp: alopecia4'a 3. Lichen planus-like a. Genital7 4. Milium-like a. Infraocular* II. Generalized A. Multifocals's'9 B. Eruptive 1~a6 1. Lichen planus-like t4 III. Down's syndromel~a° IV. Familial t7"19 *Present study.

cells in the keratin-filled cyst wails, illustrating that its origin was not from the epidermis but probably from the eccrine duct. Previous electron microscopic studies of the keratin-filled cysts by Hashimoto et a114 revealed keratohyaline granule formation identical to that of the embryonic epidermal eccrine duct and is consistent with the natural keratinizing propensity of the luminal cells of the intra-epidermal eccrine duct toward the upper strata of the epidermis. ~4 In summary, we present two patients who developed multiple localized syringomas in the infraorbital and upper cheek areas clinically presenting as milia, a previously unreported presentation. We further propose a classification of syringoma by distribution and morphologic features to include a milium-like presentation, because of unique clinical and histologic patterns. REFERENCES

1. WinkelmannRK, Gottlieb BE Syringoma:an enzymatic study. Cancer 1963;16:665-9. 2. Goltz RW. Syringoma. In: Demis DJ, ed. Clinical dermatology. New York: Harper & Row, 1981:1-4. 3. Port M, FarmerER. Syringoma of the ankle [letter]. J AM ACADDERMATOL1984;10:291-2. 4. Shelley WB, Wood MG. Occult syringomas of scalp

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5. 6. 7. 8. 9. 10. 11, 12. 13.

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F r i e d m a n and Butler

associated with progressive hair loss. Arch Dermatol 1980;116:843-4. Dupre A, Bonafe JL, Christol B. Syringomas as a causative factor for cicatricial alopecia [letter]. Arch Dermatol 1981;117:315. Neuman KM, Bumett JW. Alopecia associated with syringomas [letter]. J AM ACAD DERMATOL 1985;13: 528-9. Zalla JA, Perry HO. An unusual case of syringoma. Arch Dermatol 1971;103:215-7. Cameiro SJC, Gardner HL, Knox JM. Syringoma of the vulva. Arch Dermatol 1971;103:494-6. Cameiro SJC, Gardner HL, Knox JM. Syringoma: three cases with vulvar involvement. Obstet Gynecol 1972; 39:95-9. Hughes PSH, Apisamthanarax P. Acral syringoma. Arch Dermatol 1977; 113:1435-6. Van den Breck H, Lundquist CD. Syringomas of the upper extremities with onset in the sixth decade. J AM AcAO Dr~RMATOL1982;6:534-6. Wilms NA, Douglass MC. An unusual case of predonderantly right-sided syringomas. Arch Dermatol 1981; 117:308. Yung CW, Soltani K, Bernstein JE, Lorincz AL. Unilateral linear nevoidal syringoma, J AM ACADDERMATOL 1981;4:412-6.

14. Hashimoto K, Dibella RJ, Borsuk GM, Lever WF. Eruptive hidradenoma and syringoma: histological, histochemical, and electron microscopic studies. Arch Dermatol 1967;96:500-19. 15. Diestelmeier MR, Rodman OG. Eruptive generalized clear cell syringomas. Arch Dermatol 1983; 119:927-9. 16. Urban CD, Cannon JR, Cole RD. Eruptive syringomas in Down's syndrome. Arch Dermatol 1981;117:374-5. 17. Baden HP. Hereditary syringomas [letter]. Arch Derm a t o l 1977;113:1133. 18. Headington JT, Kowki H, Murphy PJ. Clear cell glycogenosis in multiple syringomas: description and enzyme histochemistry. Arch Dermatol 1972;106:353-6. 19. Hashimoto K, Blum D, Fukaya T, Eto H. Familial syringoma. Arch Dermatol 1985;121:756-60. 20, Butterworth T, Strean L, Beerman H, et al, Syringoma and mongolism. Arch Dermatol 1964;90:483-7. 21. Hashimoto K, Gross BG, Lever WF, Syringoma: histoehemical and electron mleroscopic studies. J Invest Derrnatol 1966;46:150-66. 22. Lever W, Schaumburg-Lever G. Histopathology of the skin. 6th ed. Philadelphia: JB Lippincott, 1983:551-3, 23. Tsuji T, Sugai T, Suzuki S. The mode of growth of eccrine duet milia. J Invest Dermatol 1975;65:388-93.

ABSTRACTS Neurologic abnormalities in Lyme disease without erythema chronicum migrans Reik L Jr, Burgdoffer W, Donaldson JO. Am J Med 1986;81:73-8 Lyme disease may have no skin lesions and no arthritis. These eight patients had aseptic meningitis, encephalitis, cranial neuritis, or dementia. Lyme disease can be almost exclusively a neurologic problem. Philip C. Anderson, M.D.

Septal panniculitis as a manifestation of Lyme disease Kramer N, Rickert RR, Brodkin RH, Rosenstein ED. Am J Med 1986;81:149-52 As in many other infectious diseases, nodular vasculitis in the skin may be part of Lyme disease. Lots of different diseases can be confused with Lyme disease. Several diseases can cause erythema chronicum migrans other than Lyme Valley borreliosis. Philip C. Anderson, M.D.

Pemphigus in pregnancy: a reevaluation of fetal risk

Acanthosis nigricans, hypothyroidism, and insulin resistance

Ross MG, Kane B, Frieder R, Gurevitch A, Hayashi R. Am J Obstet Gynecol 1986;155:30-3

Matsuoka LY, Wortsman J, Gavin JR 3d, Kupchella CE, Dietrich JG. Am J Med 1986;81:58-62

Very uncommon is pemphigus in pregnancy.This report includes a ease of intetuterine death. The prior literature is mixed, many women do well, but a definiterisk is seen for the fetus. Philip C. Anderson, M.D.

Acanthosis nigricans is linked to an endocrinopathy. Insulin resistance may be connected often, as is shown in detail by a study of these two men and one woman. Philip C. Anderson, M.D.

Central retinal artery occlusion in Sneddon's disease associated with antiphospholipid antibodies

Severe cutaneous reactions to alternative remedies

Jonas J, K61ble K, Vflcker HE, Kalden JR. Am J Ophthalmol 1986;102:37-40 A number of syndromes seem to be variations of periarterttis nodosa. Sneddon's syndromeis severelivedo reficularisin skin, with labile hypertensionand neurologicdisease.The immunologysituation is mostly speculative. The focus is on antiphospholipaseantibodies in this report. Philip C. Anderson, M.D.

Monk B: Br Med J 1986;293:665-6 Three patients developed severe cutaneous reactions following "alternative" or "complementary" treatment. These were "golden health blood purifying tablets," containing a variety of roots, barks, etc., Nat Mur 200, a homoeopathic remedy, and topical application of Dead Sea mineral salts. J. Graham Smith, Jr., M.D.