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| CASE REPORT |
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| Year : 1997 | Volume
: 63
| Issue : 6 | Page : 373-375 |
Familial syringoma
LK Gupta, M Raman
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Correspondence Address:
L K Gupta
PMID: 20944383
Three patients with a family history of syringomas seen over a 12-month period are reported. Familial syringomas may not be as rare as generally believed.
Keywords: Skin tumours, Inherited, Syringoma
How to cite this article:
Gupta L K, Raman M. Familial syringoma. Indian J Dermatol Venereol Leprol 1997;63:373-5 |
There are only a few reports of the familial occurrence of syringomas. In fact, a major textbook of dermatology states that syringomas do not appear to be hereditary. [1] We report 3 patients seen over the period of one year in whom the condition occurred in a familial pattern.
| Case Reports | |  |
Case 1 : A 20-year-old female presented with multiple asymptomatic, skin-colored, 2-5 mm sized, firm, flat and domed papules scattered over the eyelids and upper cheeks [Figure - 1]. The lesions had developed around the age of 15 and had shown little change over the years. Four female relatives were stated to have similar lesions since adolescence. We were able to examine three the patient's mother, grand mother and an aunt. All the three showed similar papules around the eyes and cheeks. The lesions in the mother and grandmother were larger, firmer and more numerous compared to the proband. All were in good health and the rest of the cutaneous and systemic examination was unremarkable. There was no history of consanguinity in the family. We performed skin biopsies in the patient and her mother. Histopathological examination in both showed the presence of cystic structures lined by two layers of cuboidal epithelium in the reticular dermis. In addition, solid strands of epithelial cells having a basaloid appearance were also seen interspersed between the cystic structure.
Case 2 : Typical asymptomatic, shiny, flat and angulated papules were seen in a 45-year-old female. The lesions appeared at the age of 33 and were predominantly distributed around the eyelids and cheeks. Three other members of her family - daughter, sister and brother had similar lesions predominantly around the eyelids. We could examine her daughter, aged 25 who had the typical lesions of syringoma which had appeared in adolescence. Histopathological examination of lesions in both mother and daughter showed features of syringoma.
Case 3 : A 30-year-old male had shiny angulated papules on eyelids and scattered lesions on cheeks for the past 15 years. These were confirmed to be syringoma on histopathology. Five other maternal relatives a brother, a first cousin (female), an aunt, an uncle and grandmother were stated to have similar lesions on the face. However, no other member of the family was available for examination.
| Discussion | | |
Syringoma is a benign appendageal tumour of eccrine acrosyringeal differentiation which usually manifests as multiple, flesh-colored papules around the eyelids and upper cheeks of adolescent females [2] Syringoma appears to be an exception to the general rule that solitary appendageal tumours are sporadic while multiple tumours most often are inherited. [3],[4] In most reported families, syringomas have been observed to occur in an autosomal dominant pattern and the condition tends to be more common in females. We observed a similar pattern in our cases. This female preponderance in both sporadic and familial forms of syringoma is well recognised but remains unexplained.
While some inherited multiple tumours have been reported to be associated with other cutaneous and extra cutaneous lesions including neoplasias, there is only a single report of palmar keratoses associated with eyelid syringomas [7]sub No other association have been described, and none were detected in our patients or their relatives. In view of the paucity of previous reports, it is intriguing that we encountered 3 families with syringomas over a 12-month period. While this may have been due merely to chance, we believe that familial occurrence of the tumour may not be as rare as generally believed[8].
| References | | |
| 1. | Mackie RE. Tumours of the skin appendages. Textbook of Dermatology, 5th edition, edited by Champion R H, Burton J L, Ebling FJG, Blackwell Scientific Publications oxford 1992; 1519.  |
| 2. | Weedon D. Eccrine tumours : A selective review. J Cutan Pathol 1984; 11: 421-436. [PUBMED] |
| 3. | Brownstein M H, Shapiro L. Desmoplastic trichoepithelioma. Cancer 1977; 40 : 2979. |
| 4. | Jelinek j E. Aspects of hereditary, syndromic associations and course of conditions in which cutaneous lesions occur solitarily or in multiplicity. J Am Acad Dermatol 1982; 7: 526. |
| 5. | Woringer F, Eichler A. Constatation et reflexion au suject d'un cas d'hidrenoma eruptive. Annls Derm Syph 1951; 78: 152-164. [PUBMED] |
| 6. | Yesudian P, Thambiya A. Familial syringoma, Dermatologica 1975; 150: 32-35. |
| 7. | Baden HP. Hereditary syringoma. Arch Dermatol 1977;113:1133. |
| 8. | Hashimoto K, Blum D, Fukuya T, et al. Familial syringoma. Case history and application of monoclonal anti-eccrine gland antibodies. Arch Dermatol 1985; 121: 756-760. |
Figures
[Figure - 1], [Figure - 2]
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