ARTICLE
A 40-year-old Philippino woman presented with multiple small papules on
both lower eyelids and the upper part of her cheeks. None of her family
members had similar lesions. Although she had been treated with insulin
for diabetes mellitus since 1994, the control of the disease was poor. The
papules appeared in 1997 and have gradually increased in number. Physical
examination revealed many flesh-colored or slightly reddish, smooth-surfaced,
half-grain sized papules on her lower eyelids and the upper part of her
cheeks (Fig. 1). Abnormal
laboratory data were as follows: triglyceride: 255 mg/dl (35-130 mg/dl);
blood sugar: 285 mg/dl (2 hrs after meal); hemoglobin A1c: 10.2% (4-6%).
Histopathological examination revealed some cell nests consisting of clear
cells in the upper dermis. The cell nests were surrounded by fibrous stroma,
and contained amorphous material in the intraductal space. The clear cell
had a large vacuolated cytoplasm with a small hyperchromatic nucleus in
the pericytoplasm (Fig. 2).
Periodic acid-Schiff (PAS) staining showed reactive spots in the cytoplasm
and the intraductal space. The PAS-positive reaction was diminished after
diastase digestion.
Clear cell syringoma
Both clinical and pathological findings are considered to be consistent
with clear cell syringoma. Although the re-initiated therapy for diabetes
mellitus has controlled her disease well, the papules have remained unchanged.
Comments
Clear cell syringoma was first described by Headington et al.
in 1972 [1]. It has been recognized to be one of the dermadromes associated
with diabetes mellitus [2]. In most cases, multiple miliary-sized papules
are seen on both eyelids. In some cases [3], however, the papules become
larger than ordinary syringoma as in our case. Although clear cells are
sometimes noted in ordinary syringomas, the cells in clear cell syringoma
are inclined to proliferate and have a smaller nucleus and dense chromatin
[4].
The pathogenesis of clear cell syringoma is still unknown. The PAS-positive
substance in the clear cell is considered to be glycogen [5]. Phosphorylase
activity in clear cell syringoma was reported to be suppressed in some
cases [1, 6]. Headington inferred that the clear cells undergo glycogenesis
because of a relative deficiency in phosphorylase [1]. In contrast, Saitoh
et al. [7] claimed that phosphorylase activity in syringoma may
be normal, but elevated glucose levels in diabetics may suppress the activity.
Recently, Ohnishi et al. demonstrated that clear cell syringoma
showed differentiation into the transitional portion between the acrosyringium
and the dermal duct, as in conventional syringoma [8]. They concluded
that clear cell syringoma is a metabolic variant of ordinary syringoma.
Article accepted on 31/8/00
References
1. Headington JT, Koski J, Murphy PJ. Clear cell glycogenosis
in multiple syringoma. Arch Dermatol 1972; 106: 353-6.
2. Nakabayashi Y, Niimura M, Hori Y. A case of clear cell syringoma
(in Japanese). Rinsho Dermatol (Tokyo) 1980; 22: 599-603.
3. Takeshige M, Yamamoto A, Inomata N. Clear cell syringoma with
milia-like papules (in Japanese). Jpn J Clin Dermatol (Tokyo) 1988;
42: 19-23.
4. Nakamura Y, Sueki H, Yasuki Y, Iijima M, Fujisawa R. A case
of clear cell syringoma (in Japanese). Jpn J Clin Dermatol (Tokyo)
1991; 45: 891-5.
5. Feibelman CE, Maize JC. Clear-cell syringoma. A study by conventional
and electron microscopy. Am J Dermatopathol 1984; 6: 139-50.
6. Furue M, Hori Y, Nakabayashi Y. Clear-cell syringoma, association
with diabetes mellitus. Am J Dermatopathol 1984; 6: 131-8.
7. Saitoh A, Ohtake N, Fukuda S, Tamaki K. Clear cells of eccrine
glands in a patient with clear cell syringoma associated with diabetes
mellitus. Am J Dermatopathol 1993; 15: 166-8.
8. Ohnishi T, Watanabe S. Immunohistochemical analysis of keratin
expression in clear cell syringoma. J Cutan Pathol 1997; 24: 370-6.
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