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International Journal of Dermatology • November 1991
Vol. 30
Syringocystadenoma Papiliiferum: Report of an Unusual Case To the Editor: A 59-year-old white woman presented to our clinic with a slowly growing papule on the dorsum of her right hand present for 6 months. She desired removal hecause she experienced a sharp pain in the lesion with minimal manipulation. Examination revealed a 6-mm smooth, dome shaped, erythematous, firm papule on the proximal portion of the dorsal right hand (Fig, 1). In addition, there was a 2-mm eccentrically located dilated pore with a visible keratinous plug. Gentle palpation of this lesion caused a sharp pain, discomforting enough to cause the patient to wince, A deep shave biopsy was performed, which histologically revealed a multiloculated cystic tumor confined to the upper dermis, communicating to the surface via a wide mouthed ostium. Most of the cystic fining was keratinizing stratified squamous epithelium, which filled much of the interconnected cystic cavities. At the side of one such space, the epithelium transitioned gradually to become low columnar in type, two cell layers thick, and having focal villous projections with decapitation type secretion (Fig, 2), A dense plasmacytic infiltrate was present in the stroma of this portion ofthe tumor. Perilesional vascularity was not increased. Neither was there evidence of lesional impingement on cutaneous nerves. Sections ofthe subsequent reexcision specimen did not reveal any residual tumor. There was, however, an easily identifiable focus of dilated ectopic apocrine glandular elements immediately adjacent to the cicatrix from the earlier biopsy. Neither specimen displayed any features of an adjacent epidermal nevus or nevus sebaceus, A variant of hidrocystoma was considered in the microscopic differential diagnosis. Due to the unusual clinical history, this case was independently reviewed hy two dermatopathologists who concur with our diagnosis of syringocystadenoma papiliiferum.
Figure 2, Close-up of one side of the cyst showing the epithelium transitioning to low columnar, with focal villous projections (H & E, original magnification XlOO),
Discussion The head and neck are the most common locations for a syringocystadenoma papiliiferum, Ofthe 145 cases reported 8 (5%) have been on the extremities. Seven ofthe 8 extremity lesions have been on the lower limbs (5-thigh, 2-toe).'"' The one previous upper extremity tumor was on the upper arm,^ Our patient represents the second
A cknowledgment
report in the English literature in which a syringocystadenoma papiliiferum was located on an upper extremity, and the first case occurring on the hand. This tumor has usually been described as primarily asymptomatic, although patients with scalp lesions may complain of mild irritation or bleeding when combing their hair. One case was reported as painful, but this was after the lesion recurred after excision,'' Our patient is unique in that her tumor was exquisitely tender, even to light palpation. The cause of this is unclear. One could conjecture that the neoplasm may have been encompassing or applying pressure on a nerve, although obvious neural involvement was not observed histologically. See also page 763,
Thomas G, Olsen, M,D, (Dermatopathology Lahoratory of Central States), and J, Michael Gagnier, M,D, (Armed Forces Institute of Pathology), reviewed this case. Jere J, Mammino, D,O. Dennis A, Vidmar, C,D,R,, M,C,, U,S,N, Greensboro, North Carolina
Figure 1. Close-up of syringocystadenoma papiliiferum on the dorsum ofthe right hand.
References 1, Stokes JH, A clinico-pathologic study of an unusual cutaneous neoplasm combining naevus syringadenomatosus papilliferus and a granuloma, J Cutan Dis, 1917;35:411-419, 2, Helwig EB, Hackney VC, Syringadenoma papiliiferum, AMA Arch Dermatol. 1955;71:361-372, 3, Pinkus H, Life history of naevus syringadenomatosus papilliferus. Arch Derm Syphilol, 1954;69:305-322, 4, Steinhart AN, Combined papillary syringocystadenoma and hidradenoma, J Am Podiatr Assoc, 1977;67:659-660, 5, Vanatta PR, Bangert JL, Freeman RG, Syringocystadenoma papiliiferum: A plasmacytotropic tumor. Am J Surg Pathol. 1985;9:678-683, 6, Rostan SE, Waller JD, Syringocystadenoma papiliiferum in an unusual location. Arch Dermatol, 1976;112:835-836,
International Journal of Dermatology • November 1991
Vol. 30
Syringocystadenoma Papiliiferum: Report of an Unusual Case To the Editor: A 59-year-old white woman presented to our clinic with a slowly growing papule on the dorsum of her right hand present for 6 months. She desired removal hecause she experienced a sharp pain in the lesion with minimal manipulation. Examination revealed a 6-mm smooth, dome shaped, erythematous, firm papule on the proximal portion of the dorsal right hand (Fig, 1). In addition, there was a 2-mm eccentrically located dilated pore with a visible keratinous plug. Gentle palpation of this lesion caused a sharp pain, discomforting enough to cause the patient to wince, A deep shave biopsy was performed, which histologically revealed a multiloculated cystic tumor confined to the upper dermis, communicating to the surface via a wide mouthed ostium. Most of the cystic fining was keratinizing stratified squamous epithelium, which filled much of the interconnected cystic cavities. At the side of one such space, the epithelium transitioned gradually to become low columnar in type, two cell layers thick, and having focal villous projections with decapitation type secretion (Fig, 2), A dense plasmacytic infiltrate was present in the stroma of this portion ofthe tumor. Perilesional vascularity was not increased. Neither was there evidence of lesional impingement on cutaneous nerves. Sections ofthe subsequent reexcision specimen did not reveal any residual tumor. There was, however, an easily identifiable focus of dilated ectopic apocrine glandular elements immediately adjacent to the cicatrix from the earlier biopsy. Neither specimen displayed any features of an adjacent epidermal nevus or nevus sebaceus, A variant of hidrocystoma was considered in the microscopic differential diagnosis. Due to the unusual clinical history, this case was independently reviewed hy two dermatopathologists who concur with our diagnosis of syringocystadenoma papiliiferum.
Figure 2, Close-up of one side of the cyst showing the epithelium transitioning to low columnar, with focal villous projections (H & E, original magnification XlOO),
Discussion The head and neck are the most common locations for a syringocystadenoma papiliiferum, Ofthe 145 cases reported 8 (5%) have been on the extremities. Seven ofthe 8 extremity lesions have been on the lower limbs (5-thigh, 2-toe).'"' The one previous upper extremity tumor was on the upper arm,^ Our patient represents the second
A cknowledgment
report in the English literature in which a syringocystadenoma papiliiferum was located on an upper extremity, and the first case occurring on the hand. This tumor has usually been described as primarily asymptomatic, although patients with scalp lesions may complain of mild irritation or bleeding when combing their hair. One case was reported as painful, but this was after the lesion recurred after excision,'' Our patient is unique in that her tumor was exquisitely tender, even to light palpation. The cause of this is unclear. One could conjecture that the neoplasm may have been encompassing or applying pressure on a nerve, although obvious neural involvement was not observed histologically. See also page 763,
Thomas G, Olsen, M,D, (Dermatopathology Lahoratory of Central States), and J, Michael Gagnier, M,D, (Armed Forces Institute of Pathology), reviewed this case. Jere J, Mammino, D,O. Dennis A, Vidmar, C,D,R,, M,C,, U,S,N, Greensboro, North Carolina
Figure 1. Close-up of syringocystadenoma papiliiferum on the dorsum ofthe right hand.
References 1, Stokes JH, A clinico-pathologic study of an unusual cutaneous neoplasm combining naevus syringadenomatosus papilliferus and a granuloma, J Cutan Dis, 1917;35:411-419, 2, Helwig EB, Hackney VC, Syringadenoma papiliiferum, AMA Arch Dermatol. 1955;71:361-372, 3, Pinkus H, Life history of naevus syringadenomatosus papilliferus. Arch Derm Syphilol, 1954;69:305-322, 4, Steinhart AN, Combined papillary syringocystadenoma and hidradenoma, J Am Podiatr Assoc, 1977;67:659-660, 5, Vanatta PR, Bangert JL, Freeman RG, Syringocystadenoma papiliiferum: A plasmacytotropic tumor. Am J Surg Pathol. 1985;9:678-683, 6, Rostan SE, Waller JD, Syringocystadenoma papiliiferum in an unusual location. Arch Dermatol, 1976;112:835-836,
