Dermatology Online Journal
- Author(s): Seirafi, Hassan H
- Akhyani, Maryam
- Naraghi, Zahra S.
- Mansoori, Parisa
- Dehkordi, Hossein Shabanzadeh
- Taheri, Arash
- Ehsani, Amir H.
- Kavusi, Susan
- Farnaghi, Farshad
- et al.
1. Departments of Dermatology, Razi Hospital, Tehran University of Medical Sciences. email@example.com 2. Departments
of Pathology, Razi Hospital, Tehran University of Medical Sciences
Hassan H Seirafi1, Maryam Akhyani1, Zahra S. Naraghi2, Parisa Mansoori1, Hossein Shabanzadeh Dehkordi1, Arash Taheri1, Amir H. Ehsani1, Susan Kavusi1, Farshad Farnaghi1
Dermatology Online Journal 11 (2): 13
Syringomas are benign adnexal tumors derived from the intraepidermal portion of eccrine sweat ducts. Usually, they present as soft, flesh-colored to slightly yellow dermal papules on the lower eyelids of healthy individuals. We report an 18-year-old man with rare presentation of eruptive syringomas involving his trunk and extremities, with linear arrangement on the arms and forearms. A biopsy obtained from the lesions of the dorsum of the hands showed eccrine syringoma with a lymphocytic inflammatory infiltration around superficial blood vessels and eccrine ducts. We used the 585-nm and 595-nm pulsed dye laser for treatment of inflammatory lesions of forearm and trunk with no success.
Syringomas are benign adnexal tumors derived from the intraepidermal portion of eccrine sweat ducts. Usually, they present as soft, skin-colored to slightly yellow dermal papules on the lower eyelids of healthy individuals. Other sites of predilection are the cheeks, axillae, neck, and abdomen. They occur predominantly in women at puberty or early adulthood. Lesions of eruptive syringoma usually arise on the anterior trunk of young persons .
An 18-year-old Iranian man presented with a 2-year history of numerous, asymptomatic lesions that had begun as a few papules on the upper chest. Over the next 6-9 months, similar lesions had gradually developed on the most of his body surface. Since then, the lesions had not subsequently changed. His medical history was unremarkable. There was no family history of similar lesions. He was not on any medications.
|Figure 1||Figure 2|
|Figure 1. Erythematous papules that are surrounded by an erythematous halo, on the dorsum of the hand.|
|Figure 2. Linear arrangement of the lesions on the arm and forearm.|
Physical examination revealed multiple flesh-colored to reddish brown, flat-topped papules 1-4 mm in diameter on the neck, chest, abdomen, back, arms, forearms, dorsum of the hands and fingers, thighs, legs, and around the eyelids. The lesions of the extremities, especially those on the dorsum of the hands, were erythematous papules, surrounded by an erythematous halo (Fig. 1).
The lesions were symmetrical in all parts of the body, except for the upper extremities, where the lesions were more numerous on the left side. The lesions showed a linear arrangement on the arms and forearms (Fig. 2). Neighboring papules had become confluent in some areas (Fig. 3, Fig. 4). The palms and soles, dorsum of the feet, axillae, groins, scalp, face (excluding eyelids), oral mucous membrane, hair, and nail were spared. The remainder of physical examination was unremarkable.
|Figure 3||Figure 4|
|FIgure 3. Confluent papules on the trunk.|
|Figure 4. Confluent papules on the chest.|
Serum cholesterol, triglyceride, fasting blood glucose, alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase, hemoglobin, white blood cell count, red blood cell count, and platelets were within normal limits.
|Figure 5||Figure 6|
Biopsy specimen from the lesions of the trunk showed a normal epidermis overlying a dermis that was filled with numerous ducts and small solid epithelial nests embedded in a fibrous stroma. The ducts were lined by two layers of cuboidal epithelium. The lumina of the ducts contained amorphous debris. Some of the ducts and solid epithelial nests possessed small, comma like tails of epithelial cells, giving them the appearance of tadpoles. These histologic features are typical for syringoma. Biopsy specimen from the lesions of the dorsum of the hand showed similar findings, but some hyperkeratosis and basal layer hyperpigmentation in the epidermis and a lymphocytic infiltrate around superficial blood vessels and eccrine ducts in upper dermis.
For treatment of the lesions, we used the 585-nm and 595-nm pulsed dye laser (PDL) at fluences of 8 to 9J/cm² and spot size of 7-mm on the forearm and trunk lesions with no success.
With eruptive syringomas, the lesions usually arise in large numbers in successive crops on the anterior trunk of young persons . Unusual locations include the vulva , and penis . In rare instances, occult syringomas of the scalp are associated with diffuse thinning of the hair  or with cicatricial alopecia . Rarely, the lesions of syringoma show a unilateral, linear arrangement . Acral syringomas, located on distal extremities, are extremely rare, with only five cases reported in the dermatological literature [7, 8, 9, 10, 11]. There is a single case report of solitary syringoma of the left ankle . Symmetrical syringomas located on the distal aspects of the upper extremities have been described in four cases as an isolated finding [7, 8] or in association with typical eruption around the eyelids [9, 10]. Another case with multiple acral syringomas and symmetrical involvement of the dorsal aspect of the hands, feet, fingers and toes, unassociated with the similar involvement of her skin elsewhere has also been reported .
Our patient had diffuse involvement of most of the body surface, including acral portions, with linear and asymmetrical arrangement of the lesions on the upper extremities. In the previous reported cases of acral syringoma, there was no involvement of proximal parts of the extremities and trunk.
Guitart et al.  described two patients who presented with an eczematous eruption that resolved leaving residual lesions. Biopsies obtained from the late lesions showed eccrine syringoma. A biopsy obtained from an incipient lesion in one of the cases showed a lymphocytic inflammatory reaction surrounding the superficial portion of the eccrine ducts. They proposed that some of the so-called eruptive syringomas may represent a hyperplastic response of the eccrine ducts to an initial inflammatory reaction rather than a true adnexal neoplasm.
There are numerous reports of syringoma or syringoma-like changes observed in a variety of cutaneous conditions. For instance, cases of diffuse alopecia associated with syringomatous changes have been reported [14, 15]. Syringomatous changes noted in alopecia scalp biopsies are not necessarily associated with a scarring process and hence are probably not the result of occlusion of the ductal lumen. A case of alopecia areata has been reported with similar proliferation of the sweat ducts . Syringomatous changes have also been reported in association with prurigo nodularis  and melanocytic nevi [18, 19]. In such cases, the syringomatous proliferation may be a response to the initial inflammatory process.
Our patient had a generalized papular eruption at the time of presentation. On examination, the lesions of the extremities were erythematous papules, surrounded by an erythematous halo. Biopsy specimen revealed perivascular and perieccrine lymphocytic infiltration. We did not see the incipient lesions of our patient and we do not know if the lesions were syringoma from the outset or the incipient eruption was an inflammatory process without eccrine duct proliferation.
We do not know the pathogenesis of syringomas and the inflammatory reaction that is observed around the proliferated eccrine ducts in syringoma. Both of them may be the result of another etiology or the inflammation may be secondary to the neoplastic process in syringoma. On the other hand eruptive syringoma may be a hyperplastic response of the eccrine ducts to an inflammatory reaction caused by another etiology rather than a true adnexal neoplasm.
Many treatment modalities such as dermabrasion, electrodesiccation with curettage, scissors excision, carbon dioxide (CO2) laser , argon laser , erbium-YAG laser , and combination of TCA and CO2 laser [23,24] have been tried with some success. To our knowledge, PDL has not been tried previously for treatment of syringoma. We used it to treat our patient with no success.
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