A 34-year-old woman with no personal or family history of interest consulted for multiple asymptomatic lesions on the trunk. The lesions had appeared gradually during the previous year.
Physical ExaminationPhysical examination revealed nondesquamative brownish papules measuring 3 to 5 mm in diameter located mainly on the abdomen and also on the thorax, axillas, and pelvis. The lesions were negative for the Darier sign (Fig. 1).
Additional TestsDermoscopy revealed a homogeneous clear brown area with a delicate pigment network (Fig. 2A).
High-frequency ultrasound (18 MHz, MyLab 25Gold, Esaote) revealed well-defined oval lesions in the superficial dermis. These were slightly hypoechogenic with respect to the adjacent dermis and heterogeneous in terms of content (Fig. 2B). Color Doppler did not reveal blood flow in the lesions.
Histology of a punch biopsy specimen of 1 of the lesions revealed scattered ductal structures in the superficial reticular dermis accompanied by slight interstitial mucin deposition and mild sclerosis (Fig. 3).
[[?]]What is your diagnosis?
DiagnosisGeneralized eruptive syringoma.
Course and TreatmentThe patient was informed about various therapeutic approaches. The initial option was topical tretinoin 0.05%, although little improvement was observed at 6 months. Subsequent alternatives were rejected by the patient.
CommentSyringomas are benign adnexal tumors that originate in the eccrine ducts. They are more common in women and are usually found in clusters on the eyelids. The eruptive variant is rare and normally appears in childhood or puberty.
There are 4 clinical variants of syringoma: localized, generalized, associated with Down syndrome, and familial. The generalized variant comprises 2 clinical forms: multifocal and eruptive. The latter was described by Jacquet and Darier as an eruptive episode of papules on the neck, trunk, axillas, and abdomen. Syringomas generally first appear in childhood and extend over 2-3 years, before becoming persistent. They consist of multiple papules that are usually brown in color and asymptomatic, although in some cases they may prove to be pruriginous.1
There are 3 subtypes of generalized eruptive syringomas: lichen planus–like, urticaria pigmentosa–like, and miliary cyst–like. The differential diagnosis should be mainly with these entities. When they affect the thorax and abdomen, the lesions are usually limited to the anterior surface, with involvement of the back and lower back being exceptional. This characteristic may be of some help in the differential diagnosis of multiple syringomas and urticaria pigmentosa or lichen planus.2
Diagnosis is usually based on histopathology. The literature contains only 3 dermatoscopic descriptions of syringoma, whose image varies depending on the subtype.3–5 Furthermore, ultrasound findings have not previously been reported for this lesion.
There have been reports of isolated cases of syringoma associated with diabetes mellitus and tumor syndromes such as Brooke-Spiegler syndrome, Nicolau-Balus syndrome, and Costello syndrome.1 Incel Uysal et al.6 recently reported a case of adult-onset eruptive syringoma associated with bilateral renal carcinoma.
Treatment is usually ineffective, and there is a risk of recurrence and scarring. Destructive methods have also been used. These include CO2 laser, dermabrasion, chemical peels, electrocautery, surgical removal, and topical treatment (retinoids and atropine). CO2 laser is probably the most effective treatment available today, and its adverse effects are acceptable.1
In conclusion, we present an uncommon case of adult-onset generalized eruptive syringoma located on the abdomen, thorax, axillas, and pelvis. Given the late age of onset and low degree of clinical suspicion, diagnosis was based on histopathology. We also provide the first description of the ultrasound characteristics of this tumor, although we found no specific defining characteristics that would enable us to distinguish this tumor from other soft tissue tumors. Finally, we wish to highlight the importance of including papular skin conditions at any age in the differential diagnosis of generalized eruptive syringoma.
Conflicts of InterestThe authors declare that they have no conflicts of interest.
Please cite this article as: Quintana-Codina M, Fernández-Figueras MT, Salleras Redonnet M. Múltiples pápulas parduzcas asintomáticas de reciente aparición en el tronco. Actas Dermosifiliogr. 2020;111:157–158.