SkIndia Quiz 28: Sudden onset generalized papules in a pubertal female.

A 15-year-old female presented to us with the complaints of sudden appearance of numerous small nonpruritic eruptions all over the body. To start with, the lesions appeared on the eyelids at the age of 7 years. At pubertal age, this was followed by successive eruptions on the upper extremities, axillae, neck and upper abdomen, respectively [Figures 1a, 2 and 3]. The lesions rapidly increased in number over the past two months. The patient did not report any other medical problems or use of over-the-counter medications. There was no spontaneous resolution of any of the lesions during the period of observation. Her mother had similar lesions, over the eyelids and face [Figure 1b] for as long as she could remember, but these were confined to these areas and had not progressed. The girl's maternal uncle also had a history of similar lesions in his adolescence, but these had resolved spontaneously without any intervention. Cutaneous examination revealed numerous small skin-colored to brown flat-topped papules about 1-2 mm in diameter scattered over the face, trunk, back, abdomen and extremities. These were symmetrical in distribution involving both follicular and nonfollicular areas. No puncta or significant surface changes were noted and Darier's sign was negative. Physical examination of the rest of the body did not reveal any other significant abnormality. A punch biopsy was done from the right forearm, which showed a normal epidermis along with dermis, which had numerous tubular structures, embedded in dense collagen stroma. The ducts were lined by two rows of epithelial cells, some of which had comma-like tails. Ductal lumina were filled with an amorphous material [Figures 4 and 5].

Figure 1

Skin-colored to brown flat-topped papules over the face of the patient (a) and her mother (b)

Figure 2

Bilaterally symmetrical distribution of flat-topped papules over the extremities

Figure 3

Brown-colored papules over the front and bank of abdomen

Figure 4

Photomicrograph showing a normal epidermis. Dermis has numerous tubular structures, embedded in dense collagen stroma (H and E, ×10)

Figure 5

Photomicrograph showing ducts lined by two rows of epithelial cells, some of them having comma-like tails. Ductal lumina were filled with an amorphous material (H and E, ×40)

ANSWER

Familial generalized eruptive syringoma.

DISCUSSION

Syringomas, benign adnexal tumors of the intraepithelial portion of the eccrine sweat glands are common lesions found mainly in women.[1] They were first reported by Darier and Jacquet in 1887. Although they are purely benign in nature and there is no other significant systemic involvement, they are indeed a cosmetic nuisance. Friedman and Butler classified them into four types, a localized type, a familial type, those associated with Down's syndrome, and a generalized type, including generalized eruptive syringoma.[2] It is seen that the eruptive variant is mainly found in pubertal females,[3] consistent with our case. Some syringomas are supposed to be a response to an inflammatory trigger. A report illustrated an atypical case of bilaterally symmetrical papular pruritic eruptive syringomas against a background of hyperkeratotic eczema.[4]

Clinically syringomas have been mistaken for acne vulgaris, sebaceous hyperplasia, milia, lichen planus, eruptive xanthoma etc., Apart from their appearance, they do not usually pose any problem because they are asymptomatic, with an occasional patient presenting with mild pruritus. The lesions are benign and may spontaneously resolve, or, more commonly, remain stable. The findings in our patient who presented to us with widespread involvement, her mother with stable lesions over the face, and her uncle with spontaneous resolution supports the description given by Teixeira et al. in 2005 that the fates of syringoma lesions could be diverse.[2]

Treatment of syringomas is purely for cosmetic reasons and thus they must be differentiated correctly from the above conditions. This is simplified by the fact that syringomas have a distinct histopathological picture, with ducts being lined by two rows of epithelial cells and some ducts having projections resembling comma-like tails.[56] Generalized eruptive syringomas also share the same histopathological features, as was also seen in the case of the 15-year-old who presented to us. The main problem lies in the fact that these, due to their widespread involvement, are cosmetically unacceptable. Treatment is mainly from a surgical approach and consists of dermabrasion, excision, cryotherapy, electrodessication and laser.[7] Among lasers, most of the literature suggests the use of carbon dioxide laser.[8] However, none of the methods eliminates the risk of recurrence and therefore, treatment of syringoma is quite disappointing. Moreover, all surgical interventions result in scarring. The search goes on for a perfect solution for these cosmetic nuisances.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.