What Is the Peculiar Papular Rash on This Adolescent’s Torso?
A 17-year-old Latino adolescent presented with a rash on his back that had been present for 2 years. The rash recently had spread to his chest.
No treatments had ever been tried. The rash was nonpruritic and did not seem to bother the patient. He had a history of Down syndrome and hypothyroidism, for which he took daily levothyroxine. He took no other medications. His family history was positive for glaucoma in his mother.
On physical examination, diffuse cystic, smooth, well-defined papules spanned across the teen’s entire back and chest, with a few small pustules interspersed. No erythema or excoriation of the surrounding skin was seen. On the back, the lesions were distributed evenly and were uniform in size between 2 and 4 mm. No puncta were present. On the chest, the lesions were clustered at the sternal area.
What is this peculiar papular rash?
(Answer and discussion on next page)
Answer: Steatocystoma Multiplex
A clinical diagnosis of steatocystoma multiplex was made based on the adolescent’s physical examination findings. Treatment was not required, since the condition does not pose a health threat, and the patient was asymptomatic. The patient and family were given reassurance that this is a benign condition.
Steatocystoma multiplex is a rare genetic disorder with an autosomal dominant pattern of inheritance, although sporadic cases have been reported.1,2 It usually presents in adolescence. The condition is a hamartomatous malformation of the pilosebaceous duct junction, and its presence is characterized by numerous small, skin-colored or yellowish, cutaneous cysts.2 The lesions typically are located on the upper trunk, neck, axillae, scrotum, and proximal extremities. In some cases, scattered lesions have occurred on the scalp or face, but rarely are lesions limited to the scalp.3
The clinical differential diagnosis of steatocystoma multiplex includes multiple epidermoid cysts, eruptive vellus hair cysts, neurofibromatosis, lipomas, and cerebrotendinous xanthomatosis. Lesions can be confused with milia, epidermal inclusion cysts, sebaceous cysts, dermoid cysts, and the cysts of acne vulgaris.4
Treatment is strictly for cosmetic purposes and can include surgery, carbon dioxide laser therapy, oral isotretinoin, and cryotherapy.
Lisa Gwynn, DO, MBA, is the medical director of the Pediatric Mobile Clinic, assistant clinical professor of pediatrics, and Director of Innovation and Community Engagement at the Mailman Center for Child Development at the University of Miami Miller School of Medicine in Miami, Florida.
Anne E. Burdick, MD, MPH, is the associate dean for Telehealth and Clinical Outreach, professor of dermatology, and the Leprosy Program Director at the University of Miami Miller School of Medicine in Miami, Florida.
References
- Kamra HT, Gadgil PA, Ovhal AG, Narkhede RR. Steatocystoma multiplex-a rare genetic disorder: a case report and review of the literature. J Clin Diagn Res. 2013;7(1):166-168.
- Naik NS. Steatocystoma multiplex. Dermatol Online J. 2000;6(1):10.
- Kim SJ, Park HJ, Oh ST, Lee JY, Cho BK. A case of steatocystoma multiplex limited to the scalp. Ann Dermatol. 2009;21(1):106-109.
- Chu DH. Steatocystoma multiplex. Dermatol Online J. 2003;9(4):18.