Dermatology Quiz and Case Discussion
From The Child's Doctor, Fall 2012
- Sarah L. Chamlin, MD
- Attending Physician, Dermatology, Ann & Robert H. Lurie Children's Hospital of Chicago; Associate Professor of Pediatrics and Dermatology, Northwestern University Feinberg School of Medicine
- Disclosure: Dr. Chamlin has no industry relationships to disclose and does not refer
to products that are still investigational or not labeled for the use
- Anita N. Swamy, MD
- Medical Director and Program Co-director, Chicago Children’s Diabetes Center at La Rabida Children’s Hospital; Attending Physician, Endocrinology, Ann & Robert H. Lurie Children's Hospital of Chicago; Instructor in Pediatrics, Northwestern University Feinberg School of Medicine
- Disclosure: Dr. Swamy has received honoraria as a speaker for
Novo Nordisk, Endo Pharmaceuticals, Sanofi Aventis, and Teva
Pharmaceutical Industries Ltd. She does not refer to products that
are still investigational or not labeled for the use in discussion.
Other Disclosure Information
At the conclusion of this activity, participants will be able to:
- Recognize the disorder described in the vignette and shown in the photograph
- Describe the clinical features and causes of the condition
- Discuss the management approaches
1. What is the most likely diagnosis?
a. Atopic dermatitis
b. Discoid lupus erythematosus
c. Nummular eczema
d. Necrobiosis lipoidica diabeticorum
2. The presence of this cutaneous finding suggests which of the following?
a. Higher risk of diabetic nephropathy
b. Higher risk of diabetic retinopathy
c. Poorly controlled diabetes
d. All of the above
Answers: 1d, 2d
Necrobiosis lipoidica diabeticorum (NLD) is a rare dermatologic complication of diabetes that occurs in 0.3% to 1.2% of all patients with type 1 diabetes mellitus (DM) and less commonly in type 2 DM. This is more common in females with an average age at presentation of approximately 30 years. Its occurrence in children with type 1 or type 2 DM is rare with few case reports and series present in the literature.
The etiology of NLD is unknown and may be independent of glycemic control. This is controversial, as it seems that tight glycemic control might prevent NLD or even improve skin lesions when present. The pathogenesis may include an immunologic reaction to an unknown stimulus, such as an exaggerated response to trauma and inflammation in tissue with diffuse capillary disease. Others suggest a diffuse microangiopathy that plays a role in the necrobiosis of collagen. In keeping with the microangiopathy seen in type 1 DM, NLD may be associated with the presence or future development of microvascular complications of diabetes, such as retinopathy, nephropathy, and neuropathy. Patients with NLD should be considered at high risk for these complications and monitored accordingly.
NLD appears as sharply defined plaques with a shiny atrophic surface and erythematous borders, most often on the lower extremities. The center often becomes telangiectatic and yellow as lipids are deposited. Although the skin lesions are usually asymptomatic, they can ulcerate and pose a challenge to heal. The diagnosis is clinical and a biopsy is rarely needed. The differential diagnosis includes granuloma annulare, sarcoidosis, and amyloidosis. In some cases the skin lesions of NLD precede the diagnosis of diabetes.
Treatment of NLD is challenging and first-line therapy often includes topical or intralesional corticosteroids. Other therapy reported includes aspirin, dipyridamole, pentoxifylline, systemic corticosteroids, chloroquine, topical tretinoin, hyperbaric oxygen and topical psoralen plus ultraviolet A therapy (PUVA ). Improved glycemic control is suggested as well. Avoidance of trauma is warranted due to ulceration risk, and some patients use cosmetic cover-ups to mask the appearance. Pulsed-dye laser can be used to improve the appearance of the surface telangiectasia.
For Further Reading
[1.] Davison JE, Davies A, Moss C, et al. Links between granuloma annulare, necrobiosis lipoidica diabeticorum and childhood diabetes: a matter of time? Pediatr Dermatol 2010;27:178-181.
[2.] Marchetti F, Gerarduzzi T, Longo F, et al. Maturity-onset diabetes of the young with necrobiosis lipoidica and granuloma annulare. Pediatr Dermatol 2006;23:247-250.
[3.] Paller AS, Mancini AJ. Endocrine disorders and the skin. In: Bonnett C, Gabbedy R, Mortimer A, eds. Hurwitz Clinical Pediatric Dermatology: A Textbook of Skin Disorders of Childhood and Adolescence. Edinburgh: Elsevier Saunders; 2011:540-542.
[4.] Scaramuzza A, Maddalena M, Tadini GL, et al. Necrobiosis lipoidica diabeticorum. Case Rep Pediatr 2012;152602:1-3.
[5.] Verrotti A, Chiarelli F, Amerio P, Morgese G. Necrobiosis lipoidica diabeticorum in children and adolescents: a clue for underlying renal and retinal disease. Pediatr Dermatol 1995;12:220-223.