CASE #2: Necrobiosis lipoidica
In 1932, Urbach coined the term necrobiosis lipoidica diabeticorum to name the particular kind of plaques seen on the legs of diabetics. In 1935, Goldsmith was the first to note the appearance of the condition in individuals without diabetes.14 Twenty-five years later, Rollins and Winkleman analyzed a number of cases of this disease and again noted its presence in nondiabetics, leading them to suggest changing the name of the condition to necrobiosis lipoidica (NL), a term encompassing all cases of this clinical condition regardless of the presence or absence of diabetes.15
NL represents a disorder of collagen degeneration with a granulomatous response, eventuating clinically in the formation of shiny brown plaques that tend to enlarge slowly, becoming increasingly yellow in color with atrophic surfaces marked by telangiectasias on the periphery.14 Occurring mostly on the leg, the plaques average approximately 1 cm but can grow to many times that size. Multiple lesions are common, and relatively minor trauma to these lesions can result in erosions and ulcers that are difficult to treat.
As noted above, NL can occur in people without diabetes, but up to 60% of NL patients have been diagnosed with diabetes prior to the development of the plaques.14 Both diagnoses are made at the same time in 25% of cases, but the presence or progression of NL does not correlate with the severity of diabetes.14
The average age of development of NL is 30 years, but the range of ages varies a great deal. Women with NL outnumber men by at least 3:1.14
The clinical picture displayed by NL is clear enough to be essentially pathognomic, although biopsy is sometimes required to differentiate NL from such conditions as morphea, sarcoidosis, granuloma annulare, and xanthomata.14,16
Microscopically, NL demonstrates interstitial and palisaded granulomas in subcutaneous tissue as well as the dermis.14 In addition, blood vessel walls are thickened, and endothelial cells are swollen in the middle to deep dermis. In some cases, clinical correlation with microscopic findings is invaluable, especially with regard to possible granuloma annulare, NL’s closest look-alike.
Fortunately, this patient’s lesion and its clinical context were quite typical, permitting an empirical diagnosis and the initiation of treatment with topical steroid cream (fluocinonide 0.05%) b.i.d. Steroid creams can be useful in treating active, enlarging lesions but can be counterproductive in older, “burnt-out” lesions. Because the cause of the condition is unknown, treatment of NL can be problematic.
Arguably, the formation of traumatic ulcers in these lesions is the source of much difficulty.14 The prevention of trauma, with pressure stockings, and an assessment of the patient’s living environment (for potential trauma from wheelchair parts or furniture, for example) are in order.
Many different treatment modalities have been used to treat NL with varying degrees of success. These include pentoxifylline (Pentopak, Pentoxil, Trental),17 photodynamic therapy,18 aspirin/dipyridamole (Permole, Persantine) combination,19 calcineurin inhibitors (e.g., tacrolimus [Hecoria, Prograf]),14 perilesional heparin injections,20 oral vitamin B, and tretinoin (Vesanoid),21 to name just a few.
Joe Monroe, PA-C, is a physician assistant specializing in dermatology at Dawkins Dermatology in Oklahoma City.
1. James WD, Berger TG, Elston DM. “Dermatologic surgery.” In: Andrews’ Diseases of the Skin: Clinical Dermatology. 10th ed. Philadelphia, Pa.: Saunders-Elsevier; 2006:876.
2. Brownell I. “Nodular basal cell carcinoma.” J Drugs Dermatol. 2007;6:1245-1246.
3. Mendenhall WM, Amdur RJ, Hinerman RW et al. “Radiotherapy for cutaneous squamous and basal cell carcinomas of the head and neck.” Laryngoscope. 2009;119:1994-1999.
4. Geisse JK, Rich P, Pandya A et al. “Imiquimod 5% cream for the treatment of superficial basal cell carcinoma: a double-blind, randomized, vehicle-controlled study.” J Am Acad Dermatol. 2002;47:390-398.
5. Efudex (fluorouracil) [package insert]. Valeant Pharmaceuticals; 2005.
7. Cabrera HN, Gómez ML. “Skin cancer induced by arsenic in the water.” J Cutan Med Surg. 2003;7:106-111.
8. Romão-Corrêa RF, Maria DA, Soma M et al. “Nucleolar organizer region staining patterns in paraffin-embedded tissue cells from human skin cancers. ” J Cutan Pathol. 2005;32:323-328.
9. Keyhani K, Ashenhurst M, Oryschak A. “Periocular basal cell carcinoma arising in a site of previous trauma.” Can J Ophthalmol. 2007;42:467-468.
10. Erba P, Farhadi J, Wettstein R et al. “Morphoeic basal cell carcinoma of the face.” Scand J Plast Reconstr Surg Hand Surg. 2007;41:184-188.
11. Fresini A, Rossiello L, Severino BU et al. “Giant basal cell carcinoma.” Skinmed. 2007;6:204-205.
12. Shindel AW, Mann MW, Lev RY et al. “Mohs micrographic surgery for penile cancer: management and long-term followup.” J Urol. 2007; 178:1980-1985.
13. Mulvany NJ, Allen DG. “Differentiated intraepithelial neoplasia of the vulva.” Int J Gynecol Pathol. 2008;27:125-135.
15. Rollins TG, Winkelmann RK. “Necrobiosis lipoidica granulomatosis. Necrobiosis lipoidica diabeticorum in the nondiabetic.” Arch Dermatol. 1960;82:537-543.
16. Mistry N, Chih-Ho Hong H, Crawford RI. “Pretibial angioplasia: a novel entity encompassing the clinical features of necrobiosis lipoidica and the histopathology of venous insufficiency.” J Cutan Med Surg. 2011; 15:15-20.
17. Littler CM, Tschen EH. “Pentoxifylline for necrobiosis lipoidica diabeticorum.” J Am Acad Dermatol. 1987;17(2 Pt 1):314-316.
18. Heidenheim M, Jemec GB. “Successful treatment of necrobiosis lipoidica diabeticorum with photodynamic therapy.” Arch Dermatol. 2006;142:1548-1550.
19. Eldor A, Diaz EG, Naparstek E. “Treatment of diabetic necrobiosis with aspirin and dipyridamole.” N Engl J Med. 1978;298:1033.
20. Jetton RL, Lazarus GS. “Minidose heparin therapy for vasculitis of atrophie blanche.” J Am Acad Dermatol. 1983;8:23-26.
21. Heymann WR. “Necrobiosis lipoidica treated with topical tretinoin.” Cutis. 1996;58:53-54.
All electronic documents accessed May 15, 2012.