biopsy revealed collagen degeneration and vascular changes
within the entire dermis, as well as extra-cellular lipid and giant
cells. Overall, these findings were consistent with necrobiosis lipoidica
and treatment for NL was started. The patient was treated
with a one-time intralesional injection of corticosteroid. Lesions
were significantly improved after 3 weeks. At one-year-followup,
no further lesions or recurrent plaques were observed.
DISCUSSION
Sarcoidosis is a multisystem disorder and can involve any organ of the body. Approximately 30% of systemic sarcoidosis appears with skin involvement.6 NL did not come to mind in the first differential diagnosis of the annular lesions of the face. Our case subject was undergoing treatment with an oral corticosteroid (prednisolone 15-10 mg/daily) for about 8 years because of suspected sarcoidosis, without improvement. After the diagnosis of NL and a one-time intralesional injection of corticosteroid, significant improvement was observed.The incidence of NL and sarcoidosis occurring in the same non-diabetic patient has been reported in a few studies.7 This occurrence may be incidental or due to an association between the 2 disorders with unclear pathophysiology. Chiba et al evaluated a 70-year-old woman with a 8-year systemic sarcoidosis history concomitant with NL, and stated that although necrobiosis was present, there were non-necrotizing granulomas in the dermis with histological diagnosis of cutaneous sarcoidosis.8 Mendoza et al evaluated 3 patients with confirmed diagnosis of sarcoidosis who developed NL skin lesions, reporting that the relationship of NL-like skin lesions with sarcoidosis is not widely appreciated.9 Igawa et al evaluated a 62-year-old woman with systemic sarcoidosis and lower leg erythematous plaques and reported that because cutaneous involvement of sarcoidosis may mimic NL clinically and/or histologically, her skin lesions were necrobiosis-like skin sarcoids.
NL is typically located on the lower extremities. In our case, given the atypical locations on the face and upper extremities, the diagnosis of NL was very difficult, explaining why the lesions were misdiagnosed as sarcoidosis. More studies are needed to explore the pathogenesis of granulomatous disorders and the associations between these disorders.
NL is typically located on the lower extremities. In our case, given the atypical locations on the face and upper extremities, the diagnosis of NL was very difficult, explaining why the lesions were misdiagnosed as sarcoidosis. More studies are needed to explore the pathogenesis of granulomatous disorders and the associations between these disorders.
CONCLUSION
In summary, we report a rare case of non-diabetic necrobiosis lipoidica. Our case is novel since after being misdiagnosed with cutaneous sarcoidosis for 13 years, our patient then improved with just one intralesional injection of corticosteroid after the new diagnosis of necrobiosis lipoidica.
There is a probable relationship between the occurrence of NL and sarcoidosis. They may represent different stages of the same granulomatous process linked through yet unknown patho-mechanisms, but at the same time they may be quite different diseases with an overlapping clinical and pathologic history. Diagnosis at the appropriate time can help in preventing unnecessary or incorrect treatment. NL may present with lesions localized in atypical sites, in which cases it may be misdiagnosed and managed.
There is a probable relationship between the occurrence of NL and sarcoidosis. They may represent different stages of the same granulomatous process linked through yet unknown patho-mechanisms, but at the same time they may be quite different diseases with an overlapping clinical and pathologic history. Diagnosis at the appropriate time can help in preventing unnecessary or incorrect treatment. NL may present with lesions localized in atypical sites, in which cases it may be misdiagnosed and managed.
DISCLOSURES
The authors have reported no conflicts.
REFERENCES
1. Katta R. Cutaneous sarcoidosis: a dermatologic masquerader. Am Fam Physician. 2002;65(8):1581-1588.
2. Peckruhn M, Tittelbach J, Elsner P. Update: Treatment of necrobiosis lipoidica. J Deutschen Dermatologischen Gesellschaft. 2017;15(2):151-158.
3. Schulman JM, LeBoit PE. Adipophilin expression in necrobiosis lipoidica, granuloma annulare, and sarcoidosis. Am J Dermatopathol. 2015;37(3):203- 209.
4. Patsatsi A, Kyriakou A, Sotiriadis D. Necrobiosis lipoidica: early diagnosis and treatment with tacrolimus. Case Rep Dermatol. 2011;3(1):89-93. 5. Wee E, Kelly R. Pentoxifylline: An effective therapy for necrobiosis lipoidica. Australas J Dermatol. 2017;58(1):65-68.
2. Peckruhn M, Tittelbach J, Elsner P. Update: Treatment of necrobiosis lipoidica. J Deutschen Dermatologischen Gesellschaft. 2017;15(2):151-158.
3. Schulman JM, LeBoit PE. Adipophilin expression in necrobiosis lipoidica, granuloma annulare, and sarcoidosis. Am J Dermatopathol. 2015;37(3):203- 209.
4. Patsatsi A, Kyriakou A, Sotiriadis D. Necrobiosis lipoidica: early diagnosis and treatment with tacrolimus. Case Rep Dermatol. 2011;3(1):89-93. 5. Wee E, Kelly R. Pentoxifylline: An effective therapy for necrobiosis lipoidica. Australas J Dermatol. 2017;58(1):65-68.
