The Free Sign in morphea and other sclerosing disorders: A clue to the presence of early sclerosis?

BACKGROUND: The Floating Sign is a histopathologic clue to the diagnosis of autoimmune sclerosing skin disorders such as morphea and interstitial granulomatous dermatitis (IGD). On the other hand, the "free-floating" sign has been associated with neoplasms, for example, dermatofibroma and interstitial mycosis fungoides. Herein, we report the Free Sign in sclerosing skin disorders. METHODS: In a case-control study, we applied detailed histopathologic definitions of Floating Sign and Free Sign to assess their presence in morphea, IGD, and other sclerosing disorders. RESULTS: Free Sign was present in most cases of morphea (46/55, 84%) and IGD (7/13, 54%) but not necrobiosis lipoidica (NL) (6/14, 42.8%) or sclerodermoid graft versus host disease (SGVHD) (2/7, 28.5%). The sensitivity and specificity of Free Sign for morphea versus other disorders was 84% and 56%, respectively. Floating Sign was not identified in most cases: NL (3/14, 21.4%), SGVHD (1/7, 14.2%), morphea (5/55, 9%), IGD (1/13, 7.7%). The diagnostic sensitivity of Floating Sign in morphea was 9%. CONCLUSIONS: The Free Sign was present in most cases of morphea in our series and may represent a clue to the presence of evolving sclerosis. Free Sign may be seen in other sclerosing disorders. Technical artifact is a potential cause of a false-positive Free Sign.

Don't Go Breaking My Heart: Not Your Ordinary Tattoo Reaction.

ABSTRACT: Necrobiosis lipoidica (NL) is a rare, chronic idiopathic granulomatous dermatitis with a somewhat controversial association with diabetes and other systemic diseases. We report a case of NL developing within a polychromic tattoo on the lower leg of a 53-year-old woman. Characteristic histopathologic findings of both active and chronic "burnt-out" NL appeared to originate from the tattoo where red ink was used 13 years prior. To the best of our knowledge, only 3 other cases of tattoo-associated NL have been reported.

Necrobiosis lipoidica of the breast associated with Crohn's disease: a case report.

BACKGROUND: Necrobiosis lipoidica located to the breast; without evidence of glucose intolerance, is extremely rare, and its association to Crohn's disease is not usual. CASE PRESENTATION: We report an interesting case of an association of necrobiosis lipoidica of the breast and Crohn's disease in a 54-year-old Moroccan woman. Skin necrobiotic changes are a characteristic feature in necrobiosis lipoidica, but they are exceptional in metastatic Crohn's disease, since there are only three published cases of necrobiotic skin lesions on the lower leg resembling erythema nodosum in metastatic Crohn's disease. CONCLUSIONS: On the basis of this rare observation, necrobiosis lipoidica without evidence of glucose intolerance should be recognized as a possible cutaneous manifestation or association of Crohn's disease.

Histopathologic features of necrobiosis lipoidica.

BACKGROUND: Necrobiosis lipoidica (NL) is an uncommon granulomatous dermatosis that can occur in patients with or without associated diabetes mellitus (DM). Prior studies have attempted to determine distinctive histopathologic features of NL in patients with and without DM. METHODS: A retrospective review of 97 patients with NL was performed to determine the similar and distinctive histopathologic features in patients with DM and without DM. RESULTS: Of the 97 patients, 32% (n = 31) had DM. Epidermal acanthosis was seen more commonly in diabetics than nondiabetics (32.3% vs. 12.1%; p = 0.017). Naked (sarcoidal/tuberculoid) granulomas were more frequently observed in nondiabetics than diabetics (22.7% vs. 3.2%; p = 0.016). Eosinophils were more common in nondiabetics than diabetics (38.5% vs. 9.7%; p = 0.004), while neutrophilic infiltration was more common in diabetics than nondiabetics (45.2% vs. 17.5%; p = 0.004). CONCLUSIONS: This study corroborates well-documented histopathologic features of NL and shows distinctive histopathologic features of NL among patients with DM-I, DM-II, and without DM. These results support the hypothesis that there are different underlying drivers of NL between diabetics and nondiabetics.

Ulcerative necrobiosis lipoidica: case report of an atypical presentation and literature review.

Necrobiosis lipoidica (NL) is a rare chronic granulomatous degenerative skin disease by unknown causes, which is mostly associated with diabetes mellitus, usually presenting with typical plaques of the shins. Although less common, some ulcerative forms may be seen in clinical practice. The occurrence of an atypical presentation in one of our patients was the occasion to review the pertinent literature.

Pediatric necrobiosis lipoidica: case report and review of the literature.

Necrobiosis lipoidica (NL) is a rare, granulomatous disease considered to be associated with diabetes. It is frequently seen in female and middle-aged patients and is rarely observed in children. We present a 14-year-old boy with poorly controlled type 1 diabetes who developed biopsy-proven NL. He had improvement, but not resolution of the plaque with improved glycemic control. Pediatric NL may be associated with diabetes and could be related to poor glycemic control. However, further investigation is warranted in this young population.

Cutaneous Manifestations of Diabetes.

Diabetes mellitus is a significant worldwide health concern and cutaneous manifestations are common. This review describes characteristic skin findings of diabetes, general skin findings related to diabetes, and findings related to diabetes treatment with a focus on clinical presentation, diagnosis, pathophysiology, epidemiology, and treatment. As the prevalence of diabetes continues to rise, cutaneous manifestations of diabetes mellitus likely will be encountered more frequently by physicians in all disciplines including dermatologists and primary care physicians. Accordingly, knowledge regarding the prevention, diagnosis, and management of cutaneous manifestations is an important aspect in the care of patients with diabetes.

Necrobiosis Lipoidica Mimicking Cutaneous Sarcoidosis Finally Treated With an Intralesional Injection of Corticosteroid: A Case Report

BACKGROUND: Necrobiosis lipoidica (NL) and sarcoidosis are granulomatous disorders with an unknown pathogenesis. They may coexist in the same patient, which suggests a possible overlap between these diseases among shared granulomatous inflammatory pathways. Case Presentation: This study presents the case of a non-diabetic 52-year-old woman who presented with red-yellowish border plaques on the face and upper extremities previously diagnosed as sarcoidosis. After 13 years of inappropriate treatment, histopathological findings consistent with the clinical and para-clinical examination suggested the diagnosis of NL. After treatment with an intralesional injection of steroids, significant improvement was observed, and no recurrent lesions were found. CONCLUSION: Necrobiosis lipoidica may mimic cutaneous sarcoidosis. Prompt recognition and treatment of NL can be helpful for managing the disease. J Drugs Dermatol. 2020;19(1):92-94. doi:10.36849/JDD.2020.4675

[Rare skin condition in a child with diabetes mellitus]. / Zeldzame huidaandoening bij een kind met diabetes mellitus.

BACKGROUND: Necrobiosis lipoidica is a painless but disfiguring skin condition regularly complicated by ulceration and that is related to diabetes mellitus. CASE DESCRIPTION: We present the case of a 17-year-old girl with type 1 diabetes mellitus in whom necrobiosis lipoidica was diagnosed. After falling with a bicycle, a traumatic ulcer developed. The patient underwent several different treatments (local and systemic glucocorticoids, antibiotics, hydroxychloroquine, calcineurin inhibitor, laser therapy and surgery), but these ultimately resulted in no more than minimal improvement of the skin condition. CONCLUSION: Necrobiosis lipoidica is difficult to treat. Treatment recommendations are based on patient descriptions and results of small clinical trials.

Non-infectious granulomatous dermatoses.

Granulomatous dermatoses comprise a wide range of etiologically and clinically distinct skin diseases that share a common histology characterized by the accumulation of histiocytes include macrophages. While the pathogenesis of these disorders is not fully understood, the underlying mechanism is thought to involve a reaction pattern caused by an immunogenic stimulus. Antigen-presenting cells and the effect of various cytokines play a key role. Our understanding of granulomatous reaction patterns has been advanced by insights drawn from observations of such reactions in patients on immunomodulatory therapy and in individuals with genetic immunodeficiency. Traditionally, a distinction is made between infectious and non-infectious granulomatous dermatoses. The present CME article addresses granulomatous skin diseases for which there is no evidence of a causative infectious agent. Common representatives include granuloma annulare, necrobiosis lipoidica and cutaneous sarcoidosis. Granulomatous dermatoses may be part of the clinical spectrum of various systemic disorders or may be associated therewith. Some neoplastic disorders may mimic granulomatous dermatoses histologically. Given the pathogenetic diversity involved, the clinical presentation, too, is quite varied. Overall, however, each disorder is characterized by typical clinical features. The diagnosis always requires thorough clinicopathologic correlation. Treatment is preferably based on the underlying pathogenesis and frequently involves anti-inflammatory agents. In most cases, however, there is insufficient study data. The dermal nature of these disorders frequently poses a therapeutic challenge, especially with respect to topical treatment.

Necrobiosis lipoidica with superimposed pyoderma vegetans.

Necrobiosis lipoidica (NL) is a granulomatous inflammatory skin disease strongly associated with diabetes mellitus (DM). Red-brown papules expanding into plaques with erythematous indurated borders on the lower extremities are characteristic of NL. Diagnosis is made clinically; however, biopsy of lesions confirms the diagnosis. Untreated NL may ulcerate and lead to further complications, but progression to superimposed pyoderma vegetans (PV) is not a known occurrence.