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PURPOSE: To report the clinical and histopathologic features of necrobiosis lipoidica and associated retinal vasculitis and describe successful treatment of both skin and eye manifestations with adalimumab, a tumor necrosis factor (TNF)-alpha inhibitor. OBSERVATIONS: A 35-year-old patient with bipolar disorder and ocular hypertension was referred for evaluation of bilateral retinal vasculitis. Fluorescein angiography revealed bilateral occlusive retinal vasculitis. Physical exam was notable for multiple annular and round erythematous hyperpigmented and atrophic patches and plaques on both lower extremities. Skin biopsy revealed a diagnosis of necrobiosis lipoidica, a rare granulomatous skin disease. Both the patients retinal vasculitis and skin patches responded favorably to treatment with adalimumab, a TNF-alpha inhibitor. CONCLUSIONS AND IMPORTANCE: This case highlights the importance of obtaining a complete history and physical exam in patients who present with ocular inflammation, as extraocular manifestations of disease may be present. It also demonstrates the effectiveness of a multidisciplinary approach to evaluation and management of these patients, as both skin and eye involvement were successfully treated with adalimumab, a TNF-alpha inhibitor.

Objective: Granulomatous dermatitis (GD) is a group of diseases grouped under two main headings: infectious and non-infectious, characterized histopathologically by granulomas and may also present with systemic findings. Granulomatous inflammation may be limited to the skin only or may occur as a symptom of a systemic disease. Material and Methods: 105 patients included who were clinically and histopathologically diagnosed with non-infectious GD in our clinic between 2006 and 2023. Results: Of the 105 patients included in our study, 79 were female and 26 were male. The mean age of the patients was 45.72 (age range 2-77). While 12 of the patients were under the age of 18, 12 patients were over the age of 65. The diagnosis of the patients were granuloma annulare (n=43), followed by sarcoidosis (n=24), necrobiosis lipoidica (n=12), interstitial GD (n=6), granulomatous rosacea (n=5), panniculitis (n=5), granuloma fasciale (n=2), Crohn’s disease (n=2), granulomatous vasculitis (n=2), piyoderma gangrenozum (n=1), post herpetic GD (n=1), lupus miliaris disseminatus faciei (n=1), granulomatous mycosis fungoides (n=1) and elastolytic giant cell granuloma (n=1). Conclusion: GDs are a heterogeneous group of diseases with varying clinical and histopathological findings. Histopathological morphology alone is rarely specific and is not sufficient to make a definitive diagnosis of the disease that plays a role in etiology. Conclusion: Differential diagnosis of GDs should be made in the light of histopathological findings, microbiological examination and clinical data. [ABSTRACT FROM AUTHOR]

Necrobiosis Lipoidica (NL) is a dermatological condition characterized by the development of granulomatous inflammation leading to the degeneration of collagen and subsequent formation of yellowish-brown telangiectatic plaques usually localized on the pretibial skin of middle-aged females. Due to its rarity and unclear etiopathogenesis, therapeutic options for NL are not well-standardized. Among them, photodynamic therapy (PDT) is an emerging tool, although its efficacy has primarily been evaluated in single case reports or small case series. This study reports the real-life experience of a cohort of NL patients treated with PDT at the Section of Dermatology of the University Hospital of Messina and Reggio-Emilia. From 2013 to 2023, 17 patients were enrolled —5 males (29%) and 12 females (71%) aged between 16 and 56 years (mean age: 42 ± 13 years), with a median duration of NL of 8 years. The overall complete clearance (>75% lesion reduction) was 29%, while the partial clearance (25–75% lesion reduction) was 59%, with 12% being non-responders. This study adds to the little amount of evidence present in the literature regarding the effectiveness of PDT in the treatment of NL. Variability in treatment responses among patients underscores the need for personalized protocols, optimizing photosensitizers, light sources, and dosimetry. The standardization of treatment protocols and consensus guidelines are essential to ensure reproducibility and comparability across studies. [ABSTRACT FROM AUTHOR]

Necrobiosis lipoidica (NL) is a rare granulomatous disease of a not fully understood etiopathogenesis. Classically, NL is associated with insulin-dependent diabetes mellitus. The disease often fails to respond to conventional treatments and adversely affects patients' quality of life. First-line medications are usually topical corticosteroids, but patients respond to them with varying degrees of success. Other options include tacrolimus, phototherapy, cyclosporine, fumaric acid esters, and biologics (adalimumab, etanercept, and infliximab). Our review aims to present new therapeutic approaches potentially effective in patients with refractory lesions, describe the presumed etiopathogenesis, and provide diagnostic guidance for clinicians. The review concludes that Janus kinase inhibitors and biologics such as ustekinumab and secukinumab can be used effectively in patients with recalcitrant NL. Another promising treatment option is tapinarof (an aryl hydrocarbon receptor agonist). However, studies on larger groups of patients are still needed to evaluate the effectiveness of different therapeutic options and to define consistent treatment regimens for NL. It is advisable to improve the awareness of physicians of various specialties regarding necrobiosis lipoidica as lesions diagnosed earlier usually have a better response to treatment. [ABSTRACT FROM AUTHOR]

Background: Necrobiosis lipoidica (NL) is a rare granulomatous skin disorder with a predilection for females, often associated with diabetes mellitus (DM). This paper aims to comprehensively review the literature on NL, focusing on its association with DM, thyroid disorders, and the metabolic syndrome. Methods: A systematic search was conducted in English-language literature from inception to October 2023, utilizing PubMed. We identified 530 studies and selected 19 based on clinical significance, statistical support, and relevance to the paper's goals. Results: The coexistence of NL and DM is prevalent, with rates ranging from 11% to 65.71%. NL may precede DM diagnosis and a correlation between NL and increased daily insulin requirements has been observed in such patients. NL is suggested as a potential prognostic marker for DM complications; however, recent studies question this association, highlighting the need for further research. Studies in the context of NL and Thyroid Disease indicate a correlation, especially with autoimmune thyroiditis. Regarding NL and Metabolic Syndrome, the prevalence of metabolic syndrome among NL patients is notably higher than in the general population. Additionally, DM patients with ulcerated NL commonly exhibit hypertension or obesity, raising questions about the potential influence of hypertension and obesity on NL ulcerations. Conclusion: Additional research is required to untangle the complex connections between NL and various comorbidities. [ABSTRACT FROM AUTHOR]

Purpose: To report the clinical and histopathologic features of necrobiosis lipoidica and associated retinal vasculitis and describe successful treatment of both skin and eye manifestations with adalimumab, a tumor necrosis factor (TNF)-alpha inhibitor. Observations: A 35-year-old patient with bipolar disorder and ocular hypertension was referred for evaluation of bilateral retinal vasculitis. Fluorescein angiography revealed bilateral occlusive retinal vasculitis. Physical exam was notable for multiple annular and round erythematous hyperpigmented and atrophic patches and plaques on both lower extremities. Skin biopsy revealed a diagnosis of necrobiosis lipoidica, a rare granulomatous skin disease. Both the patient's retinal vasculitis and skin patches responded favorably to treatment with adalimumab, a TNF-alpha inhibitor. Conclusions and importance: This case highlights the importance of obtaining a complete history and physical exam in patients who present with ocular inflammation, as extraocular manifestations of disease may be present. It also demonstrates the effectiveness of a multidisciplinary approach to evaluation and management of these patients, as both skin and eye involvement were successfully treated with adalimumab, a TNF-alpha inhibitor.

Key Clinical Message: Necrobiosis lipoidica is a rare cutaneous granulomatous disease that mainly affects diabetic patients. The perforating type of the disease is an uncommon variant that is resistant to therapy and can be easily identified using dermoscopy. Perforating necrobiosis lipoidica (NL) is a rare NL variant that primarily affects patients with diabetes mellitus (DM). Dermoscopy helps to differentiate this type of disease. The disseminated form of perforating NL mainly occurs in the setting of DM. Here we present a case of disseminated perforating NL in a 24‐year‐old woman with type 1 DM. [ABSTRACT FROM AUTHOR]

The article presents a description of a clinical case of necrobiosis lipoidica complicated by ulcera-tion in a 55-year-old woman and a brief review of the literature on the topic of modern ideas about necrobiosis lipoidica. The disease manifested itself in 2005 after traumatization of the upper third of both shins as a result of a fall. The patient did visit a dermatologist office for medical help, there was a slow progression of the disease. In 2017, as a result of visiting a dermatologist office at her place of residence, she was diagnosed with necrobiosis lipoidica. The ongoing therapy with vasodi-lators and topical steroids did not lead to significant improvement. In 2021, against the background of vaccination against COVID-19 (22.07.20201 and 12.08.2021 Gam-COVID-Vac Sputnik V) the patient notes a sharp deterioration of the process after the injection of the second component of the vaccine there were an increased in the area of lesion, ulceration. No data for diabetes mellitus were obtained during the examination. There is a history of a multi-nodular non-toxic goiter, for which a complete resection of the left lobe of the thyroid gland was performed in 1995 and a partial resection of the right lobe of the thyroid gland in 2003. The patient has been receiving L-Thyroxine replacement therapy since 2003. A biopsy of the affected skin was per-formed. The final diagnosis: necrobiosis lipoidica erythematous-ulcerative variant was established according to the pathoanatom-ic examination of the biopsy and atypical clinical picture. As a result of the therapy, the patient was discharged from the clinic with improvement.

Abstract Background Extensive loss of soft tissue and bone due to neoplasia, trauma, or infection in extremities often leads to amputation. Case presentation We present the case of a 72-year-old female patient presenting with an extended cutaneous squamous cell carcinoma of the lower leg, developed on top of necrobiosis lipoidica. After achieving the R0 resection, a 26 × 20-cm soft tissue and 15-cm tibial bone defect resulted. The contralateral leg had been lost due to the same disease 18 years before. We achieved a successful reconstruction of the leg using a pedicled fibula transplantation, an extended anterolateral thigh perforator flap, and an internal fixation with plate and screws. Two years after the original surgery, the patient is relapse-free and mobile, with adequate function of the reconstructed foot. Conclusions Our case presented a unique combination of pedicled fibula transplantation and free extended ALT perforator flap to reconstruct an extensive defect after resection of a rare cSCC on top of NL. In selected cases, the boundaries of limb salvage can be pushed far beyond the current standards of treatment.

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.

Necrobiosis Lipoidica (NL) is a dermatological condition characterized by the development of granulomatous inflammation leading to the degeneration of collagen and subsequent formation of yellowish-brown telangiectatic plaques usually localized on the pretibial skin of middle-aged females. Due to its rarity and unclear etiopathogenesis, therapeutic options for NL are not well-standardized. Among them, photodynamic therapy (PDT) is an emerging tool, although its efficacy has primarily been evaluated in single case reports or small case series. This study reports the real-life experience of a cohort of NL patients treated with PDT at the Section of Dermatology of the University Hospital of Messina and Reggio-Emilia. From 2013 to 2023, 17 patients were enrolled —5 males (29%) and 12 females (71%) aged between 16 and 56 years (mean age: 42 ± 13 years), with a median duration of NL of 8 years. The overall complete clearance (>75% lesion reduction) was 29%, while the partial clearance (25–75% lesion reduction) was 59%, with 12% being non-responders. This study adds to the little amount of evidence present in the literature regarding the effectiveness of PDT in the treatment of NL. Variability in treatment responses among patients underscores the need for personalized protocols, optimizing photosensitizers, light sources, and dosimetry. The standardization of treatment protocols and consensus guidelines are essential to ensure reproducibility and comparability across studies.

Necrobiosis lipoidica (NL) is a rare granulomatous disease of a not fully understood etiopathogenesis. Classically, NL is associated with insulin-dependent diabetes mellitus. The disease often fails to respond to conventional treatments and adversely affects patients’ quality of life. First-line medications are usually topical corticosteroids, but patients respond to them with varying degrees of success. Other options include tacrolimus, phototherapy, cyclosporine, fumaric acid esters, and biologics (adalimumab, etanercept, and infliximab). Our review aims to present new therapeutic approaches potentially effective in patients with refractory lesions, describe the presumed etiopathogenesis, and provide diagnostic guidance for clinicians. The review concludes that Janus kinase inhibitors and biologics such as ustekinumab and secukinumab can be used effectively in patients with recalcitrant NL. Another promising treatment option is tapinarof (an aryl hydrocarbon receptor agonist). However, studies on larger groups of patients are still needed to evaluate the effectiveness of different therapeutic options and to define consistent treatment regimens for NL. It is advisable to improve the awareness of physicians of various specialties regarding necrobiosis lipoidica as lesions diagnosed earlier usually have a better response to treatment.

Background: Necrobiosis lipoidica (NL) is a rare granulomatous skin disorder with a predilection for females, often associated with diabetes mellitus (DM). This paper aims to comprehensively review the literature on NL, focusing on its association with DM, thyroid disorders, and the metabolic syndrome. Methods: A systematic search was conducted in English-language literature from inception to October 2023, utilizing PubMed. We identified 530 studies and selected 19 based on clinical significance, statistical support, and relevance to the paper’s goals. Results: The coexistence of NL and DM is prevalent, with rates ranging from 11% to 65.71%. NL may precede DM diagnosis and a correlation between NL and increased daily insulin requirements has been observed in such patients. NL is suggested as a potential prognostic marker for DM complications; however, recent studies question this association, highlighting the need for further research. Studies in the context of NL and Thyroid Disease indicate a correlation, especially with autoimmune thyroiditis. Regarding NL and Metabolic Syndrome, the prevalence of metabolic syndrome among NL patients is notably higher than in the general population. Additionally, DM patients with ulcerated NL commonly exhibit hypertension or obesity, raising questions about the potential influence of hypertension and obesity on NL ulcerations. Conclusion: Additional research is required to untangle the complex connections between NL and various comorbidities.

Key Clinical Message Necrobiosis lipoidica is a rare cutaneous granulomatous disease that mainly affects diabetic patients. The perforating type of the disease is an uncommon variant that is resistant to therapy and can be easily identified using dermoscopy. Abstract Perforating necrobiosis lipoidica (NL) is a rare NL variant that primarily affects patients with diabetes mellitus (DM). Dermoscopy helps to differentiate this type of disease. The disseminated form of perforating NL mainly occurs in the setting of DM. Here we present a case of disseminated perforating NL in a 24‐year‐old woman with type 1 DM.

Necrobiosis lipoidica is a granulomatous skin condition commonly associated with diabetes. When associated with diabetes mellitus, the name expands to necrobiosis lipoidica diabeticorum (NLD). In these patients, this rare condition has an incidence rate of 0.3% to 1.6%. The cause of NLD remains unknown even though many speculations exist in the medical literature. The treatment of this condition has most researchers agreeing on the use of topical steroids for the anti-inflammatory effect on NLD; however, the role of glucose control in the treatment of this disorder has been debatable. The following case presents a patient who, despite better glucose control, did not improve until the introduction of doxycycline. [ABSTRACT FROM AUTHOR]

Background: Extensive loss of soft tissue and bone due to neoplasia, trauma, or infection in extremities often leads to amputation. Case presentation: We present the case of a 72-year-old female patient presenting with an extended cutaneous squamous cell carcinoma of the lower leg, developed on top of necrobiosis lipoidica. After achieving the R0 resection, a 26 × 20-cm soft tissue and 15-cm tibial bone defect resulted. The contralateral leg had been lost due to the same disease 18 years before. We achieved a successful reconstruction of the leg using a pedicled fibula transplantation, an extended anterolateral thigh perforator flap, and an internal fixation with plate and screws. Two years after the original surgery, the patient is relapse-free and mobile, with adequate function of the reconstructed foot. Conclusions: Our case presented a unique combination of pedicled fibula transplantation and free extended ALT perforator flap to reconstruct an extensive defect after resection of a rare cSCC on top of NL. In selected cases, the boundaries of limb salvage can be pushed far beyond the current standards of treatment. [ABSTRACT FROM AUTHOR]

Abstract 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.

Necrobiosis lipoidica is a chronic granulomatous disease of unknown etiopathogenesis, which is often difficult to treat. While data from randomized controlled trials for the treatment of necrobiosis lipoidica is lacking, several treatments of varying efficacy for necrobiosis lipoidica have been reported in the scientific literature. We present a case of a 29-year-old female with uncontrolled diabetes and treatment-resistant necrobiosis lipoidica which was responsive to ustekinumab.

We present a case of necrobiosis lipoidica (NL) of the right abdomen in a 75-year-old man. A skin biopsy performed showed a layered infiltrate of mono and multinucleated histiocytes palisaded around degenerated collagen bundles. Laboratory workup was unremarkable. The patient was treated with topical corticosteroids with cessation of progression of his disease, although the eruption did not resolve. There are a number of treatments for NL reported in the literature, all with varying efficacy. Although NL lesions are usually asymptomatic, patients with NL must be monitored closely for signs of ulceration or malignant transformation, in which case more aggressive treatment options may be warranted.

Necrobiosis lipoidica (NL) is a chronic granulomatous idiopathic disorder. It usually presents as yellow-brown, atrophic, telangiectatic plaques with an elevated violaceous rim, typically in the pretibial region of both extremities with and without ulceration [1, 2]. There are few reported cases of childhood-onset NL in atypical locations. We report a case of type 1 diabetic 13-year-old girl who developed yellow atrophic plaque with telangiectasia on the extensor aspect of her upper arm.

Necrobiosis lipoidica (NL) is a rare granulomatous skin disease of unknown origin that usually occurs in adults and requires long-term treatment. Here was reported a 7-year-old boy with histopathologically proven necrobiosis lipoidica (NL) on his lower back. He was successfully treated with intralesional and topical corticosteroids and tacrolimus ointment. This case is remarkable for its age of onset, location on the trunk, and absence of diabetes mellitus, which usually is significantly associated with the rare cases of NL in children. [ABSTRACT FROM AUTHOR]

Photodynamic therapy (PDT) is a photochemotherapy based on local application of a photosensitive compound and subsequent exposure to a light source of adequate wavelength. It is a non-invasive therapeutic procedure widely used in oncodermatology for treatment of numerous skin cancers, but in the last years its use has been gradually extended to an increasing list of skin diseases of both infectious and inflammatory nature. Although PDT is proven as a safe and effective therapeutic option in adults, its use is not well standardized in the pediatric population. In this review, we will focus on clinical applications, mechanisms of action, protocols, and adverse events in children and adolescents. Most of pediatric experiences concerned treatment of skin cancers in Gorlin syndrome and xeroderma pigmentosum, acne vulgaris, and viral warts, but other applications emerged, such as cutaneous lymphoma and pseudo-lymphomas, necrobiosis lipoidica, hidradenitis suppurativa, dissecting cellulitis, leishmaniasis, angiofibromas, verrucous epidermal nevus, and linear porokeratosis. In these pediatric diseases, PDT appeared as an effective therapeutic alternative. The results on vitiligo were limited and not fully encouraging. Although highly versatile, PDT is not a therapy for all skin diseases, and a deeper knowledge of its mechanisms of action is required to better define its spectrum of action and safety in pediatric patients. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Noninfectious (inflammatory) cutaneous granulomatous disorders include cutaneous sarcoidosis (CS), granuloma annulare (GA), necrobiosis lipoidica (NL), and necrobiotic xanthogranuloma (NXG). These disorders share macrophage-predominant inflammation histologically, but the inflammatory architecture and the pattern of extracellular matrix alteration varies. The underlying molecular explanations for these differences remain unclear., Objective: We sought to understand spatial gene expression characteristics in these disorders., Methods: We performed spatial transcriptomics in cases of CS, GA, NL, and NXG to compare patterns of immune activation and other molecular features in a spatially resolved fashion., Results: CS is characterized by a polarized, spatially organized type 1-predominant response with classical macrophage activation. GA is characterized by a mixed but spatially organized pattern of type 1 and type 2 polarization with both classical and alternative macrophage activation. NL showed concomitant activation of type 1, type 2, and type 3 immunity with a mixed pattern of macrophage activation. Activation of type 1 immunity was shared among, CS, GA, and NL and included upregulation of IL-32. NXG showed upregulation of CXCR4-CXCL12/14 chemokine signaling and exaggerated alternative macrophage polarization. Histologic alteration of extracellular matrix correlated with hypoxia and glycolysis programs and type 2 immune activation., Conclusions: Inflammatory cutaneous granulomatous disorders show distinct and spatially organized immune activation that correlate with hallmark histologic changes., Competing Interests: Disclosure statement Supported by a K08 grant from the National Institute of Allergy and Infectious Diseases (K08AI159229 to W.D.). Disclosure of potential conflict of interest: B. E. Shields has served as a consultant for Arcutis Biotherapeutics. C. A. Nelson has received research support from Boehringer Ingelheim. W. Damsky has served as a consultant for Pfizer, Eli Lilly, TWI Biotechnology, Fresenius Kabi, Epiarx Diagnostics, Boehringer Ingelheim, CSL Behring, AbbVie, and Sanofi; has been provided research support from Pfizer, Advanced Cell Diagnostics/Bio-Techne, AbbVie, Bristol Myers Squibb, and Incyte; and receives licensing fees from EMD/Millipore/Sigma. The rest of the authors declare that they have no relevant conflicts of interest., (Copyright © 2024 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

We present a 42-year-old woman with no history of diabetes or glucose intolerance who had a 5-year history of ulcerative necrobiosis lipoidica (NL). Despite failure of multiple medications, she experienced clearing of her ulcers after her treatment was changed to ustekinumab. We discuss our patient’s disease course and elaborate upon mechanistic reasons for her improvement related to ustekinumab therapy.