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.

A Breakthrough in the Treatment of Necrobiosis Lipoidica? Update on Treatment, Etiopathogenesis, Diagnosis, and Clinical Presentation.

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.

Shedding Light on Photodynamic Therapy in the Treatment of Necrobiosis Lipoidica: A Multicenter Real-Life Experience.

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 séptica de un fibroma uterino / Septic Necrobiosis of Uterine Fibroma

Los miomas o fibromas, son tumores benignos que aparecen frecuentemente en el útero, y aunque a veces pueden ser asintomáticos, en muchas mujeres son la razón más común de las intervenciones ginecológicas. Algunos autores consideran que la causa es desconocida, otros le dan valor al estímulo estrogénico mantenido o aumentado, que activa los genitoblastos, mesodérmico y celular embrionario que responden a este estímulo. Se presenta el caso de un fibroma uterino complicado con una necrobiosis séptica diagnosticada en una paciente de 63 años de edad. El apoyo diagnóstico con ultrasonografía es de vital importancia en la sospecha diagnóstica de este tipo tumor y sus complicaciones.

Myomas or fibroids are benign tumors that frequently appear in the uterus, and although they can sometimes be asymptomatic, in many women they are the most common reason for gynecological interventions. Some authors consider that the cause is unknown. Others give value to the maintained or increased estrogenic stimulus, which activates the embryonic cell, mesodermal and genitoblasts that respond to this stimulus. We present the case of a complicated uterine fibroid with a septic necrobiosis diagnosed in a 63-year-old patient. Ultrasonography is of vital importance in the diagnosis when there is suspicion of this type of tumor and its complications.

Diabetes mellitus and the skin

Abstract: Several dermatoses are routinely associated with diabetes mellitus, especially in patients with chronic disease. This relationship can be easily proven in some skin disorders, but it is not so clear in others. Dermatoses such necrobiosis lipoidica, granuloma annulare, acanthosis nigricans and others are discussed in this text, with an emphasis on proven link with the diabetes or not, disease identification and treatment strategy used to control those dermatoses and diabetes.

Necrobiosis lipoidica en un paciente con prediabetes / Necrobiosis lipoidica in a prediabetic patient

La necrobiosis lipoidica es una dermatosis poco frecuente, de origen desconocido. Puede observarse en pacientes sin alteración de la glucosa, pero frecuentemente está asociada a la diabetes mellitus bien establecida. Menos común es que estas lesiones en la piel aparezcan en estadios prediabéticos. Se describe el caso de una paciente femenina, de 65 años de edad, que hace 10 años comenzó a presentar una lesión papuloeritematosa de 1 cm, con bordes bien definidos, en la cara lateral interna de la pierna derecha. Recientemente le realizan biopsia de la lesión dermatológica y prueba de tolerancia a la glucosa oral, y se diagnostica necrobiosis lipoidica y tolerancia a la glucosa alterada respectivamente. Se piensa que los pacientes con esta enfermedad deben tener seguimiento a largo plazo, para diagnosticar oportunamente diabetes mellitus u otros estados prediabéticos(AU)

Necrobiosis lipoidica is a rare dermatosis of unknown origin. It can be observed in patients with unaltered glucose indexes but frequently associated to well-established diabetes mellitus. It is even less common that this type of skin lesions occur in pre-diabetic staging. This is the case of 65 years-old woman, who had presented 1 cm-long papulloerythematous lesion with well-defined contours for 10 years in the inner lateral side of her right leg. She had been recently biopsied in her cutaneous lesion and made a glucose-tolerance test, which yielded necrobiosis lipoidica and altered glucose tolerance. It is considered that the patients with this disease should be followed-up for a long period to timely diagnose diabetes mellitus or other pre-diabetic conditions(AU)

Necrobiosis lipoidica en un paciente con prediabetes / Necrobiosis lipoidica in a prediabetic patient

La necrobiosis lipoidica es una dermatosis poco frecuente, de origen desconocido. Puede observarse en pacientes sin alteración de la glucosa, pero frecuentemente está asociada a la diabetes mellitus bien establecida. Menos común es que estas lesiones en la piel aparezcan en estadios prediabéticos. Se describe el caso de una paciente femenina, de 65 años de edad, que hace 10 años comenzó a presentar una lesión papuloeritematosa de 1 cm, con bordes bien definidos, en la cara lateral interna de la pierna derecha. Recientemente le realizan biopsia de la lesión dermatológica y prueba de tolerancia a la glucosa oral, y se diagnostica necrobiosis lipoidica y tolerancia a la glucosa alterada respectivamente. Se piensa que los pacientes con esta enfermedad deben tener seguimiento a largo plazo, para diagnosticar oportunamente diabetes mellitus u otros estados prediabéticos(AU)

Necrobiosis lipoidica is a rare dermatosis of unknown origin. It can be observed in patients with unaltered glucose indexes but frequently associated to well-established diabetes mellitus. It is even less common that this type of skin lesions occur in pre-diabetic staging. This is the case of 65 years-old woman, who had presented 1 cm-long papulloerythematous lesion with well-defined contours for 10 years in the inner lateral side of her right leg. She had been recently biopsied in her cutaneous lesion and made a glucose-tolerance test, which yielded necrobiosis lipoidica and altered glucose tolerance. It is considered that the patients with this disease should be followed-up for a long period to timely diagnose diabetes mellitus or other pre-diabetic conditions(AU)

Necrobiosis lipoídica: una manifestación más de la diabetes mellitus / Necrobiosis lipoidica: A complication of diabetes mellitus

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Pápulas perladas en el antihélix / Pearly White Papules on the Antihelix

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Necrobiosis lipoídica / Necrobiosis lipoidica

Se presenta el caso de una paciente portadora de necrobiosis lipoídica, entidad que pertenece al grupo de las dermatosis cutáneas ulcerativas, es poco común, de causa desconocida, estrechamente relacionada a la diabetes mellitus, con manifestaciones clínicas características y de difícil tratamiento. Teniendo en cuenta los resultados del examen físico y complementarios, incluida la biopsia, se le diagnosticó necrobiosis lipoídica no diabética. Se le indicó tratamiento con Pentoxifilina y Ozonoterapia, evolucionando favorablemente (AU)

This is a case of a female patient with necrobiosis lipoidica, an entity that belongs to the group of uncommon ulcerative cutaneous dermatosis of unknown cause, closely related to diabetes mellitus, with characteristic clinical manifestations and very difficult to be treated. Considering the results of physical examination and investigations, including biopsy, she was diagnosed with non-diabetic necrobiosis lipoidica. She was treated with pentoxifylline and ozone therapy, so her condition is improving (AU)

Necrobiosis lipoídica diabética / Diabetic lipoidic necrobiosis

Se describe el caso clínico de una fémina de 39 años de edad, con diabetes mellitus de tipo II, quien ingresó en el Servicio de Dermatología del Hospital Provincial Clinicoquirúrgico Docente "Celia Sánchez Manduley" de la provincia de Granma por presentar, desde hacía más de un año, múltiples lesiones en los miembros inferiores, superiores y tronco. Teniendo en cuenta los resultados de los exámenes físico y complementarios, incluida la biopsia, se le diagnóstico necrobiosis lipoídica diabética. Se le indicó tratamiento con triamcinolona y evolucionó favorablemente.

The case report of a 39 years female patient with diabetes mellitus type II who was admitted in the Dermatology Service of "Celia Sánchez Manduley" Teaching Clinical Surgical Provincial Hospital of Granma province is described. She presented, for more than a year, multiple lesions in the lower and higher extremities and trunk. Keeping in mind the results of the physical and complementary exams, including biopsy, she had a diagnosis of diabetic lipoidic necrobiosis. She was prescribed a treatment with triamcinolona and she had a favorable clinical course.

Necrobiosis lipoídica en paciente diabético tipo 1: reporte de un caso / Necrobiosis lipodica in a type 1 diabetic patient: case report

La necrobiosis lipoídica es una enfermedad crónica granulomatosa de la piel de origen desconocido, que se caracteriza por placas inflamatorias amarronadas con bordes indurados y centro atrófico. Dos tercios de los casos se reportan en pacientes diabéticos, con predominio en el sexo femenino. Se describe el caso de una mujer de 18 años de edad con diagnóstico de diabetes tipo 1 de más de 15 años de evolución, en tratamiento con insulina detemir 88 U/día, con inadecuada adherencia al tratamiento y mal control metabólico. Presentó lesiones hipotróficas en cara anterior de ambas piernas (región pretibial derecha y antepié izquierdo), de centro ulcerado, no dolorosas, con bordes indurados, de meses de evolución. Se realizó diagnóstico de necrobiosis lipoídica mediante biopsia de piel. La paciente mejoró con tratamiento local de las lesiones (corticoides intralesionales y antibióticos tópicos) y adecuado control de la glucemia. En la literatura se discuten numerosas opciones de tratamiento, pero no existen recomendaciones terapéuticas firmes basadas en estudios controlados. (AU)

Necrobiosis lipoidica is a chronic granulomatous skin disease of unknown origin characterized by inflammatory plaques with indurated edges and brownish atrophic center. Two thirds of cases are reported in diabetic patients, with a predominance in females. The case of a 18-year-old woman with type 1 diabetes for 15 years is described; she was on insulin detemir 88 U/day, with inadequate adherence to treatment and poor metabolic control. She presented painless hypotrophic dermic lesions in the anterior aspect of both legs and in the left forefoot; they had ulcerated center and indurated edges; the evolution was chronic (several months). Diagnosis of necrobiosis lipoidica was made by skin biopsy. The patient improved with local treatment of lesions (intradermic steroids and topical antibiotics) and adequate glycemic control. In the literature many treatment options are discussed, but there are no firm therapeutic recommendations based on controlled studies. (AU)

Necrobiosis lipoídica en paciente diabético tipo 1: reporte de un caso / Necrobiosis lipodica in a type 1 diabetic patient: case report

La necrobiosis lipoídica es una enfermedad crónica granulomatosa de la piel de origen desconocido, que se caracteriza por placas inflamatorias amarronadas con bordes indurados y centro atrófico. Dos tercios de los casos se reportan en pacientes diabéticos, con predominio en el sexo femenino. Se describe el caso de una mujer de 18 años de edad con diagnóstico de diabetes tipo 1 de más de 15 años de evolución, en tratamiento con insulina detemir 88 U/día, con inadecuada adherencia al tratamiento y mal control metabólico. Presentó lesiones hipotróficas en cara anterior de ambas piernas (región pretibial derecha y antepié izquierdo), de centro ulcerado, no dolorosas, con bordes indurados, de meses de evolución. Se realizó diagnóstico de necrobiosis lipoídica mediante biopsia de piel. La paciente mejoró con tratamiento local de las lesiones (corticoides intralesionales y antibióticos tópicos) y adecuado control de la glucemia. En la literatura se discuten numerosas opciones de tratamiento, pero no existen recomendaciones terapéuticas firmes basadas en estudios controlados.

Necrobiosis lipoidica is a chronic granulomatous skin disease of unknown origin characterized by inflammatory plaques with indurated edges and brownish atrophic center. Two thirds of cases are reported in diabetic patients, with a predominance in females. The case of a 18-year-old woman with type 1 diabetes for 15 years is described; she was on insulin detemir 88 U/day, with inadequate adherence to treatment and poor metabolic control. She presented painless hypotrophic dermic lesions in the anterior aspect of both legs and in the left forefoot; they had ulcerated center and indurated edges; the evolution was chronic (several months). Diagnosis of necrobiosis lipoidica was made by skin biopsy. The patient improved with local treatment of lesions (intradermic steroids and topical antibiotics) and adequate glycemic control. In the literature many treatment options are discussed, but there are no firm therapeutic recommendations based on controlled studies.

Necrobiosis lipoídica diabética / Diabetic lipoidic necrobiosis

Se describe el caso clínico de una fémina de 39 años de edad, con diabetes mellitus de tipo II, quien ingresó en el Servicio de Dermatología del Hospital Provincial Clinicoquirúrgico Docente Celia Sánchez Manduley de la provincia de Granma por presentar, desde hacía más de un año, múltiples lesiones en los miembros inferiores, superiores y tronco. Teniendo en cuenta los resultados de los exámenes físico y complementarios, incluida la biopsia, se le diagnóstico necrobiosis lipoídica diabética. Se le indicó tratamiento con triamcinolona y evolucionó favorablemente(AU)

The case report of a 39 years female patient with diabetes mellitus type II who was admitted in the Dermatology Service of Celia Sánchez Manduley Teaching Clinical Surgical Provincial Hospital of Granma province is described. She presented, for more than a year, multiple lesions in the lower and higher extremities and trunk. Keeping in mind the results of the physical and complementary exams, including biopsy, she had a diagnosis of diabetic lipoidic necrobiosis. She was prescribed a treatment with triamcinolona and she had a favorable clinical course(AU)