Lupus pernio vs. lupic perniosis: a case report

Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease that is difficult to diagnose due to the wide array of signs and symptoms it displays that may be associated to multiple clinical conditions, including perniosis (a rare inflammatory condition), lupus pernio (a manifestation of sarcoidosis), and lupus perniosis (a form of SLE), which can be easily mistaken. Case description: A 29-year-old Colombian mestizo woman with no family history of autoimmune, inflammatory or cutaneous diseases was diagnosed with SLE after ruling out several differential diagnoses. Although the patient presented with features of lupus pernioticus (lupus perniosis), it was established that she had lupus pernio, a type of sarcoidosis. The patient was given the indicated treatment, which led to an improvement in her quality of life. Conclusion: Based on the epidemiology, clinical history and histopathologic findings, it was possible to establish that the patient presented with lupus perniosis and not lupus pernio. In that regard, considering that these three conditions (perniosis, lupus pernio and lupic perniosis) can be easily confused, the present case highlights the importance of a thorough clinical evaluation and precise use of diagnostic terms, because these are three different conditions despite their similar names.

Introducción. El lupus eritematoso sistémico (LES) es una enfermedad autoin-munitaria difícil de diagnosticar debido a la gran variedad de síntomas y signos que ocasionan las múltiples condiciones clínicas que puede provocar, tales como la perniosis (una condición inflamatoria rara), el lupus pernio (una manifestación de la sarcoidosis) y la perniosis lúpica (una forma de LES), las cuales pueden ser fácilmente confundidas. Presentación del caso. Mujer colombiana de 29 años, mestiza y sin antecedente familiar de enfermedades autoinmunes, inflamatorias o cutáneas, quien luego del descarte de varios diagnósticos diferenciales, fue diagnosticada con LES. Aunque la paciente presentó características de lupus perniótico (perniosis lúpica), se estableció que presentaba lupus pernio, un tipo de sarcoidosis. A la paciente se suministró el tratamiento indicado, con lo cual logró una mejoría en sus condiciones de vida. Conclusión. Considerando la epidemiología, la historia clínica y los hallazgos histopatológicos, se puede establecer que la paciente presentó perniosis lúpica y no lupus pernio. En este sentido, teniendo en cuenta que la perniosis, el lupus pernio y la perniosis lúpica pueden confundirse, el presente caso pone de manifiesto la importancia de hacer una evaluación clínica completa y usar los términos diagnósticos más precisos, pues aunque sean similares en nombre, estas son tres condiciones diferentes.

Chilblain lupus with nail involvement: a case report

Chilblain lupus is a rarely manifested variant of chronic lupus. Its appearance can be sporadic or hereditary associated with an autosomal dominant mutation. The diagnosis is clinical and histopathological. The case is presented of a patient with systemic lupus erythematosus presenting with chilblain lupus and nail involvement, despite the use of antimalarials and immunomodulators.

La perniosis lúpica es una variante del lupus crónico que se manifiesta con poca frecuencia, su aparición puede ser esporádica o hereditaria, asociada con una mutación autosómica dominante, en tanto que su diagnóstico es clínico e histopatológico. Se reporta el caso de una mujer con lupus eritematoso sistémico con manifestación de perniosis lúpica y compromiso ungueal, a pesar del uso de antimaláricos e inmunomoduladores.

Lesiones en piel en pediatría en periodos de COVID-19. Reporte de un caso

RESUMEN En noviembre del 2019 inicia en Wuhan, lo que sería una nueva pandemia, por un coronavirus que no había sido identificado previamente, el SARS-CoV-2. Posteriormente fue reportado en Paraguay el primer caso de covid-19 en marzo del 2020. A pesar de que se trata de un virus de manifestación preferentemente respiratoria, sabemos hasta la fecha que pueden presentar otros síntomas, incluso ser totalmente asintomáticos. Se ha visto que las lesiones en piel, al igual que en muchas otras enfermedades infecciosas, también son causadas por este nuevo virus, por lo que el dermatólogo juega un rol importante en esta batalla. Reportamos el primer caso registrado por el servicio de Dermatología del Hospital Nacional de Itaugua, de manifestaciones cutáneas en un lactante, secundarias a la enfermedad de COVID-19.

ABSTRACT In November 2019, in Wuhan, China, what would be a new pandemic started, due to a coronavirus that had not been previously identified, SARS-CoV-2. Subsequently, the first case of COVID-19 was reported in Paraguay in March 2020. Despite the fact that it is a virus of preferential respiratory manifestation, we know to date that it can present with other symptoms, including asymptomatically. Skin lesions, like in many other infectious diseases, have been shown to be caused by this new virus as well, making the dermatologist play an important role in this battle. We report the first case registered by the Dermatology service of the Itaugua National Hospital of cutaneous manifestations in an infant, secondary to COVID-19 disease.

Acrosíndromes / Acrosyndromes

En la medicina convencional se denominan acrosíndromes a todas las enfermedades que afectan la microcirculación cutánea; sin embargo es de suma importancia diferenciarlos entre ellos, puesto que aunque comparten características clínicas similares, su causa, pronóstico y tratamiento es diferente. En este artículo se describen las enfermedades más representativas de este grupo de entidades, tales como fenómeno de Raynaud, acrocianosis, livedo reticularis, eritromelalgia, eritema pernio y enfermedad de Buerger; haciendo una descripción detallada de cada una, para que el lector consiga hacer un adecuado diagnóstico diferencial y logre enfocar correctamente los pacientes en la práctica médica.

In conventional medicine, are called acrosyndromes all diseases that affect the skin microcirculation, however it is important to differentiate between them, because although they share similar clinical characteristics; cause, prognosis and treatment is different, and the distinction between them, will achieve impact in the quality of life of patients. Throughout this article, the most representative diseases in this group are described as: Raynaud's phenomenon, acrocyanosis, livedo reticularis, erythromelalgia, erythema pernio and Buerger disease; doing a detailed description of each, emphasizing what makes them unique, thus the reader gets to make an appropriate differential diagnosis and will approach properly the patients with these conditions.

Case for diagnosis / Caso para diagnóstico

Chilblains are an inflammatory skin condition that develops following exposure to the cold. They consist of pruritic and/or painful erythematous-to-violaceous acral lesions and are the result of an abnormal vascular response to exposure to the cold. This benign condition is common in southern Brazil, particularly in Rio Grande do Sul where climatic conditions, together with cultural and economic aspects, facilitate the development of these sores.

Eritema pérnio é uma condição inflamatória da pele que ocorre após exposição ao frio e se manifesta com lesões eritêmato-violáceas, pruriginosas e dolorosas nas extremidades. Ocorre devido a uma resposta vascular alterada à exposição ao frio. Esta condição benigna é frequente na região sul do Brasil, especialmente no Rio Grande do Sul, onde as características clínicas, associadas a aspectos culturais e econômicos, promovem o desenvolvimento do eritema pérnio.

Lupus Pernio as the Early Cutaneous Presentation of Sarcoidosis / 대한피부과학회지

Lupus pernio is the most characteristic cutaneous lesion of sarciodosis and this presents as an indurated red to blue-purple, swollen, shiny lesion that is often localized on the nose, cheeks, lips or ears of middle-aged female adults. It is a chronic malady that rarely shows spontaneous remission, and it sometimes causes deformity. It generally occurs as cutaneous lesions of sarcoidosis patients with a long duration of disease, and not as early cutaneous lesions of sarcoidosis. This case concerns a 43-year-old woman who presented with a 1 year history of firm erythematous and violaceous plaque with multiple erythematous and violaceous papules on the collumella and right nasal cavity. Skin biopsy showed noncaseasting granuloma. Herein, we report on a typical case of lupus pernio as the early cutaneous presentation of sarcoidosis, which has rarely been reported in the Korean literature. We suggest that early diagnosis and treatment is necessary to prevent deformity.

A Case of Childhood Pernio Treated with Nifedipine / 대한피부과학회지

Pernio, or chilblains, are a localized inflammatory lesion of the skin resulting from an abnormal response to cold. The condition is characterized by cutaneous lesions located on the distal parts, such as the fingers, toes, nose and ears. Pernio develops among susceptible individuals who are exposed to non-freezing condition and most commonly occurs among young women and children. However, reports on childhood pernio are rarely found. Several conditions have been described as predisposing factors to pernio, including excessive cold exposure, low-grade vasculitis, the presence of cryoproteins, anorexia nervosa, systemic lupus erythematous and antiphospholipid antibodies. It is important, therefore, when evaluating a patient with chronic pernio who is resistant to conservative management, to exclude an underlying condition and also to determine whether additional testing is necessary. Herein, we present an uncommon case of a 5-year-old female patient with chronic pernio that may be associated with the presence of cold agglutinin. She received topical steroids prior to evaluation but no improvement was seen. Howere, treatment with calcium channel blocker, nifedipine, was of benefit to our patient.

Clinical and Histopathologic Features of Pernio / 대한피부과학회지

BACKGROUND: Pernio or chilblain is an abnormal reaction to damp cold air, resulting in pruritic erythematous swellings on acral skin. The histologic features are subepidermal edema, diffuse fluffy edema of endothelial cell, and superficial or superficial and deep perivascular lymphocytic infiltrates. Many studies with a large sample size have been conducted in western countries, but there were no collective studies of pernio that have been reported in Korea. OBJECTIVE: The purpose of this study was to clarify the clinical and histopathologic features of pernio in Korea. METHODS: We reviewed the clinical and histopathologic findings in ten patients with pernio who visited the Department of Dermatology, Kosin Medical College Gaspel Hospital during the 10 year period from 1988 to 1997. RESULTS: The results were as follows.1. The age distribution was in the range of 6-50 year old. The prevalance was heighest in the third and fourth decade. Sex ratio was 1:4 (2 males :8 females).The duration of symptoms ranged from 4 days to 30 years. All patients developed skin lesions on the acral area. Two male patients developed skin lesions on the hand associated with cold exposure and outdoor works. All patient had itching and burning papules and/or plaques, and one had a painful hard nodule. Laboratory studies were all negative and within normal ranges.2. Histopathologic features revealed subepidermal edema, diffuse fluffy edema of endothelial cell, and superficial or superficial and deep perivascular lymphocytic infiltrates in all specimens. 3. Treatment includes prophylatic measures against cold and nifedipine(calcium channel inhibiting drug). In more severe cases, pentoxifylline might be helpful. CONCLUSION: Our study suggests that pernio commonly occurs in twenty to forty year old females during fall and winter. Upon exposure to cold temperatures, the acral skin develops pruritic erythematous papules and plaques. If these lesions persisit for a long time or recur, they may progress to vesicle and ulceration and possibly develop vasculitis which does not respond to therapy well. Thus, a thorough understanding of clinical and histopathologic features of pernio is vital in making a diagnosis, a treatment plan and determining the prognosis.

A Case of Lupus Pernio / 대한피부과학회지

A 36-year-old female patient with sarcoidosis is presented showing lupus pernio, papular skin lesions, cervical lymph node enlargement and lung lesions. Diagnosis was confirmed by characteristic histologic findings of noncaseating granulomas in skin and cervical lymph node, supported by compatible clinical and laboratory features. Methatrexate was administered orally, which resulted in considerable improvement of skin lesions.