Erythema elevatum diutinum and hypothyroidism: coincidence or causal relationship?

AbstractErythema elevatum diutinum is a rare chronic leukocytoclastic vasculitis of unknown etiology. It is believed to be due to deposition of immune complexes in the vessels. Clinically it is manifested as erythematous violaceous papules and nodules, isolated or confluent with hardened consistency, symmetrical, usually located on the extensor surface of the extremities, particularly over the joints. Diagnosis is based on clinical and histological findings. We report the case of a woman, 71 years old, with erythematous violaceous nodules on the hands, elbows, back and legs, beginning two years ago, with pain and itching. Histopathological analysis revealed leukocytoclastic vasculitis, confirming the clinical suspicion. Laboratory tests revealed hypothyroidism. We report the case because of its rarity, with subsequent review of the literature.

Concurrence of Sweet's Syndrome, Pathergy Phenomenon and Erythema Nodosum-like Lesions

We report the case of a 54-year-old woman with concurrent Sweet's syndrome, pathergy phenomenon and eythema nodosum-like lesions associated with suppurative tonsillitis. Tender, violaceous and highly edematous papules and plaques were detected on the forearms and legs, some of which were pseudovesicular. Similar edematous papules were detected on the dorsum of the hands at the sites of intravenous injection. Biopsies of a plaque on the forearm and a papule at the site of intravenous injection confirmed the presence of Sweet's syndrome. Multiple, tender, violet-red, subcutaneous erythematous nodules were revealed on the legs. A biopsy taken from a nodule on the leg revealed septal panniculitis. To our knowledge, no patient with concurrent Sweet's syndrome, pathergy phenomenon, and erythema nodosum-lesions had been reported yet in previous literature.

Hanseníase históide símile: desafio diagnóstico / Histoid like leprosy: diagnostic challenge

Em 1963, Wade descreveu a hanseníase histoide, que acometia pacientes previamente tratados com dapsona.Caracteriza-se por lesão com aspecto queloideano, na histopatologia vemos histiócitos fusiformes e um grande número de bacilos. Relata-se caso de hanseníase dimorfa virchoviana com padrão de lesão históide, um verdadeiro desafio diagnóstico, a fim de atentar para manifestações atípicas dessa doença e reforçar a importância da confirmação histológica de casos suspeitos de hanseníase históide.

In 1963, Wade described Histoid Leprosy, that affected patients previously treated with dapsone. This type of leprosy is characterized by keloid-like lesions, in which spindle histiocytes and a large number of bacilli can be seen in the histopathology. A case of borderline lepromatous leprosy with histoid pattern is described, a true diagnostic challenge, in order to attempt to atypical manifestations of the disease and reinforce the importance of histological confirmation in suspected cases of histoid leprosy.

Infection of the lymphatic system by Aureobasidium pullulans in a patient with erythema nodosum leprosum

Aureobasidium pullulans is a causal agent of phaeohyphomycosis, occasionally found in men and animals. As an agent of different opportunistic fungal processes, it may cause fungemia, systemic infections and abscesses in different viscera. This paper aims to report a case of a patient with infection of the lymphatic system by A. pullulans. A 23-year-old patient being treated for erythema nodosum leprosum presented a 60-day complaint of daily fever, hoarseness, odynophagia and weight loss. Laboratory tests showed pancytopenia with severe neutropenia, cervical adenomegaly and solid contrast uptake lesion in the oropharyngeal region. Due to neutropenia and sepsis the patient was initially treated with cefepime and vancomycin, but there was no clinical improvement. Lymph node puncture-aspiration showed yeast-form fungus identified as A. pullulans by sequencing ITS region. The patient was treated with amphotericin B deoxycholate, leading to complete recovery of bone marrow function and regression of adenomegaly and the oropharyngeal lesion.

Crohn's Disease Presenting with Erythema Nodosum as an Early Sign: A Case Report / 대한소화기학회지

Erythema nodosum is the most common form of septal panniculitis and the most frequent skin manifestation associated with inflammatory bowel disease, affecting up to 15% of Crohn's disease patients. Since the development of erythema nodosum is closely related with a variety of disorders and condition, it can serve as an important early sign of systemic disease. Here, we present the occurrence of erythema nodosum as an early sign of Cronh's disease in a 16-year-old woman.

Síndrome de Lõfgren / Lõfgren’s Syndrome

A sarcoidose é uma doença crônica granulomatosa, de origem desconhecida, que pode envolver praticamente qualquer órgão, mas na qual a presença de artrite é incomum. Apresentamos um caso no qual a artrite foi o achado inicial, associada ao eritema nodoso, levando à investigação e ao diagnóstico da síndrome de Lõfgren. Nessa forma de apresentação da sarcoidose, além da artrite ou artralgias e do eritema nodoso, também deve estar presente a linfadenopatia hilar bilateral como critério diagnóstico. A síndrome de Lõfgren é chamada algumas vezes de sarcoidose aguda, estando geralmente associada com um bom prognóstico e resolução espontânea.

Sarcoidosis is a chronic granulomatous disease, of unknown origin, that may involve virtually any organ, but in which the presence of arthritis is uncommon. Here we present a case in which arthritis was the first finding, associated with erythema nodosun, leading to the investigation and diagnosis of Lõfgren Syndrome. In this form of sarcoidosis presentation, in addition to arthritis or arthralgia and erythema nodosun there must be bilateral hilar lymphadenopathy as a diagnostic criterion. Lõfgren’s syndrome is sometimes called acute sarcoidosis, usually being associated with good prognosis and spontaneous resolution.

Histopathological study of ocular erythema nodosum leprosum and post-therapeutic scleral perforation: a case report.

Leprosy is a chronic granulomatous disease caused by Mycobacterium leprae , clinically present either as tuberculoid, borderline or lepromatous type. Erythema nodosum leprosum (ENL) is an acute humoral response in the chronic course of lepromatous leprosy. Although very severe ENL reactions are known in systemic leprosy, such severity is rare in ocular tissues. A leprosy uveitis patient suffered from a severe form of post-therapeutic ENL reaction which resulted in perforation of the globe at the site of preexisting subconjunctival leproma. Painful blind eye was enucleated. Histopathological study revealed infiltration of numerous polymorphs and macrophages packed with acid-fast bacilli in the conjunctiva, cornea, ciliary body, ora serrata and sclera. A profuse influx of neutrophils on a background of macrophages packed with M. leprae confirmed the ocular ENL reaction. This case is reported to alert the ophthalmologists to a rare ocular complication of ENL.

Cutaneous sarcoidosis: clinical profile of 23 Indian patients.

BACKGROUND: Sarcoidosis is a multisystem disease of undetermined etiology. Indian studies on cutaneous sarcoidosis are not many and mainly comprise case reports. AIMS: This retrospective study was carried out to assess the clinical profile of sarcoidosis patients presenting with cutaneous lesions. METHODS: All histopathologically proven cases of cutaneous sarcoidosis seen consecutively between 1999 and 2004 were studied. Their age, sex, presenting features, evolution of disease and laboratory parameters were analyzed. RESULTS: A total of 23 patients (F:M 15:8) between 31 to 78 years (mean 44.3 years) of age had the mean duration of skin lesions of 1.4 years. Six patients had one to four lesions; two patients each had scar sarcoidosis and angiolupoid and one patient each had recurrent erythema nodosum, leg lymphedema and subcutaneous sarcoidosis. Others showed combination of papules, nodules, plaques and psoriasiform lesions. Peripheral lymph nodes were involved in two patients. Among 10 patients of pulmonary involvement, three had become symptomatic four months to four years after the cutaneous lesions. Routine laboratory investigations including serum calcium estimation were normal in all cases. Serum angiotensin-converting enzyme levels were raised in 3 out of 6 patients. Asymptomatic lytic lesions of digital bones were detected in hand X-ray of one patient. CONCLUSION: Skin lesions of sarcoidosis are like the tip of an iceberg indicating more changes in other organs. The symptomatology and abnormal laboratory results do not necessarily correlate with the severity of cutaneous involvement in general.

Coccidioidomycosis presenting as a massive pleural effusion in a postpartum woman.

A 33-year-old, postpartum woman presented with a massive, left pleural effusion following a self-limiting course of erythema nodosum. Pleural biopsy confirmed a diagnosis of coccidioidomycosis. This is the first reported case in the literature of coccidioidomycosis related to pregnancy, presenting as a massive pleural effusion.

Necrotic erythema nodosum leprosum.

A 30 year-old farmer presented himself with mild degree fever, joint pain and multiple ulcers of 15 days' duration. Ulcers were multiple, well-defined, irregular in shape with necrotic base and elevated, sloppy margins over anus, forearms, wrists, buttocks, lower half of thighs and shins. A few of the ulcers were covered with black-coloured scab. They were tender and with serosanguinous discharge. Black atrophic scars and ill-defined, irregular, brown black-pigmented macules of various sizes were present over thighs and legs. Excision biopsy of the ulcer showed features of erythema nodosum leprosum. The patient was treated with systemic corticosteroids.

A Case of Erythema Nodosum and Serositis Associated with Myelodysplastic Syndrome

Myelodysplastic syndrome (MDS) is a heterogenous group of stem cell disorders usually characterized by progressive refractory cytopenias, which could progress to acute myeloid leukemia. MDS may be associated with a wide spectrum of skin lesions, including neoplastic cell infiltration, Sweet's syndrome, pyoderma gangrenosum, erythema elevatum diutinum, vasculitis, and panniculitis. However, erythema nodosum is rarely associated with MDS. Unusual rheumatologic manifestations in patients with MDS also have been reported, which range from asymptomatic serological abnormalities to classic connective tissue disorders such as Sjogren's syndrome, relapsing polychondritis, systemic lupus erythematosus, rheumatoid arthritis and mixed connective tissue disease. However, concurrent erythema nodosum and serositis has rarely been reported. We describe a case of MDS with erythema nodosum and immune-mediated pericardial effusion in a 34-year-old woman.

Does pentoxifylline find a place in the armementarium of leprologists in type II reaction?

Alternative therapeutic interventions in Type II lepra reaction are being considered following serious problems associated with the use of steroids and thalidomide. Pentoxifylline (PTX) has been used in Type II reaction with varying degrees of success. The results of a study on the use of this drug in a dose of 1200 mg per day for a period of 2 months in patients with ENL reaction are discussed. Five patients, one of whom was HIV positive--all with severe Type II reaction, were regularly evaluated for regression of inflammatory symptoms and clinical involution of ENL lesions while on PTX therapy and thereafter. It was found that PTX led to a total elimination of systemic symptoms within a week. ENL lesions regressed in two weeks. However, in one patient, lesions recurred after one month of therapy. It appears that PTX is well tolerated and could be used as an additional drug in the armamentarium of leprologists in the management of Type II reaction, especially in HIV co-infection, where long-term steroids are contraindicated. However, further studies to compare the effects of PTX with currently, widely used drugs for the treatment of ENL reaction are necessary.

Características clínico-histopatológicas dos estados reacionais na hanseníase em pacientes submetidos à poliquimioterapia (PQT) / Clinical and histopathological characteristics of reactional states in leprosy patients submitted to multi-drug therapy

O objetivo deste estudo foi analisar os aspectos clínicos e histopatológicos dos estados reacionais em 169 pacientes multibacilares submetidos à poliquimioterapia. Em adiçäo foram avaliadas a frequência e a distribuiçäo desses episódios reacionais. Os casos foram classificados, com base nos dados clínicos e histopatológicos, em eritema nodoso hansênico - ENH (51 porcento), reaçäo reversa - RR (42 porcento) e neurite isolada (7 porcento). Verificou-se que os episódios reacionais säo complicaçäo freqüente (59 porcento) e ocorrem em qualquer momento no curso da doença. Observou-se que 77 porcento dos pacientes que desenvolveram ENH e 43 porcento dos pacientes com RR apresentaram manifestaçöes sistêmicas. A RR predominou entre os pacientes portadores da forma borderline-borderline (74 porcento), enquanto o ENH predominou naqueles diagnosticados pela forma lepromatosa-lepromatosa (86 porcento). O ENH foi significativamente mais recorrente do que a RR, que predominou no primeiro ano de PQT, enquanto o ENH no segundo ano

Eritema nodoso: estudio prospectivo de su etiología en treinta y ocho pacientes / Erythema nodosum: prospective study of its etiology in 38 patients

Objetivo del trabajo: Hallar la etiología del EN en los pacientes de nuestra institución y determinar si existen caracteres clínicos o exámenes complementarios sugestivos de esas etiologías. Material y métodos: Entre abril de 1988 y abril de 1995 se estudiaron en forma prospectiva 38 pacientes con EN. Todos fueron examinados mediante una matriz de datos clínicos única y a todos se les solicitó los mismos estudios de laboratorio. En 34 pacientes se obtuvo una biopsia de piel. Se definieron previamente los criterios diagnósticos que permitieran establecer luego la etiología del EN (estreptocócico, medicamentoso, tuberculoso, etc). Resultados: La etiología pudo ser determinada en el 69 por ciento de los pacientes. En 18 casos EN estreptocócico, en 4 casos coincidió con medicamentos, en 3 casos fue atribuido al embarazo, en otros 3 a TBC (2 casos de adenitis TBC y uno asociado a tuberculide papulonecrótica) y en un caso se diagnosticó una enfermedad de Hodgkin. En una paciente, el EN se asoció a síndrome de Sweet y en otra a necrobiosis lipoídica

Eritema nodoso asociado con enfermedad periodontal: reporte de un caso / Nodosum erythema in association with periodontal disease: report of a case

El eritema nodoso (EN) es una reacción inflamatoria caracterizada por lesiones nodulares subcutáneas en superficies extensoras y más frecuentemente, en las extremidades inferiores. El EN se asocia con una variedad de padecimientos, en su mayoría infecciosos, inducidos por fármacos o por problemas neoplásicos. Este es un caso en donde el EN crónico está asociado a enfermedad periodontal recurrente. En cuanto la enfermedad periodontal se resolvió, el EN remitió

Síndrome de Sweet y eritema nodoso / Sweet's syndrome and erythema nodosum

El síndrome de Sweet y el eritema nodoso representan dermatosis reactivas a diversos estímulos o trastornos asociados, algunos de los cuales han sido involucrados en el origen de ambos procesos por separado. Por este motivo, su concurrencia no debe ser considerada una asociación fortuita, sino más bien como diferentes respuestas cutáneas a un factor precipitante común. Presentamos 3 mujeres con aparición simultánea de síndrome de Sweet y eritema nodoso. En dos de ellas, ambas dermatosis ocurrieron tras una infección de la vía aérea superior, respondiendo rápidamente a prednisona oral. La tercera presentaba una celulitis bacteriana asociada que fue tratada exitosamente con cloxacilina; ésto se acompañó de una remisión completa de las lesiones de síndrome de Sweet, conjuntamente con las de eritema nodoso