Lupus Pernio Research Articles

Skinsarcoidosis: A trick for primary case physicians

Sarcoidosis is a systemic disease of unknown etiology. Skin lesions occur in about a quarter of patients with sarcoidosis and specific manifestations include erythema nodosum, maculopapular eruption, plaques, lupus pernio and scar sarcoidosis. A 39-year old male presented with cutaneous involvement of sarcoidosis. The skin biopsy revealed non-caseating granuloma. Our patient had skin manifestation of makulopapular eruption form of skinsarcoidosis along with hilar and mediastinal lymphadenopathy. The 40 mg/day oral methylprednisolone was started and skin lesions were fully recovered. We report a case of skin involvement as a first sign of sarcoidosis.

抗セントロメア抗体陽性を示したびまん浸潤型皮膚サルコイドーシス(lupus pernio)の1例

抗セントロメア抗体陽性を示したびまん浸潤型皮膚サルコイドーシス(lupus pernio)の1例

Successful Treatment of Lupus Pernio With Adalimumab

Successful Treatment of Lupus Pernio With Adalimumab

Specific cutaneous lesions in patients with systemic sarcoidosis: relationship to severity and chronicity of disease

Specific (granulomatous) cutaneous lesions are seen in 9-37% of cases of systemic sarcoidosis, and are usually classified into maculopapules, plaques, lupus pernio (LP), scar sarcoidosis, and subcutaneous sarcoidosis. Their prognostic significance has not been fully established. To analyse the relationship between the clinical type of granulomatous cutaneous lesions and the systemic features and prognosis of systemic sarcoidosis. The clinical charts of 86 patients (19 men, 67 women, mean age 46.82 years) with systemic sarcoidosis and granulomatous cutaneous involvement followed up for > 2 years at Bellvitge University Hospital were reviewed. Cutaneous lesions developed before or at the time of diagnosis of systemic sarcoidosis in 80.23% of patients. The main cutaneous lesions were classified as maculopapules (28 patients), plaques (31), LP (6), scar sarcoidosis (7) and subcutaneous sarcoidosis (14). Erythema nodosum (EN) was seen in 30 patients. Radiological stage was 0 for 8 patients, I for 48, II for 24, III for 5 and IV for 1. Systemic sarcoidosis activity persisted for > 2 years in 47 patients, and 42 received systemic corticosteroid treatment for their disease. Maculopapular and subcutaneous sarcoidosis were mainly seen in patients with EN and radiological stage I. Plaques and LP were associated with chronic disease and requirement for systemic corticosteroids. Cutaneous granulomatous lesions are usually present at the diagnosis of systemic sarcoidosis, and the type of cutaneous involvement may have prognostic significance.

Infliximab for treating sarcoidosis patients, Portuguese experience

Despite aggressive treatment, sarcoidosis may be debilitating and progressive. The role of tumor necrosis factor (TNF) ap in the genesis of granulomas is ambiguous. It has proved to be critical in the formation and maintenance of granulomatous inflammation and its antagonist, Infl iximab, has therefore been used with success in the treatment of patients with sarcoidosis. There are, however, reports of onset of sarcoidosis in patients treated for other conditions and where there had been no outbursts before submission to this therapy. We used Infl iximab in the treatment of patients with sarcoidosis who either had not responded to corticosteroids and other conventional drugs or had developed unacceptable side effects to these drugs. The initial dose was 5 mg/kg body weight and subsequent doses were given at weeks 2, 4 and then every other 8 weeks for a total period of one year. We treated ten patients with biopsy proven sarcoidosis, five men and five women, with a mean age of 47.1 years ranging from 28 to 63 years of age. Three patients had severe neurological symptoms, two had hepatic cirrhosis, one had granulomatous inflammation of the lachrymal gland and had already undergone repeated surgery, one had extensive pulmonary involvement (stage III), one had disfiguring lupus pernio and two presented disabling cutaneous nodules. Eight patients had more than one organ with evidence of disease. All patients were submitted to at least seven infusions of Infliximab. In four patients the dosage of corticosteroids or other immunosuppressive drugs was suspended, in three the dosage was reduced and in one, corticosteroids were added to the Infl iximab therapy. In five of the patients there was an important improvement. One of the patients with neurological symptoms displayed a complete recovery, while another had significant improvement of vision deficit enabling her to read again. Two patients withdrew from therapy, one due to lack of improvement of neurological symptoms and the other due to the onset of organizing pneumonia spawned by Infl iximab. Two patients developed anti-histone antibodies during treatment.Infl iximab seems effective in treating patients who are either resistant or develop side effects to a standard regimen of corticosteroids and immunosuppressive agents. These patients, treated with Infl iximab, should be under tight surveillance in order to quickly identify possible secondary effects.

Infliximab no tratamento da sarcoidose - Experiência de um hospital central

Despite aggressive treatment, sarcoidosis may be debilitating and progressive. The role of tumor necrosis factor (TNF)-α in the genesis of granulomas is ambiguous. It has proven to be critical in the formation and maintenance of granulomatous inflammation and its antagonist, Infliximab, has therefore been used with success in the treatment of patients with sarcoidosis. There are, however, reports of onset of sarcoidosis in patients in treatment for other conditions and that had no outbursts before submission to this therapy. We used Infliximab in the treatment of patients with sarcoidosis who either didn’t respond to corticosteroids and other conventional drugs or developed unacceptable side effects to these drugs. The initial dose was 5 mg/Kg body weight and subsequent doses were given at weeks 2, 4 and then every other 8 weeks for a total period of one year. We treated ten patients with biopsy proven sarcoidosis, five men and five women, with a mean age of 47.1 years ranging from 28 to 63 years of age. Three patients had severe neurological symptoms, two had hepatic cirrhosis, one had granulomatous inflammation of the lachrymal gland and had already been submitted to many surgeries, one had extensive pulmonary involvement (stage III), one had disfiguring lupus pernio and two presented disabling cutaneous nodules. In four patients the dosage of corticosteroids or other immunosuppressive drugs was suspended, in three the dosage was reduced and in one, corticosteroids were added to the Infliximab therapy. In five of the patients there was a significant improvement. One of the patients with neurological symptoms displayed a complete recovery, while another had significant improvement of vision deficit enabling her to read again. Two patients withdrew from therapy, one due to lack of improvement of neurological symptoms and the other due to the onset of organizing pneumonia spawned by Infliximab. Two patients developed anti-histone antibodies during treatment. Infliximab seems effective in treating patients who are either refractory or develop side effects to a standard regimen of corticosteroids and immunosuppressive agents. These patients, treated with Infliximab, should be under tight surveillance in order to quickly identify possible secondary effects.

Traitement de la sarcoïdose

Sarcoidosis is a common disorder with heterogeneous severity. Corticosteroids are the cornerstone of its treatment and allow a disease remission but only with a suspensive effect. Immunosuppressive drugs, hydroxychloroquine and infliximab may be useful in some patients. Half of the patients recover spontaneously without any treatment. In the remaining patients, a treatment is necessary either at presentation or during the follow-up in the presence of a disease flare. Treatment duration should be of at least 12months. The main indications of a systemic treatment include ophthalmologic, neurologic, cardiovascular, renal, laryngeal involvements, severe pulmonary manifestations, lupus pernio and marked hypercalcaemia. Initially, patients should receive prednisone or prednisolone at 0.5to 1mg/kg daily for 6to 12weeks to obtain a complete remission, and then followed by a gradual dose reduction every 6to 12weeks. After treatment completion, a 36-month duration monitoring is warranted to confirm recovery. Low-dose methotrexate and azathioprine are the most useful immunosuppressive drugs and are indicated in patients with of failure or contra-indication of corticosteroids or in those patients who needs a long term prednisone dose higher than 10mg daily to control the sarcoidosis. Cyclophosphamide used is severe neurologic or heart involvement that is resistant to corticosteroids and other immunosuppressive drugs. Hydroxychloroquine and chloroquine are indicated in extensive skin lesions or as corticosteroids sparing agents. Infliximab can be useful in some severe sarcoidosis patients refractory to classical treatment, except for cardiac involvement. Topical corticosteroids can be used for limited skin involvement, anterior uveitis or cough. In the future, a more individualized treatment could be drawn from pharmacogenetic studies. The key point remains the availability of etiologic or innovative drugs for sarcoidosis refractory to currently available therapy.

Lupus pernio. Presentación de una serie de 8 pacientes

Objectives Although lupus pernio (LP) is the most characteristic cutaneous lesion of chronic sarcoidosis, only a few cases have been reported in our country. The aim of this study was to review the frequency and clinical characteristics of patients with LP in a large series of patients with sarcoidosis. Methods A retrospective review of the frequency and characteristics of patients diagnosed as having LP from the series of sarcoidosis of our institution for a period of 35 years was performed. Results Eight (1.6%) out of 507 patients with sarcoidosis were diagnosed of LP. Mean age was 42 years. In 6 patients, LP was the presentation form of sarcoidosis. Five patients had involvement of the nasal skin and one patient severe involvement of the nasal mucosa. All the patients were treated with antimalarial drugs, 4 with oral corticosteroids, 2 with laser therapy, or with combinations with other drugs. None of the patient having nasal skin involvement showed remission of LP. Conclusions LP is a rare clinical form of sarcoidosis in our country. No treatment is effective for nasal skin involvement. The recent introduction of infliximab may represent an advance in the treatment of LP.

Lupus pernio with multiple bone cysts in the fingers

A 32-year-old Japanese man presented with a 3-year history of purple reddish, and painful swelling of his fingers along with indurated erythema on his nose and ears. He was diagnosed as having sarcoidosis 8 years prior because of uveitis and bilateral hilar lymphadenopathy. X-rays of the hands revealed multiple cystic lesions in the phalanges. Histological examination of the ear revealed epithelioid cell granulomas in the dermis. Oral prednisolone 20 mg/day improved his finger swelling and pain; however, his finger deformities and erythema remain unchanged. Bone involvement is sometimes seen in sarcoidosis and the hands are the most frequently affected areas. The frequency of bone involvement is higher in lupus pernio in comparison with other types of skin sarcoidosis. Systemic corticosteroids could be the first choice of treatment to relieve the symptoms.

Cutaneous Sarcoidosis

Sarcoidosis is a systemic disease with skin manifestations. Skin manifestations are classified as nonspecific if they are not characterized by granulomatous inflammation and specific if the lesions have granulomas histologically. Erythema nodosum is the most common nonspecific skin manifestation, and it portends a good prognosis. Specific skin lesions have a varied clinical appearance, although often they can be distinguished by their yellow translucent character. Despite the potential variable appearance, there are common clinical presentations. Lupus pernio lesions are nodular violaceous specific skin lesions found predominantly on the face associated with scarring and a poor prognosis. Treatment of cutaneous sarcoidosis is primarily done to avoid scarring and cosmetic disfigurement. Local and systemic corticosteroids are the mainstay of treatment for the disease. Corticosteroid-sparing agents used to manage the disease include antimalarials, methotrexate, and tetracycline antibiotics. Tumor necrosis factor-alpha (TNF-alpha) antagonists such as infliximab may have a role in cutaneous sarcoidosis, especially in refractory cases that are resistant to the standard regimens.

Specific (granulomatous) oral lesions of sarcoidosis ; Report of two cases

Sarcoidosis is a multisystemic granulomatous disease of unknown aetiology that usually presents with bilateral hiliar adenopathies, pulmonary infiltrations, and ocular and cutaneous lesions. The specific cutaneous lesions, or granulomatous, are produced in from 9% to 37% of patients with systemic sarcoidosis. Nevertheless, few patients have been described having specific lesions from sarcoidosis in the oral mucous membrane. We present 2 patients with systemic sarcoidosis who developed specific sarcoidosis lesions in the oral mucous membrane. The first patient presented a plaque on the tongue of a chronic nature associated with facial lupus pernio. The second patient presented a rapidly growing nodular lesion on the lower lip that was in fact the presentation sign of the disease. Although infrequent, oral lesions may constitute the first sign of systemic sarcoidosis. For this reason the oral mucous membrane needs to be explored when there is suspicion of sarcoidosis, and systemic sarcoidosis must be included in the differential diagnosis of oral granulomatous lesions.

DISSEMINATED STAGE 2 SARCOIDOSIS WITH LUPUS PERNIO AND ICHTHYOSIS PRESENTING WITH INTRASCROTAL MASSES

INTRODUCTION: Sarcoidosis is a multisystem disorder characterized by the formation of noncaseating granulomas (NCGs) in virtually any organ. Scrotal sarcoidosis is an extremely rare manifestation and usually appears with disseminated disease. We report a case of an unusual presentation of disseminated Stage 2 sarcoidosis with histologically proven bilateral intrascrotal involvement, along with classic pulmonary, lymphoid, and dermatological manifestations. The cutaneous findings observed include the specific lupus pernio and the unusual ichthyosis.

A retrospective analysis of 34 patients with cutaneous sarcoidosis assessed in a dermatology department

Sarcoidosis is a multisystem granulomatous disease of unknown aetiology, which most often involves the lungs and lymphatic system. Cutaneous involvement is found in approximately 25% of cases of sarcoid. Most previous studies of cutaneous sarcoidosis have been drawn from populations with defined pulmonary disease, so may represent a population with more systemic involvement. We describe a cohort of patients with cutaneous sarcoid seen in a dermatology department. Case records were reviewed for patients with a histopathological diagnosis of noncaseating sarcoidal granuloma on skin biopsy, taken between 1996 and 2005. In total, 34 patient records were analysed; 21 patients were found to have extracutaneous systemic sarcoid and 10 patients had sarcoid localized to the skin. Patients with lupus pernio and with ulcerated sarcoid lesions all had extracutaneous disease. No other cutaneous features, including the extent of cutaneous disease, were found to be predictive of systemic involvement. All patients presenting to a dermatology department with cutaneous sarcoidal granulomas require investigation for systemic sarcoid. Our data suggest that approximately 30% of patients seen in a dermatology clinic with cutaneous sarcoidal granulomas will have disease apparently limited to the skin.

Cutaneous sarcoid with varied morphology associated with hypercalcaemia and renal impairment

Sarcoidosis is a multisystem disorder of unknown aetiology, which presents with hilar lymphadenopathy, pulmonary infiltration, and ocular and cutaneous involvement. Cutaneous lesions often present as erythema nodosum, maculopapular, plaque, scar, subcutaneous nodule or lupus pernio. Most patients with cutaneous involvement have a single type of skin lesion, but some cases may have > or = 2 types. We report a case of sarcoidosis presenting with various types of skin lesions. The case was also complicated by hypercalcaemia and renal dysfunction, and was successfully treated with oral corticosteroids. Presentation of various skin lesions may indicate systemic organ involvement requiring treatment with systemic corticosteroid.

Improving cosmesis of lupus pernio by excision and forehead flap reconstruction

Sarcoidosis is a granulomatous multiorgan disease that frequently affects the skin. Lupus pernio (LP) is a cutaneous presentation resulting in violaceous discolouration and swelling of the nose. We report a severe case resistant to conventional treatment, which ultimately underwent nasal resurfacing by forehead flap rhinoplasty. Flap reconstruction has not previously been reported for deforming LP. We review the long-term outcome and advocate consideration of plastic surgery input in severe disease resistant to medical treatments.

The Treatment of Lupus Pernio: Results of 116 Treatment Courses in 54 Patients

Lupus pernio is a disfiguring sarcoidosis skin lesion that is difficult to treat and often causes a major psychosocial impact that may adversely affect the patient's quality of life. We reviewed the treatment outcome of 54 patients with lupus pernio who received 116 individual courses of treatment in our sarcoidosis clinic. Lupus pernio patients were identified from an institution-approved database. All patients were assessed at each clinic visit with facial photographs. By examining the photographs, the percentage of face involved (< 10%, 10 to 25%, > 25 to 50%, > 50%) was determined as was the effect of therapy (resolution, near resolution, improvement, no change, worsening). Medications included infliximab-containing regimens; systemic corticosteroids; noninfliximab, noncorticosteroid agents; and corticosteroids plus noncorticosteroid agents. In terms of achieving resolution or near resolution, infliximab regimens were superior to all others (infliximab, 77%; corticosteroids plus noncorticosteroids, 29%; corticosteroids, 20%; noncorticosteroids, 11%; infliximab vs other therapies: corticosteroids plus noncorticosteroids, p = 0.0015; corticosteroids, p = 0.0005; noncorticosteroids, p = 0.0002). The percentage of facial involvement also improved most with infliximab. Evaluating a secondary analysis of achieving resolution, near resolution, or improvement, infliximab (92%) was superior to noncorticosteroids (20%; p < 0.0001) and corticosteroids plus noncorticosteroids (56%; p = 0.0098), but not corticosteroids (72%; p = 0.2456); and noncorticosteroid agents were inferior to all other regimens. Infliximab appears superior to systemic corticosteroids with or without additional agents for the treatment of lupus pernio. Noninfliximab, noncorticosteroid-containing regimens are of limited use for this condition.

Cutaneous Nasal Nodule; a warning sign for Sinonasal Sarcoidosis

Sinonasal sarcoidosis can be associated with cutaneous sarcoid of the facial skin (lupus pernio). On this occasion we are reporting a case of lupus pernio with sinonasal involvement. A literature review is included, regarding the nature of sarcoidosis, (with special focus on the sinonasal involvement and lupus pernio), the clinical presentations, histological appearance and treatment modalities.

Infliximab Is the Most Effective Treatment for Lupus Pernio

The term lupus pernio was coined by Besnier to describe potentially scarring facial lesions of sarcoidosis that often occur in conjunction with

LUPUS PERNIO ON EAR LOBE THAT OCCURED AFTER TRAUMA

In the present 35 years old male patient, there were lupus pernio plaque in the right ear lobe developed after blunt trauma and radiologically typical sarcoidosis findings. In histopatholigical examination, clusters of epithelioid cells scattered irregularly through the dermis were observed. The patient responded well to systemic and topical steroid therapy.

Ulcerative Sarcoidosis: Case Report and Review of the Japanese Literature

,Sarcoidosis is a chronic multi-system disease of unknown origin involving the eyes, skin, lungs, heart, bones and nerves. Skin lesions are relatively common, and are clas-sified as either specific or non-specific (1). Specific le-sions are those that show the typical sarcoid granulomas, including lupus pernio, infiltrated plaques, maculopapular eruptions, subcutaneous nodules and infiltration of old scars (1). The most typical non-specific skin lesion is erythema nodosum. The patient described here had skin lesions that first suggested a diagnosis of erythema indu-ratum of Bazin, but we ultimately diagnosed sarcoidosis based on other findings, including uveitis, bilateral hilar lymphadenopathy, an increased level of serum angio-tensin-converting enzymes (ACE) and a negative response to purified protein derivative of tuberculin (PPD).CASE REPORT