Pulmonary Sarcoidosis - Cutaneous Presentation

2/06/2008

Question:  How often does pulmonary sarcoidosis present with cutaneous symptoms?

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<1>

Unique Identifier [PMID]: 16097511

Authors: Ahmad K. Powell FC.

Institution: Regional Dermatology Centre, Mater Misericordiae Hospital, Eccles Street, Dublin 7. kashif786@yahoo.co.uk

Title: Cutaneous sarcoidal reaction in a patient with pulmonary tuberculosis.

Source: Irish Medical Journal. 98(6):182, 2005 Jun.

Abstract: Sarcoidosis is a systemic disorder characterized by non-caseating epithelioid granulomas that may affect any organ system. The etiology of sarcoidosis is unknown, but several immune aberrations are thought to play a role in its pathogenesis. The possible role of mycobacterial infection in the pathogenesis of sarcoidosis has been suggested. In recent studies mycobacterial DNA was detected in lung tissues and bronchial lavage fluid of 30% to 50% of patients with sarcoidosis. We report a patient with pulmonary tuberculosis treated successfully three years earlier who developed cutaneous sarcoidosis with negative polymerase chain reaction (PCR) assay for mycobacterial DNA of the skin lesion.

Publication Type: Case Reports. Journal Article.

<2>

Unique Identifier [PMID]: 15758836

Authors: Ramos-Casals M. Mana J. Nardi N. Brito-Zeron P. Xaubet A. Sanchez-Tapias JM. Cervera R. Font J. HISPAMEC Study Group.

Institution: Department of Autoimmune Diseases, Hospital Clinic, Institut d'Investigacions Biomediques August Pi i Sunyer (IDIBAPS), School of Medicine, University of Barcelona, Barcelona, Spain. mramos@clinic.ub.es

Title: Sarcoidosis in patients with chronic hepatitis C virus infection: analysis of 68 cases. [Review] [67 refs]

Source: Medicine. 84(2):69-80, 2005 Mar.

Abstract: We describe the clinical characteristics, the patterns of association, and the role of antiviral therapies in patients with sarcoidosis associated with chronic hepatitis C virus (HCV) infection. Sixty-eight patients were included in the current study, 56 cases identified in the literature search plus 12 unpublished cases from our department. In 50 HCV patients, sarcoidosis appeared after starting antiviral therapy. Antiviral therapy associated with triggered sarcoidosis consisted of alpha-interferon monotherapy in 20 cases and combined therapy with alpha-interferon and ribavirin in 30. Sarcoidosis appeared during the first 6 months after starting therapy in 66% of patients. The clinical picture of sarcoidosis included predominantly pulmonary disease in 38 (76%) patients and cutaneous sarcoidosis in 30 (60%). Antiviral therapy was discontinued in 60% of patients and continued or adjusted in 14%, while sarcoidosis appeared after completed therapy in the remaining cases. Specific therapy for sarcoidosis was started in only 21 patients, mainly with oral corticosteroids. The outcome of patients was detailed in 46 cases: remission or improvement was observed in 38/46 (83%) patients, stabilization of sarcoidosis in 5/46 (11%), and reactivation of sarcoidosis after an initial improvement in 3/46 (6%). Finally, 18 treatment-naive HCV patients presented sarcoidosis, with 14/18 (87%) patients presenting with pulmonary involvement and 8/18 (44%) with cutaneous involvement.In summary, sarcoidosis may be observed in HCV patients in 2 different situations: triggered by antiviral therapy (in 75% of cases) and unrelated to treatment. Sarcoidosis during antiviral therapy may present mainly as cutaneous or pulmonary disease, with a benign, uncomplicated evolution in more than 85% of cases. However, more complicated cases are observed, especially in HCV patients with preexisting sarcoidosis and/or with previous antiviral treatment. Clinicians should be aware of the possibility that sarcoidosis may initially manifest or be reactivated during or shortly after treatment with antiviral therapy in patients with chronic HCV infection. [References: 67]

Publication Type: Journal Article. Review.

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Unique Identifier [PMID]: 15732434

Authors: Landers MC. Skokan M. Law S. Storrs FJ.

Institution: Department of Dermatology, Oregon Health & Science University, Portland 97239, USA.

Title: Cutaneous and pulmonary sarcoidosis in association with tattoos.

Source: Cutis. 75(1):44-8, 2005 Jan.

Abstract: Sarcoidosis encompasses a heterogeneous spectrum of clinical presentations, including sarcoidosis in association with tattoos. We report the development of cutaneous and pulmonary sarcoidosis in a patient with long-standing eyebrow tattoos whose cutaneous sarcoidosis almost completely resolved when treated with tacrolimus 0.1% ointment. A 70-year-old woman with a 3-year history of an erythematous eruption circumscribing her eyebrow tattoos presented with a chronic, nonproductive cough of 8 months' duration. Skin biopsy results demonstrated naked tubercles consistent with sarcoidosis. Results of radiographs and a computed tomography scan of the chest revealed multiple pulmonary nodules with mediastinal and hilar adenopathy. The results of transbronchial biopsy were consistent with the diagnosis of pulmonary sarcoidosis. Initial treatment with oral prednisone only improved the pulmonary sarcoidosis. The cutaneous sarcoidosis almost completely resolved after the addition of tacrolimus 0.1% ointment.

Publication Type: Case Reports. Journal Article.

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Unique Identifier [PMID]: 15125500

Authors: Yoo SS. Mimouni D. Nikolskaia OV. Kouba DJ. Sauder DN. Nousari CH.

Institution: Department of Dermatology, Division of Immunodermatology, Johns Hopkins University, Baltimore, Maryland, USA.

Title: Clinicopathologic features of ulcerative-atrophic sarcoidosis.

Source: International Journal of Dermatology. 43(2):108-12, 2004 Feb.

Abstract: BACKGROUND: Sarcoidosis is a chronic granulomatous disease of unknown etiology. Cutaneous disease is common and includes two clinicopathologic categories: granulomatous infiltration or a reactive phenomenon. In the granulomatous infiltrative group, clinical manifestations can be variable. Ulcers in sarcoidosis are uncommonly recognized and have been categorized previously under the rubric of atrophic, necrobiosis-like, or ulcerative sarcoidosis. PATIENTS AND METHODS: We evaluated retrospectively sarcoidosis patients presenting to the Johns Hopkins Department of Dermatology between June 1989 and May 2002. Multiple skin biopsies were performed for histopathologic evaluation. Investigation for extracutaneous manifestations, including routine serologic assays, chest radiography, pulmonary function tests, electrocardiogram, and angiotensin-converting enzyme level, and referral for ophthalmologic examination were performed in all patients. RESULTS: Of 147 consecutive patients presenting with cutaneous sarcoidosis, seven demonstrated ulcerative-atrophic sarcoidosis lesions. All patients were African-American (five females and two males). All patients had ulcers surrounded by atrophic necrobiosis lipoidica-like plaques on the pretibial areas. All patients had other mucocutaneous manifestations of sarcoidosis, with the majority having evidence of internal disease. Combined immunosuppressive and immunomodulatory therapy was effective in controlling the cutaneous manifestations of all patients with ulcerative sarcoidosis. CONCLUSIONS: The ulcerative variant is a poorly defined subset of cutaneous sarcoidosis. Trauma, superimposed on atrophic plaques, appears to be the principal mechanism of this rare variant of cutaneous sarcoidosis.

Publication Type: Case Reports. Journal Article.

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Unique Identifier [PMID]: 12102610

Authors: Lenner R. Schilero GJ. Padilla ML. Teirstein AS.

Institution: Department of Pulmonary and Critical Care Medicine, Mount Sinai-NYU Medical Center, New York, NY, USA. egandrobi@aol.com

Title: Sarcoidosis presenting in patients older than 50 years.

Source: Sarcoidosis Vasculitis & Diffuse Lung Diseases. 19(2):143-7, 2002 Jun.

Abstract: BACKGROUND: Sarcoidosis occurs most often between 20 and 40 years of age, but also presents in children and older adults. Newly diagnosed sarcoidosis in older patients has received little attention. In order to characterize sarcoidosis in older patients, the clinical, radiographic and laboratory features of sarcoidosis presenting in patients aged 50 or older were compared to patients whose sarcoidosis was diagnosed at an earlier age. METHODS: The medical records of 181 consecutive patients with sarcoidosis were reviewed. They were divided into 92 patients diagnosed at 50 years of age or older (group A), and 89 whose diagnosis preceded age 50 (group B). RESULTS: Comparison of group A with group B revealed that the two groups were similar with regard to race, gender, smoking habits, common presenting symptoms, organ system involvement, pulmonary function data, radiographic stage, PPD status, and laboratory values. At the time of diagnosis, most patients in both groups presented with either respiratory symptoms or asymptomatic chest roentgenogram abnormalities. The most prevalent pulmonary function abnormality was reduced diffusing capacity in both groups. Most patients exhibited either stage I or II chest roentgenograms. Organ systems most commonly involved included lung, lymph nodes, and skin. CONCLUSION: Sarcoidosis presents with similar clinical features whether diagnosed in young adults or in patients over the age of 50. The diagnosis of sarcoidosis should be considered in patients presenting over age 50 with characteristic signs and symptoms including chest radiographic evidence of mediastinal lymphadenopathy.

Publication Type: Comparative Study. Journal Article.

<6>

Unique Identifier [PMID]: 11734441

Authors: Baughman RP. Teirstein AS. Judson MA. Rossman MD. Yeager H Jr. Bresnitz EA. DePalo L. Hunninghake G. Iannuzzi MC. Johns CJ. McLennan G. Moller DR. Newman LS. Rabin DL. Rose C. Rybicki B. Weinberger SE. Terrin ML. Knatterud GL. Cherniak R. Case Control Etiologic Study of Sarcoidosis (ACCESS) research group.

Institution: University of Cincinnati Medical Center, Cincinnati, Ohio 45267-0565, USA. Bob.Baughman@uc.edu

Title: Clinical characteristics of patients in a case control study of sarcoidosis.[see comment].

Source: American Journal of Respiratory & Critical Care Medicine. 164(10 Pt 1):1885-9, 2001 Nov 15.

Abstract: Sarcoidosis may be affected by sex, race, and age. A Case Control Etiologic Study of Sarcoidosis (ACCESS) enrolled 736 patients with sarcoidosis within 6 mo of diagnosis from 10 clinical centers in the United States. Using the ACCESS sarcoidosis assessment system, we determined organ involvement for the whole group and for subgroups differentiated by sex, race, and age (less than 40 yr or 40 yr and older). The study population was heterogeneous in terms of race (53% white, 44% black), sex (64% female, 36% male), and age (46% < 40 yr old, 54% > or = 40 yr old). Women were more likely to have eye and neurologic involvement (chi(2) = 4.74, p < 0.05 and chi(2) = 4.60, p < 0.05 respectively), have erythema nodosum (chi(2) = 7.28, p < 0.01), and to be age 40 yr or over (chi(2) = 6.07, p < 0.02) whereas men were more likely to be hypercalcemic (chi(2) = 7.38, p < 0.01). Black subjects were more likely to have skin involvement other than erythema nodosum (chi(2) = 5.47, p < 0.05), and eye (chi(2) = 13.8, p < 0.0001), liver (chi(2) = 23.3, p < 0.0001), bone marrow (chi(2) = 18.8, p < 0.001), and extrathoracic lymph node involvement (chi(2) = 7.21, p < 0.01). We conclude that the initial presentation of sarcoidosis is related to sex, race, and age.

Publication Type: Comparative Study. Journal Article. Research Support, U.S. Gov't, P.H.S..

<7>

Unique Identifier [PMID]: 3227185

Authors: Stadnyk AN. Rubinstein I. Grossman RF. Baum GL. Hiss Y. Solomon A. Rosenthal T.

Institution: Mount Sinai Hospital, University of Toronto, Canada.

Title: Clinical features of sarcoidosis in elderly patients.

Source: Sarcoidosis. 5(2):121-3, 1988 Sep.

Abstract: We reviewed 17 cases of biopsy-proven sarcoidosis occurring in patients who were 65 years of age or older at the time of diagnosis in order to determine the clinical features of sarcoidosis in this group of elderly patients. A female preponderance (76%) was noted, as well as significant respiratory and cutaneous symptoms. There were no characteristic diagnostic features of sarcoidosis in this group; therefore, we suggest that tissue biopsy is needed to exclude pulmonary diseases such as neoplasm or tuberculosis. Most elderly patients having sarcoidosis remained clinically stable during the period of follow-up and did not require therapy for sarcoidosis. We propose that sarcoidosis should be considered in the differential diagnosis of elderly patients presenting with pulmonary or multisystem disease.

Publication Type: Journal Article.

<8>

Unique Identifier [PMID]: 3589506

Authors: Munro CS. Campbell DA. Du Bois RM. Mitchell DN. Cole PJ. Poulter LW.

Title: Dendritic cells in cutaneous, lymph node and pulmonary lesions of sarcoidosis.

Source: Scandinavian Journal of Immunology. 25(5):461-7, 1987 May.

Abstract: To determine whether dendritic cells (DC) are a consistent feature of lesions of sarcoidosis, we have used monoclonal antibodies to identify the HLA-DR-expressing populations of cells in cryostat sections of 15 lymph node, pulmonary and cutaneous lesions. The commonest HLA-DR positive cells in granulomas were epithelioid and giant cells, although lymphocytes within granulomas and tissue macrophages around them were also positive. Dendritic cells with Langerhans cells (NA1/34+ = OKT6+) and interdigitating cell (RFD1+) phenotype were consistently associated with granulomas only in skin lesions. In lymph nodes, interdigitating cells (NA1/34-/RFD1+/HLA-DR++) were confined to paracortical zones as in normal nodes, although a small area of NA1/34+/RFD1+ cells was found in one of three nodes. In lung lesions NA1/34+/RFD1+ dendritic cells were uncommon or absent, except in one chronic case. We conclude that while sometimes present in extracutaneous sites, DC are not an essential feature of sarcoid lesions, and that cells of the classical macrophage group are the most significant HLA-DR-expressing population. We suggest that the presence of DC in lesions of sarcoidosis may indicate an immunological response distinct from that causing granulomas to form. The variability of their involvement may have immunoregulatory significance.

Publication Type: Journal Article. Research Support, Non-U.S. Gov't.

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Unique Identifier [PMID]: 3556705

Authors: Gupta AK. Haberman HF. From GL. Lipa M.

Title: Sarcoidosis with extensive cutaneous ulceration. Unusual clinical presentation.

Source: Dermatologica. 174(3):135-9, 1987.

Abstract: A 70-year-old white woman with sarcoidosis and insulin-resistant diabetes mellitus presented with extensive cutaneous ulcerations. Both the cutaneous lesions and the systemic features of sarcoidosis showed a dramatic improvement during oral corticosteroid therapy. When extensive cutaneous ulcerations are present, it is important to consider sarcoidosis, as these may be the only presenting sign of the disease. Unlike ulcerated necrobiosis lipoidica diabeticorum, sarcoidal ulcerations may respond well to treatment with oral corticosteroids.

Publication Type: Case Reports. Journal Article.

<10>

Unique Identifier [PMID]: 3453863

Authors: Suskovic T.

Title: [Scarring of the skin--an indicator in the diagnosis of pulmonary sarcoidosis]. [Croatian]

Source: Lijecnicki Vjesnik. 108(9):372-5, 1986 Sep.

Publication Type: Case Reports. English Abstract. Journal Article.

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Unique Identifier [PMID]: 7239755

Authors: Bower JS.

Title: Pulmonary evaluation of patients presenting with dermatological manifestations of sarcoidosis.

Source: International Journal of Dermatology. 20(5):385-9, 1981 Jun.

Publication Type: Journal Article.

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Unique Identifier [PMID]: 456024

Authors: Herzlinger DC. Marland AM. Barr RJ.

Title: Verrucous ulcerative skin lesions in sarcoidosis. An unusual clinical presentation.

Source: Cutis. 23(5):569-72, 1979 May.

Abstract: A twenty-seven year old man with a two year history of sarcoidosis and systemic manifestations of this disease along with verrucous ulcerative skin lesions is presented. Biopsy specimens of the cutaneous lesions showed typical noncaseating granulomas as well as necrotizing granulomatous inflammation. After other possible etiologies were excluded, prednisone therapy was instituted with prompt resolution of the skin lesions, leaving atrophic scars.

Publication Type: Case Reports. Journal Article.

<13>

Unique Identifier [PMID]: 1119835

Authors: Fong YW. Sharma OP.

Title: Pruritic maculopapular skin lesions in sarcoidosis. An unusual clinical presentation.

Source: Archives of Dermatology. 111(3):362-4, 1975 Mar.

Abstract: Pruritic maculopapular rash occurred in a 23-year-old black man. The lesion remained undiagnosed and required frequent hospital admissions. Twenty years after the onset of symptoms, the skin and lymph node biopsy specimens showed noncaseating granulomas. Sarcoidosis should always be included in the differential diagnosis of pruritic skin lesion.

Publication Type: Journal Article.

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Unique Identifier [PMID]: 13761725

Authors: LEWIS JG.

Title: The evolution of sarcoidosis into caseating tuberculosis of the lungs and skin.

Source: Tubercle. 42:95-100, 1961 Mar.

Publication Type: Case Reports. Journal Article.