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

Background. 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. Aim. To analyse the relationship between the clinical type of granulomatous cutaneous lesions and the systemic features and prognosis of systemic sarcoidosis. Methods. 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. Results. 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. Conclusions. Cutaneous granulomatous lesions are usually present at the diagnosis of systemic sarcoidosis, and the type of cutaneous involvement may have prognostic significance.     

Subcutaneous sarcoidosis--clinicopathological study of 10 cases

BACKGROUND: Subcutaneous sarcoidosis is a specific cutaneous lesion of sarcoidosis that is rarely reported. OBJECTIVE: Our purpose was to analyse the clinicopathological features of 10 patients with subcutaneous sarcoidosis and its relationship with the systemic features of the disease. PATIENTS AND METHODS: The patients with systemic sarcoidosis, diagnosed from 1974 to 2002 at a university hospital in Barcelona, Spain, who developed subcutaneous involvement, were included in the study. The diagnosis of systemic sarcoidosis was made according to conventional criteria. All the patients were monitored prospectively at the sarcoidosis clinic of the hospital. Skin biopsies were performed when granulomatous cutaneous involvement was suspected clinically. RESULTS: Granulomatous cutaneous involvement was demonstrated in 85 of 480 patients with systemic sarcoidosis. In 10 of these 85 patients subcutaneous sarcoidosis was diagnosed (11.8%). The lesions were most frequently located in the extremities, involving the forearms in nine patients. Indurated linear bands from the elbow to the hand were observed in five patients. In all of our patients the subcutaneous nodules appeared at the beginning of the disease. In six patients, the nodules remitted spontaneously in less than 2 years. In two cases foreign particles were detected under polarized light. CONCLUSIONS: Subcutaneous sarcoidosis is a quite uniform clinicopathological entity usually appearing at the beginning of the disease. It usually heralds forms of sarcoidosis with nonsevere systemic involvement and is not associated with chronic fibrotic disease.     

Cutaneous sarcoidosis in black South Africans

BACKGROUND: It has been observed that, in the USA, sarcoidosis is more common in African-Americans than in other races. It has also been noted that sarcoidosis in African-Americans is characterized by more severe extrapulmonary involvement and more exuberant skin lesions. There is little information on sarcoidosis in black Africans. METHODS: Fifty-four black South African patients with cutaneous lesions of sarcoidosis proven by biopsy were prospectively studied. Dermatologic and ophthalmologic examinations and chest X-rays were performed in all patients. Other investigations relevant in the diagnosis of extracutaneous sarcoidosis were also performed in a variable number of patients. RESULTS: In 40 patients (71%), systemic sarcoidosis was found with lung, eye, and acral bone involvement being most common. Great variations in the morphology of skin lesions were observed. In one-quarter of patients, atypical cutaneous lesions (hypopigmented, ichthyosiform, lymphedematous, mutilating, ulcerative, verrucous) were found. Lupus pernio, once thought to be confined to Northern Europe, was observed in five patients in the subtropical milieu of South African Transvaal. Sarcoidal dactylitis with nail changes was seen in eight patients. Fibrinoid necrosis was found in 12% of the biopsies. CONCLUSIONS: Sarcoidosis in black South Africans is characterized by extensive cutaneous involvement. The lesions are morphologically extremely variable, frequently atypical, and often demonstrate fibrinoid necrosis on histology.     

Endobronchial involvement in a patient presenting with cutaneous sarcoid

Half of all patients with cutaneous sarcoidosis will develop pulmonary disease. We report a case of cutaneous and endobronchial sarcoidosis and describe a scheme for identifying pulmonary involvement in patients with cutaneous sarcoid.     

Cutaneous sarcoidosis with cardiac involvement.

Despite the clinical importance of cardiac involvement in the prognosis of sarcoidosis, it is often overlooked because of the subclinical disease progression and difficulty in diagnosis. We report here five patients with cutaneous sarcoidosis lesions where cardiac involvement was detected with the appearance of mild cardiac symptoms on a careful examination of the heart after cutaneous sarcoidosis was diagnosed. In four of the five cases, the patients had annular lesions while the fifth case showed a nodular eruption on the face. Three of the five patients showed complete AV block while one showed complete right bundle block and left bundle anterior branch block. All four patients with conduction disturbances underwent a permanent pacemaker implantation with prednisolone tapering therapy. The remaining patient had congestive heart failure and was treated with prednisolone alone. The present findings support the belief that sarcoidosis patients with cutaneous lesions, especially facial annular lesions, should be carefully examined and monitored for cardiac involvement, even in cases without apparent cardiac symptoms.     

Mutilating facial sarcoidosis

We report a patient with massive facial sarcoidosis. While skin involvement is a common manifestation of sarcoidosis, it is unusual to see it in the dramatic form of cutaneous tumors with mutilation of the central face. There are few reports of tumoral cutaneous sarcoidosis like that of our patient.     

Subcutaneous sarcoidosis in a melanoma scar

Cutaneous sarcoidosis often has been referred to. as the great imitator because skin lesions can present with various morphologies. Skin lesions may be the only site of involvement or may accompany systemic disease. Occasionally, sarcoidosis also may infiltrate scars from prior trauma, tattoos, or surgery. We report a case of subcutaneous sarcoidosis limited to a melanoma scar without any other cutaneous or systemic involvement. Familiarity with and proper diagnosis of cutaneous sarcoidosis can allow for appropriate systemic screening and timely management of the disease.     

Foreign bodies in granulomatous cutaneous lesions of patients with systemic sarcoidosis

OBJECTIVE: To assess the presence of foreign material in the granulomatous cutaneous lesions of patients with systemic sarcoidosis. DESIGN AND SETTING: Observational study reevaluating histological specimens at a university referral hospital. PATIENTS: Sixty-five patients diagnosed as having sarcoidosis who developed granulomatous cutaneous involvement. MAIN OUTCOME MEASURES: To detect the presence of polarizable foreign particles in cutaneous biopsy specimens and to evaluate the association with clinical features of the patients. RESULTS: Granulomatous cutaneous involvement was demonstrated in 65 (15.3%) of 425 patients with systemic sarcoidosis. In 14 (22%) of the 65 patients, the cutaneous biopsy specimen showed foreign particles in polarized light. The skin lesions corresponded to 3 different clinical patterns: an admixture of papules and infiltration of previously undetected minute scars (n = 6); scar sarcoidosis (n = 4); and subcutaneous nodules (n = 4). The lesions were located most frequently in the extremities, involving the knees in 10 patients. CONCLUSIONS: The presence of polarizable foreign body material in granulomatous cutaneous lesions is not infrequent in patients with systemic sarcoidosis. Inoculation of foreign matter from a previous inapparent minor trauma may induce granuloma formation in individuals with sarcoidosis.     

Cutaneous sarcoidosis: clinico–epidemiological profile of 72 patients at a tertiary hospital in São Paulo,Brazil

BackgroundSarcoidosis is a multisystem disease of unknown cause that is characterized by the presence of granulomas in various organs. Cutaneous involvement is common and the reported incidence has varied from 9% to 37%. Studies on cutaneous sarcoidosis in Brazil are lacking.ObjectivesTo describe the clinical and epidemiological aspects of patients with cutaneous sarcoidosis diagnosed at the Department of Dermatology of the University of São Paulo, from May 1994 to March 2018.MethodsClinical data of patients with confirmed cutaneous sarcoidosis were retrospectively reviewed and classified according to gender, ethnicity, age at diagnosis, cutaneous presentation, systemic involvement and treatment.ResultsCutaneous sarcoidosis was diagnosed in 72 patients with a female predominance (74%). The mean age at diagnosis was 49.6 years and most of the patients were white (61%). Papules and plaques were the most common lesions. Systemic sarcoidosis was detected in 81% of patients, affecting mainly the lungs and thoracic lymph nodes (97%). Typically, cutaneous lesions were the first manifestation (74%). Systemic therapy was necessary for 72% of patients; the dermatologist managed many of these cases. Oral glucocorticoids were the most commonly used systemic medication (92%). The mean number of systemic drugs used was 1.98 per patient.LimitationsInsufficient data in medical records.ConclusionsThis series highlights the dermatologist role in recognizing and diagnosing cutaneous sarcoidosis, evaluating patients for systemic disease involvement and treating the skin manifestations. Cutaneous sarcoidosis was once considered exceedingly infrequent in Brazil in comparison to infectious granulomatous diseases; however, the present series seems to suggest that the disease is not so rare in this region.     

Seasonal cutaneous sarcoidosis: a photo-induced variant

Sarcoidosis is a multisystem granulomatous disease, with cutaneous involvement in up to 35% of cases. Owing to its heterogeneous clinical presentation, sarcoidosis is often referred to as the 'great imitator' of dermatological disease. A rare variant of photosensitive cutaneous sarcoidosis has been infrequently reported in the literature. We describe an unusual case of recurrent, photo-distributed cutaneous sarcoidosis presenting only during the summer months.     

Cutaneous lesions as initial signs of interferon α-induced sarcoidosis: report of three new cases and review of the literature

Sarcoid reactions are well-recognized adverse events during interferon (IFN) therapy. They are frequently underdiagnosed because misinterpreted as IFN-induced side effects. Sarcoid cutaneous lesions may therefore represent useful hints to an early diagnosis, but their incidence is unknown. We report three new cases of mono-localized, purely cutaneous IFNα-induced sarcoidosis. In addition, an extensive review of the literature, with special attention to skin involvement, was performed through a PubMed search. The analysis of the retrieved articles showed that cutaneous lesions are frequent signs of IFN-induced sarcoidosis. Skin involvement is documented in 56% of the reports and it appears among the presenting and diagnostic signs of a sarcoid reaction in 51%. Special attention to dermatologic signs is imperative in the course of IFN therapy because even minimal skin involvement may offer a clue to an early diagnosis of IFN-induced sarcoidosis.     

Sarcoidosis subcutánea

—Subcutaneous sarcoidosis is characterized by mobile, painless, limb nodules composed of sarcoidal granulomas within fatty tissue, without epidermal involvement. Although cutaneous involvement is found in about a quarter of patients with sarcoidosis, subcutaneous nodules, which may occur in association with other cutaneous lesions, are rare. Less than 40 cases of subcutaneous sarcoidosis have been published the literature.We here report the case of a 63 year-old woman who presented deep, nodular and subcutaneous plaques in extremities for the last 15 days. Histopathology of the cutaneous lesions revealed noncaseating granulomas in subcutaneous tissue, consistent with subcutaneous sarcoidosis. Special stains failed to show foreign material, fungi or acid-fast organism. Thoracic lymphadenopathia were noted in a chest X-ray. Treatment with steroids during 2 months induced an excellent response.     

Sarcoidosis del cuero cabelludo

—Approximately 25 % of patients with sarcoidosis show skin involvement. Although this usually accompanies systemic involvement, it may be the only location. Sarcoidosis of the scalp is a rare form of cutaneous sarcoidosis, and it does not often present as its only manifestation. Most cases have been described in African-American women. We discuss a case of sarcoidosis of the scalp, which presented as cicatricial alopecia, as the only cutaneous manifestation in a Caucasian woman, two years before systemic involvement, and which adopted the morphology of localized cicatricial alopecia.     

Ocular sarcoidosis

Ocular involvement in sarcoidosis is the second most common manifestation of the disease, preceded only by pulmonary abnormalities with hilar adenopathy. Ocular disease is the presenting manifestation in 9% of cases,¹ and 25–50% of patients with systemic sarcoidosis will at some time show ocular involvement.^1–3 Chronic ocular sarcoidosis may correlate with cutaneous lesions in 38% of cases. All patients with suspected sarcoidosis should have a thorough ophthalmologic examination.     

Cutaneous sarcoidosis in southern Taiwan: clinicopathologic study of a series with high proportions of lesions confined to the face and angiolupoid variant

Background Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology. Our clinical experience suggests that facial involvement and the angiolupoid variant appear more common in our patients compared with series reported from the western countries. Objective To characterize the clinicopathologic features of cutaneous sarcoidosis diagnosed in our department and to compare our data with those in the literature. Methods We conducted a clinicopathologic review of biopsy‐proved cases of cutaneous sarcoidosis diagnosed during January 2002–December 2010. Results Our study consisted of 37 patients, ages 26–84 years (mean 54.3 years), of whom 84% were females. Systemic involvement was detected in 73%, affecting the lung in 57%, lymph nodes in 65% and eyes in 43%. Most skin lesions were the papulonodular type (70%) and confined to the face (54%). The angiolupoid variant, while rare in Europe and America, was the most common variant (38%) in our series and often associated with eye involvement. The histology was characterized by infiltration of naked sarcoidal granulomas, mostly (86%) mixed with variable amounts of tuberculoid granulomas in the dermis and/or the subcutis. Other findings included fibrinoid necrosis (23%), foreign bodies (16%), osteoclast‐like cells (14%) and granuloma annulare‐like and necrobiosis lipoidica‐like features. Conclusion The present series of cutaneous sarcoidosis was characterized by a marked female predominance and by high proportions of facial involvement and the angiolupoid variant. Angiolupoid sarcoidosis was often associated with eye involvement. A complete dermatologic examination and biopsy of suspicious skin lesions should be routinely performed to facilitate early diagnosis of sarcoidosis.     

Ichthyosiform sarcoidosis. Report of two cases and a review of the literature

Although acquired ichthyosis has been associated with a number of systemic illnesses, an association with sarcoidosis has rarely been reported. We report two patients with acquired ichthyosis of the lower extremities whose diagnosis of cutaneous sarcoidosis was established by histologic examination. Systemic involvement in both patients included ocular and pulmonary disease. A diagnosis of sarcoidosis must be considered when a patient presents with acquired ichthyosis.     

Nail dystrophy due to sarcoidosis

Nail involvement in sarcoidosis is uncommon. It may be a sign of chronic sarcoidosis even in the absence of other cutaneous signs in the affected digits, as illustrated by the following case.     

Scar sarcoidosis in childhood: case report and review of the literature

Sarcoidosis is a rare disease among children, manifesting differently in children below and above 4-5 years of age. Although the exact incidence and prevalence of childhood sarcoidosis is not known, the cutaneous involvement is frequent in both children and adults. Infiltration of old cutaneous scars with sarcoid granuloma in the active phase of disease, known as scar sarcoidosis is one of the uncommon cutaneous manifestations of sarcoidosis. We report a case of scar sarcoidosis in an 11-year-old child along with a brief review of the literature.     

Disseminated angiolupoid sarcoidosis

A case of a patient with a rare variant of cutaneous sarcoidosis, angiolupoid sarcoidosis, with pulmonary involvement and an unusual number of widely disseminated elements is presented. Disseminated angiolupoid sarcoidosis is not mentioned in major textbooks of dermatology.     

Micropapular cutaneous sarcoidosis: case series successfully managed with hydroxychloroquine sulfate

Micropapular lesions constitute a rare morphologic variety of cutaneous sarcoidosis. We report 3 patients with this unusual entity and highlight the universal suppressive response to oral hydroxychloroquine sulfate. Although ocular involvement has been found to be common in conjunction with micropapular cutaneous sarcoidosis, none of our patients had demonstrable eye disease.