Sarcoidosis

A 54-year-old man presented with symmetric, bulbous, nodular enlargement of the nasal tip and alae nasi. At first inspection, the changes appeared to be consistent with rhinophyma, but a biopsy specimen showed noncaseating granulomata that were consistent with sarcoidosis. His laboratory, radiographic, and pulmonary function studies were consistent with systemic involvement.     

Sarcoidosis

Sarcoidosis is a systemic noncaseating granulomatous disorder of unknown origin. The cutaneous manifestations of sarcoidosis often enable the dermatologist to be the first physician to make the diagnosis. This article reviews essential sarcoidosis pathophysiology, clinical polymorphisms, systemic evaluation, and treatment modalities for cutaneous sarcoidosis to further enhance the dermatologist's understanding of this disease entity. Learning objective: At the conclusion of this learning activity, participants should be familiar with the theories of the pathogenesis of sarcoidosis, its cutaneous manifestations, its various syndromes and associations, and its presentation in children. Participants should also be more knowledgeable about diagnostic evaluation, measurement of disease progression, treatment modalities, and the prognosis and mortality data of sarcoidosis.     

Sarcoidosis

Sarcoidosis is characterized by noncaseating granuloma that can occur in any organ of the body. The lymph nodes, lungs, skin, liver, spleen, phalangeal bones, parotid glands, and eyes are the most common sites of involvement. Although sarcoidosis rarely causes death, its course is highly variable, and its treatment can be challenging to the clinician. This article presents an updated review of the clinical manifestations of sarcoidosis as well as discussions of the diagnostic evaluation and treatment of this idiopathic disease.     

Sarcoidosis

Sarcoidosis is a systemic granulomatous disease of unknown cause that most commonly affects the lungs, lymph nodes, skin, eyes, spleen, bone, and glandular tissue. The diagnosis is made when characteristic histologic findings are demonstrated and other granulomatous processes are excluded. It can be an acute or chronic debilitating disease, but in patients with acute disease the process can be self-limited. Multiple therapeutic options have been described both for the cutaneous and systemic lesions of sarcoidosis. Steroids, however, remain the cornerstone of therapy.     

Sarcoidosis

A 48-year-old woman with an 8-year history of intermittently pruritic papules on the lower extremities suddenly developed pruritus and generalized spread of the lesions over a 1-month period. The lesions appeared as smooth, flat-topped, violaceous, round papules on all extremities, trunk, and back. A biopsy specimen showed sarcoidosis. Cutaneous sarcoidosis has many morphologic presentations and often mimics other dermatologic diseases. Despite widespread cutaneous involvement, she has no systemic involvement. Treatment options are reviewed.     

Sarcoidosis cicatrizal

—Skin involvement in the form of scar sarcoidosis represents one of the most unusual forms of skin sarcoidosis. We present the case of a 56-year-old woman with changes in old scars as the onset of systemic sarcoidosis. The patient presented violaceous infiltration in some of her old, previously stable scars. Through a histopathological study, epithelioid-cell granulomas were observed in the superficial dermis and part of the reticular dermis. The lesions progressed, involving healthy skin as well, coinciding with the development of bilateral anterior uveitis. The symptoms were controlled with topical ophthalmic corticosteroids and chloroquine. The pathogenesis of scar sarcoidosis seems to be attributable to a hypersensitivity reaction in which the macrophage previously stimulated by an inert substance contaminating the injury is reactivated by sarcoidosis, thus infiltrating these old scars.     

Cutaneous Necrotizing Sarcoidosis

A case of cutaneous myoepithelioma is reported. A 37 year‐old woman presented with a 2 cm well‐circumscribed dermal nodule of the forehead. The tumor was enucleated with the clinical diagnosis of an epidermal inclusion cyst. Histologically, the tumor was located within the dermis and was well circumscribed. It was composed of spindle‐shaped and epithelioid cells arranged in organoid nests. Focal areas of extracellular hyalinized stroma were present. Well‐defined glandular structures, chondroid matrix, and significant nuclear pleomorphism were absent. The tumor expressed widespread cytoplasmic positivity for cytokeratin (using AE1/AE3) and nuclear positivity for p63. Variable reactivity was noted with EMA and S‐100. The tumor was negative for smooth muscle actin, GFAP, chromogranin, synaptophysin, and CEA. These immunohistochemical results supported myoepithelial differentiation. Myoepitheliomas of the skin are rare neoplasms that have only recently been recognized in the skin. A single publication has reported the diagnostic utility of p63 in the diagnosis of myoepithelial tumors of the skin. Myoepitheliomas often display variable expression of myoepithelial markers, with no single marker that is 100% sensitive. The current case highlights the need for a battery of markers, including p63, to detect myoepithelial differentiation.     

Sarcoidosis mimicking lipodermatosclerosis

The clinical presentation of cutaneous sarcoidosis is highly variable. Rare presentations include ulcerated plaques, morpheaform lesions, and unilateral lower extremity edema. We report the case of a woman who presented with unilateral ulcerating sarcoidosis of the lower leg with progressive fibrosis and edema mimicking lipodermatosclerosis. This case is unique in that the patient exhibited all 3 of the rare manifestations of sarcoidosis; to our knowledge, this presentation has not been previously reported in the literature.     

Sarcoidosis in Children

Sarcoidosis is a multisystem disease of unknown etiology that is rarely diagnosed in children. When mass screening is performed, the incidence of the disease in children approaches that of adults with similar demographics. Most childhood cases occur around ages 9 to 15 years, with small clusters of cases occurring in children under age 4 years. The disease in these two age groups has very different clinical features. Children under age 4 have a clinical triad of rash, arthritis, and uveitis. The classic syndrome in older children involves primarily lungs, lymph nodes, and eyes. In older children, constitutional symptoms (fatigue, lethargy, malaise) and pulmonary symptoms (cough, dyspnea) predominate. Mortality in childhood sarcoidosis is about 5%, with long-term sequelae in 10% to 20%. Early recognition may prevent complications such as blindness, pulmonary insufficiency, and renal impairment.     

Sarcoidosis simulating syringomas

Sarcoidosis is a granulomatous disease of unknown etiology. The skin is commonly affected. Cutaneous manifestations can mimic other diseases and autoimmune disorders. The dermatologist plays a critical role in elucidating the clinical diagnosis and assisting other specialists in the investigation of a systemic disease. We report a patient with typical cutaneous manifestation of sarcoidosis with pulmonary involvement.     

Sarcoidosis and malignancy

Malignancy in patients with sarcoidosis occurs in 3 settings. The first setting relates to patients with hematologic malignancies. It includes the sarcoidosis-lymphoma syndrome, which refers to the development of lymphoma at least 1 to 2 years after the diagnosis of sarcoidosis. It also includes patients with sarcoidosis who develop other hematologic malignancies. In addition, this subset of individuals includes patients with cancer and hematologic malignancies who subsequently develop sarcoidosis. The second setting consists of patients with sarcoidosis who develop solid tumors and oncologic patients in whom sarcoidosis subsequently appears; in addition to melanoma and nonmelanoma skin cancer, the neoplasms most commonly associated involve the cervix, liver, lung, testicles, and uterus. The third setting of malignancy-related sarcoidosis occurs when sarcoidosis presents as a paraneoplastic syndrome for the associated cancer, specifically when the discovery of cancer is concurrent with or within 1 year of the diagnosis of sarcoidosis or vis-a-vis. Antineoplastic treatment of either the hematologic malignancy or the solid tumor has also been observed to either induce the initial onset or flare the activity of sarcoidosis. Malignancy can also be associated with the occurrence of sarcoid reactions that typically are restricted to the regional lymph nodes or the visceral organ of tumor origin; rarely, the sarcoid reaction can also be observed in the skin or is only limited to the skin.     

Cutaneous Sarcoidosis Clinically Mimicking Necrobiosis Lipoidica in a Patient with Systemic Sarcoidosis

A 70-year-old woman with an 8-year history of systemic sarcoidosis developed round, red-brown eruptions, with central atrophic lesions on her lower legs. The features of the biopsy specimen resembled those of necrobiosis lipoidica (NL), but although necrobiosis was present there were well-formed non-necrotizing granulomas in the dermis. The histological diagnosis was cutaneous sarcoidosis. Systemic sarcoidosis presenting with NL has rarely been reported. The histological features of cutaneous sarcoidosis sometimes mimic those of other granulomatous diseases, including NL and granuloma annulare, which are difficult to distinguish. We discuss the novel association between sarcoidosis and other granulomatous diseases.