*3.1.1.2 Nonspecific lesions of sarcoid*

Erythema nodosum, the most common nonspecific lesion, develops in 20–25% of sarcoidosis cases [6, 49]. Frequently, it is the initial manifestation of disease. It is the marker of acute and benign sarcoidosis and tends to affect younger people than infiltrative cutaneous lesions [12]. Sarcoidosis is the second most common cause of erythema nodosum [50]. If sarcoid is associated with EN, it usually runs a benign and self-limited course [51]. Women are affected three to six times more frequently than men [52]. EN can occur in all age groups, but it is typically seen between the second and fourth decades of life. The higher prevalence of erythema nodosum among young people is considered to be due to the higher incidence of sarcoidosis in this age group [11]. EN is characterized by sudden onset of symmetric, tender, erythematous, warm nodules and raised plaques, usually located on the shins, ankles, and knees (**Figure 12**). Nodules are from 1 to 5 cm or more in diameter and

**77**

half of the cases [56].

**Figure 12.** *Erythema nodosum.*

*Clinical Manifestations of Sarcoidosis and Granulomatous Disorders*

distributed bilaterally. Nodules may become confluent, resulting in erythematous plaques. In rare instances, more extensive lesions may appear, involving the thighs, extensor aspects of the arms, the neck, and even the face. Initially the nodules are bright red in color; within a few days, they become flat, with a livid red or purplish color and finally a yellow or greenish appearance, often taking on the look of a deep bruise (erythema contusiformis). This contusiformis color evolution is quite characteristic of erythema nodosum and allows a specific diagnosis in late-stage lesions [53]. Ulceration is never seen in erythema nodosum, and the nodules heal without atrophy or scarring [54]. Usually, acute bouts of erythema nodosum are associated with a fever of 38–39°C, fatigue, malaise, arthralgia, headache, abdominal pain, vomiting, cough, or diarrhea. Episcleral lesions and phlyctenular conjunctivitis may also accompany the cutaneous lesions. Less frequent clinical manifestations associated with erythema nodosum are lymphadenopathy, hepatomegaly, splenomegaly, and pleuritis [55]. Eruption generally lasts from 3 to 6 weeks, but persistence beyond this time is not unusual. Recurrences are not uncommon. Erythema nodosum in children has a much shorter duration than in adults, arthralgias are seen in a minority of the patients, and fever is an accompanying manifestation in fewer than

Another nonspecific lesion of sarcoid is Lofgren's syndrome, an acute form of sarcoidosis characterized by erythema nodosum, bilateral hilar lymphadenopathy (BHL), and symmetric polyarthritis [57]. Arthritis in sarcoidosis is usually symmetrical; the ankles are involved in more than 90% of the cases; the knees, small joints of the hands or feet, wrists, and elbows are involved in 15–40% [58–60]. Local pain, soft-tissue swelling, periarticular tenderness, edema, and joint effusion may be present [61]. In 1953, Lofgren characterized 212 adult patients of bilateral hilar lymphadenopathy who were practically regarded as having sarcoidosis based on the absence of tuberculosis. Lofgren demonstrated that EN was present at the onset of

*DOI: http://dx.doi.org/10.5772/intechopen.92236*

**Figure 12.** *Erythema nodosum.*

distributed bilaterally. Nodules may become confluent, resulting in erythematous plaques. In rare instances, more extensive lesions may appear, involving the thighs, extensor aspects of the arms, the neck, and even the face. Initially the nodules are bright red in color; within a few days, they become flat, with a livid red or purplish color and finally a yellow or greenish appearance, often taking on the look of a deep bruise (erythema contusiformis). This contusiformis color evolution is quite characteristic of erythema nodosum and allows a specific diagnosis in late-stage lesions [53]. Ulceration is never seen in erythema nodosum, and the nodules heal without atrophy or scarring [54]. Usually, acute bouts of erythema nodosum are associated with a fever of 38–39°C, fatigue, malaise, arthralgia, headache, abdominal pain, vomiting, cough, or diarrhea. Episcleral lesions and phlyctenular conjunctivitis may also accompany the cutaneous lesions. Less frequent clinical manifestations associated with erythema nodosum are lymphadenopathy, hepatomegaly, splenomegaly, and pleuritis [55]. Eruption generally lasts from 3 to 6 weeks, but persistence beyond this time is not unusual. Recurrences are not uncommon. Erythema nodosum in children has a much shorter duration than in adults, arthralgias are seen in a minority of the patients, and fever is an accompanying manifestation in fewer than half of the cases [56].

Another nonspecific lesion of sarcoid is Lofgren's syndrome, an acute form of sarcoidosis characterized by erythema nodosum, bilateral hilar lymphadenopathy (BHL), and symmetric polyarthritis [57]. Arthritis in sarcoidosis is usually symmetrical; the ankles are involved in more than 90% of the cases; the knees, small joints of the hands or feet, wrists, and elbows are involved in 15–40% [58–60]. Local pain, soft-tissue swelling, periarticular tenderness, edema, and joint effusion may be present [61]. In 1953, Lofgren characterized 212 adult patients of bilateral hilar lymphadenopathy who were practically regarded as having sarcoidosis based on the absence of tuberculosis. Lofgren demonstrated that EN was present at the onset of

the disease in 113 cases in which articular symptoms were common (101 cases, 89%). There was either pain only in the joints (20%) or pain accompanied by swelling (69%) [62]. Lofgren's syndrome was regarded as a self-limiting disease that is generally resolved within the first year, with the mean duration ranging from 3 weeks to 3.7 months [57, 60]. However, 8% of patients had active symptoms 2 years after the onset; 6% had episodes of recurrent sarcoidosis 2.20 years after the diagnosis [62].
