35: Sarcoidosis Flashcards
What is sarcoidosis and how is it characterized?
Sarcoidosis is a multisystem disease characterized by granulomatous inflammation of unknown etiology, commonly affecting the lungs and skin, but it can involve any organ system.
What are the common clinical features of sarcoidosis?
Common clinical features of sarcoidosis include:
- Lupus pernio: Symmetric, violaceous, indurated plaques and nodules on the nose, earlobes, cheeks, and digits.
- Mikulicz syndrome: Sarcoidal granulomas on mucosal surfaces, particularly in the lacrimal, parotid, sublingual, and submandibular glands.
- Darier-Roussy sarcoid: Persistent subcutaneous nodules that may be tender or painless, often occurring on extremities.
- Lofgren syndrome: Erythema nodosum with bilateral hilar adenopathy, arthralgia, and fever.
- Heerfordt syndrome: Fever, parotid gland enlargement, facial palsy, and anterior uveitis.
What are the epidemiological characteristics of sarcoidosis?
Epidemiological characteristics of sarcoidosis include:
- Occurs worldwide and affects all ages and races.
- Most common onset in the third decade of life, slightly higher in women.
- Higher incidence with greater distance from the equator.
- Highest prevalence found in whites in Denmark and Sweden, and in persons of African descent in the United States.
What types of cutaneous findings are associated with sarcoidosis?
Cutaneous findings in sarcoidosis include:
- Various morphologies such as macules, papules, patches, plaques, and nodules.
- Alopecia with scalp involvement and nail changes.
- Epidermal changes like scaling, telangiectasia, and atrophy.
- Lesions can occur before, coincident with, or after systemic involvement.
- Lesions are classified into specific (granulomatous inflammation) and nonspecific (reactive without sarcoidal granulomas).
What is the most common presentation of specific cutaneous lesions in sarcoidosis?
The most common presentation of specific cutaneous lesions in sarcoidosis is papules and plaques, with the papular form being the typical manifestation.
What are the two categories of cutaneous lesions in sarcoidosis?
Cutaneous lesions in sarcoidosis are divided into specific lesions, which have granulomatous inflammation histologically similar to other organs, and nonspecific lesions, which are reactive and do not exhibit sarcoidal granulomas.
What is the significance of the ‘apple jelly’ appearance in granulomatous lesions?
The ‘apple jelly’ appearance in granulomatous lesions is significant as it is reminiscent of the color change observed in these lesions. It is assessed by pressing a glass slide against the skin (diascopy), which accentuates the yellow-brown coloration. This appearance is not pathognomonic for sarcoidosis, as similar properties can be seen in other conditions like lupus vulgaris and granuloma annulare.
What dermoscopic findings are associated with cutaneous sarcoidosis?
Dermoscopic findings associated with cutaneous sarcoidosis include:
- Pink homogenous background
- Translucent orange areas
- Fine white scaling
- Scar-like depigmentation
These findings are significantly associated with cutaneous sarcoidosis when compared to necrobiosis lipoidica, granuloma annulare, and rheumatoid nodules.
What are the common characteristics of papular lesions in cutaneous sarcoidosis?
Common characteristics of papular lesions in cutaneous sarcoidosis include:
- Waxy appearance reflecting mild epidermal atrophy
- Location: Most commonly on the face and neck, particularly periorbital skin
- Symptoms: Typically asymptomatic without pain or pruritus
- Main complaint: Cosmetic disfigurement
- Color: May retain translucent yellow-brown coloration.
What is lupus pernio and what are its clinical features?
Lupus pernio is characterized by:
- Relatively symmetric, violaceous, indurated plaques and nodules
- Common locations: Nose, earlobes, cheeks, and digits
- Misnomer: The term ‘lupus pernio’ is preserved from the mistaken belief that these lesions are related to lupus vulgaris and cold injury.
- Potential complications: Lesions may directly extend into the nasal sinus, leading to epistaxis, nasal crusting, and possible bone involvement.
What is the most common presentation of specific cutaneous lesions in sarcoidosis?
The most common presentation of specific cutaneous lesions in sarcoidosis is the papular form, which often appears as firm, translucent red-brown or yellow-brown papules.
What diagnostic technique can be used to accentuate the yellow-brown coloration of sarcoidosis lesions?
Diascopy, which involves pressing a glass slide to the skin, can accentuate the yellow-brown coloration of sarcoidosis lesions, giving them an ‘apple jelly’ appearance.
What are angiolupoid lesions and where do they typically occur?
Angiolupoid lesions are pink and violaceous papules and plaques with prominent telangiectasias that usually occur on the face. They are often considered a variant of lupus pernio.
What is Scar Sarcoid and where does it typically occur?
Scar Sarcoid is a form of cutaneous sarcoidosis that occurs preferentially within scar tissue, at traumatized skin sites, and around embedded foreign material such as silica and tattoo ink.
What are the clinical features of mucous membrane involvement in sarcoidosis?
Sarcoidal granulomas may cause papules and plaques on the mucosal surface and tongue, leading to conditions such as ‘straw-berry gums’ which mimic Wegner granulomatosis.
What is erythema nodosum and its significance in sarcoidosis?
Erythema nodosum is the main nonspecific cutaneous manifestation of sarcoidosis, often presenting with bilateral hilar adenopathy, arthralgia, and fever, and is frequently the initial manifestation of sarcoidosis (Lofgren syndrome).
What are the pulmonary implications of sarcoidosis?
The lung is the most common organ involved with sarcoidosis, with findings on pulmonary examination usually absent. Patients may experience dyspnea, cough, chest pain, hypoxemia, and wheezing. Sarcoid lesions can lead to pulmonary fibrosis, pulmonary hypertension, and chronic Aspergillus infection.
What is the condition called when a patient presents with violaceous, indurated plaques on the nose and cheeks?
This condition is called lupus pernio. It is associated with upper respiratory tract involvement, pulmonary fibrosis, and bony cysts.
What is the term for the manifestation that occurs preferentially within scar tissue in sarcoidosis?
This manifestation is called scar sarcoid, which occurs preferentially within scar tissue and may be the only finding in a patient with significant systemic involvement.
What is the term for the strawberry-like gingival lesions in sarcoidosis?
This manifestation is called ‘strawberry gums’ and can mimic Wegener granulomatosis.
What are the potential complications of granulomatous inflammation in the lungs?
Granulomatous inflammation in the lungs can lead to pulmonary fibrosis, pulmonary hypertension, and chronic Aspergillus infection.
What is the most common symptom of pulmonary sarcoidosis?
The most common symptom of pulmonary sarcoidosis is dyspnea, although patients are often asymptomatic.
What is the term for lesions that occur preferentially within scar tissue in sarcoidosis?
Lesions that occur preferentially within scar tissue are called scar sarcoid.
What is the term for the relatively symmetric, violaceous, indurated plaques and nodules occurring on the nose and cheeks?
These lesions are called lupus pernio.