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Plate 4-69 Integumentary System

CUTANEOUS MANIFESTATIONS OF SARCOID

Skin
lesions
SARCOID

Sarcoid, or sarcoidosis, is a relatively common con-
dition that can affect many organ systems. There is a
wide spectrum of disease activity, from localized skin
disease to widespread involvement of the integu-
mentary, pulmonary, cardiac, renal, gastrointestinal, Bilateral
ophthalmic, endocrine, neurological, and lymphatic parotid gland
systems. However, most cases are mild in nature and involvement
can be controlled with proper care. Although an infec-
tious etiology has often been theorized, no conclusive Lacrimal
evidence has been established. This idiopathic condi- gland involve-
tion can produce multiple skin findings. The skin find- ment
ings should cause the attending physician to look for
systemic involvement.
Clinical Findings: Sarcoidosis can occur in any
ethnic population, but it is seen at a higher rate in
African Americans. It also has a higher incidence in
women. The usual age at onset is before 40 years. Up
to 90% of patients with sarcoid have a benign clinical
course with no increased mortality. Sarcoidosis has
been reported to occur in a familial form, which has led
researchers to look for specific genetic defects that Paralysis due
could explain the disease. However, sarcoid remains an to involvement
idiopathic multisystem disease. There are many distinct of facial
clinical expressions of the disease that are common (VII) nerve
enough that they have been named, including Löfgren’s
syndrome, lupus pernio, Darier-Roussy sarcoid, Bone destruction
Heerfordt syndrome, and Mikulicz syndrome. of terminal
Sarcoid can affect the skin in a multitude of ways. phalanges
There are both specific and nonspecific skin findings.
The most common nonspecific skin finding is erythema
nodosum. Erythema nodosum affects the lower ante-
rior extremities. It manifests as tender subcutaneous
nodules or plaques. Examination of biopsy specimens
shows a nonspecific form of panniculitis. The etiology
of erythema nodosum in patients with sarcoid is poorly
understood.
The lesions of sarcoid that occur within the integu-
mentary system are quite varied. The most common
specific skin lesion is a slightly brownish to red-brown
papule, plaque, or nodule with varying amounts of Biopsy of nodule.
hyperpigmentation. Sarcoid is a mimicker of many Typical sarcoidal
other conditions, especially in its skin lesions. Macular granuloma (dense
lesions, ulcerations, subcutaneous nodules, annular infiltration with
plaques, ichthyosiform erythroderma, and alopecia macrophages,
have all been described as potential presentations epithelioid cells,
of sarcoid. and occasional
The extracutaneous organ system most commonly multinucleated
involved is the pulmonary system. There is a relatively giant cells [arrow])
straightforward classification that describes the stages
of pulmonary sarcoid based on radiographic findings.
The higher the radiographic stage, the more severe the Positive Kveim test. Intracutaneous injection
disease. Isolated bilateral hilar adenopathy is the most of saline suspension of human sarcoidal
common pulmonary finding, and it is the basis for stage spleen or lymph nodes causes appearance
I radiographic disease. These patients are most com- of erythematous nodule in 2 to 6 weeks.
monly asymptomatic, and the adenopathy is found on
routine radiographic testing. Any findings of pulmonary
sarcoid should prompt referral of the affected individual erythrocyte sedimentation rate is uniformly elevated. nose and the rest of the face. This form of sarcoid is
to a pulmonologist for pulmonary function testing. For some unknown reason, this syndrome is most com- quite resistant to therapy, runs a more prolonged
Löfgren’s syndrome is defined by the acute onset monly seen in young Caucasian women. This form of course, and is often difficult to treat. The skin findings
of erythema nodosum, almost exclusively in young sarcoidosis typically resolves spontaneously within 2 to are typically shiny brown-red plaques, papules, and
adult women; it is seen in association with fever, 3 years. nodules overlying the nose and other regions of the
bilateral hilar adenopathy, and uveitis. Other, non- Lupus pernio is the name given to the clinical find- face. The involvement can become so severe as to cause
specific constitutional signs are often present. The ings of specific cutaneous sarcoid involvement of the disfigurement of the nose by shiny red-brown papules

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