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Sarcoidosis - Hair and Scalp

See also in: Overview,External and Internal Eye,Oral Mucosal Lesion
Contributors: Haya Raef MD, Jeffrey M. Cohen MD, Paritosh Prasad MD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Sarcoidosis is an immune-mediated systemic disorder typified by granuloma formation in involved organs, most commonly of the lung parenchyma and the skin. The inciting immune activating agent remains unknown (hypotheses include autoimmune, infectious, and environmental triggers). There are genetic predispositions related to components of the inflammatory response. Family history of the disease increases the risk. The disease affects all ages and races and ethnicities, with peak incidence at age 30-50 in men and 50-60 in women. It is slightly more common in women. Cutaneous disease is often the first sign of systemic sarcoidosis.

Sarcoidosis commonly presents with abnormalities identified incidentally on chest radiography. Although the disease can affect different organs, systemic symptoms such as fever, night sweats, and weight loss are common. Sarcoidosis can affect the lungs, with symptoms such as chest pain, shortness of breath, cough, and most commonly, pronounced fatigue. It can also affect the peripheral lymph nodes, heart, kidneys, gastrointestinal tract, central nervous system (CNS), liver, spleen, bone, muscle, and endocrine glands. Venous thromboembolism and pulmonary hypertension are potential complications of sarcoidosis. Approximately 90% of patients will have lung involvement. Pulmonary fibrosis and bronchiolectasis result in "honeycombing" of the lung and represent end-stage lung disease due to chronic granulomatous inflammation. Hilar lymphadenopathy is asymptomatic and affects 90% of patients. Approximately 10% of patients have hypercalcemia.

Neurosarcoidosis commonly manifests as cranial-nerve deficits. Any part of the neurologic system can be impacted by sarcoidosis; a vast range of neurologic signs and symptoms may be present. Delays in diagnosis and treatment can lead to permanent disability. The diagnostic workup for neurosarcoidosis should include an MRI of the head, cerebrospinal fluid analysis, and detection of sarcoidosis outside the nervous system.

Approximately 25% of patients will have cutaneous involvement, and some patients may have skin-limited disease. Asymptomatic red-brown dermal papules and/or plaques that favor the face, neck, upper extremities, and upper trunk are the most common specific cutaneous findings. Less common manifestations include sarcoid lesions with epidermal change such as scale (ichthyosiform sarcoid), hypopigmentation, subcutaneous nodules, and ulceration. Sarcoidosis has a predilection for scars and may be seen within tattoos.

Sarcoidosis of the scalp is a rarely reported cutaneous finding. Both nonscarring and scarring alopecia may occur in association. Scarring plaques are usually localized, and nonscarring alopecia may be localized or generalized. Underlying plaques may be red-brown, violaceous, and/or hypopigmented. They may be raised or depressed and atrophic. Scaling can be seen, and scalp involvement can resemble seborrheic dermatitis. An annular morphology has been reported. Most reported cases have been seen in African American women. Scalp sarcoid has been correlated with the presence of systemic involvement. Scalp sarcoid can be recalcitrant to treatment.

The pathogenesis of sarcoidosis is poorly understood. However, it is characterized by noncaseating epithelioid granulomas made up mostly of CD4+ helper T-cells, a predominantly Th1 type immune response, and elevated levels of interferon (IFN)-gamma and interleukin (IL)-2.

Codes

ICD10CM:
D86.3 – Sarcoidosis of skin

SNOMEDCT:
31541009 – Sarcoidosis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Sarcoidosis is a diagnosis of exclusion, on a clinical and histologic level. Clinically, sarcoid papules, nodules, and plaques are not unique, and a histologic evaluation is often required. The histologic differential is broad, and detailed histologic findings are beyond the scope of this text. Special stains for acid-fast and fungal organisms and tissue cultures should be obtained when necessary.

The following is a clinical differential of sarcoidosis of the scalp:

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed:07/21/2020
Last Updated:11/10/2022
Copyright © 2022 VisualDx®. All rights reserved.
Sarcoidosis - Hair and Scalp
See also in: Overview,External and Internal Eye,Oral Mucosal Lesion
A medical illustration showing key findings of Sarcoidosis (Common Skin Lesions) : Face, Neck, Arms
Clinical image of Sarcoidosis - imageId=192732. Click to open in gallery.  caption: 'A close-up of a cluster of smooth reddish-brown papules arranged in an annulus.'
A close-up of a cluster of smooth reddish-brown papules arranged in an annulus.
Copyright © 2022 VisualDx®. All rights reserved.