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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 of the lung parenchyma and the skin, but it can affect many other organs. The inciting immune activating agent remains unknown (hypotheses include autoimmune, infectious, and environmental triggers). The disease affects all ages and ethnicities and both sexes, with peak incidence demonstrating a bimodal age distribution: ages 25-35 and 45-65. It is most commonly observed 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, peripheral lymph nodes, heart, kidneys, gastrointestinal tract, central nervous system (CNS), liver, spleen, bone, muscle, and endocrine glands. 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.

Approximately 25% of patients will have cutaneous involvement and, commonly, many patients 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:07/28/2020
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Sarcoidosis - Hair and Scalp
See also in: Overview,External and Internal Eye,Oral Mucosal Lesion
Sarcoidosis (Common Skin Lesions) : Face, Neck, Arms
Clinical image of Sarcoidosis
A close-up of a cluster of smooth reddish-brown papules arranged in an annulus.
Copyright © 2021 VisualDx®. All rights reserved.