The Evolving Landscape of Cutaneous Sarcoidosis

Pathogenic Insight, Clinical Challenges, and New Frontiers in Therapy

Julie H. Wu; Sotonye Imadojemu; Avrom S. Caplan

Disclosures

Am J Clin Dermatol. 2022;23(4):499-514. 

In This Article

Abstract and Introduction

Abstract

Sarcoidosis is a multisystem disorder of unknown etiology characterized by accumulation of granulomas in affected tissue. Cutaneous manifestations are among the most common extrapulmonary manifestations in sarcoidosis and can lead to disfiguring disease requiring chronic therapy. In many patients, skin disease may be the first recognized manifestation of sarcoidosis, necessitating a thorough evaluation for systemic involvement. Although the precise etiology of sarcoidosis and the pathogenic mechanisms leading to granuloma formation, persistence, or resolution remain unclear, recent research has led to significant advances in our understanding of this disease. This article reviews recent advances in epidemiology, sarcoidosis clinical assessment with a focus on the dermatologist's role, disease pathogenesis, and new therapies in use and under investigation for cutaneous and systemic sarcoidosis.

Introduction

Sarcoidosis is a multisystem disease characterized by non-caseating granulomas accumulating in affected tissues. Granulomas in sarcoidosis can evolve in any organ system, leading to progressive organ damage, chronic dysfunction, fibrosis and scarring.[1] Cutaneous manifestations are among the most common disease manifestations in sarcoidosis, occurring in approximately 30% of cases.[2] Cutaneous involvement is frequently present at diagnosis or is the initial presenting symptom, thus highlighting the importance of clinician recognition of the disease for prompt diagnosis and initiation of appropriate therapies.[3] The clinical presentation and long-term progression of sarcoidosis can be highly variable and necessitates a comprehensive systemic workup at the outset. Dermatologist recognition of sarcoidosis and the protean manifestations of this disease is therefore essential.

The clinical manifestations, diagnostic approach, and therapeutic options for cutaneous sarcoidosis have been detailed in a prior review published in this journal in 2014.[4] Here, we provide an overview and an update for dermatologists to increase awareness of sarcoidosis and highlight the evolving understanding of this disease, focusing on updates in pathogenesis, diagnostic challenges, novel therapies, and therapeutic targets.

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