Cutaneous sarcoidosis: clinico–epidemiological profile of 72 patients at a tertiary hospital in São Paulo, Brazil

Marcella Karen Souza Da Costa,Marcello Menta Simonsen Nico,Mariana Fernandes Torquato

doi:10.1016/j.abd.2019.06.004

Marcella Karen Souza Da Costa, Marcello Menta Simonsen Nico + Show 1 more

Open Access

https://doi.org/10.1016/j.abd.2019.06.004

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Abstract

BackgroundSarcoidosis is a multisystem disease of unknown cause that is characterized by the presence of granulomas in various organs. Cutaneous involvement is common and the reported incidence has varied from 9% to 37%. Studies on cutaneous sarcoidosis in Brazil are lacking. ObjectivesTo describe the clinical and epidemiological aspects of patients with cutaneous sarcoidosis diagnosed at the Department of Dermatology of the University of São Paulo, from May 1994 to March 2018. MethodsClinical data of patients with confirmed cutaneous sarcoidosis were retrospectively reviewed and classified according to gender, ethnicity, age at diagnosis, cutaneous presentation, systemic involvement and treatment. ResultsCutaneous sarcoidosis was diagnosed in 72 patients with a female predominance (74%). The mean age at diagnosis was 49.6 years and most of the patients were white (61%). Papules and plaques were the most common lesions. Systemic sarcoidosis was detected in 81% of patients, affecting mainly the lungs and thoracic lymph nodes (97%). Typically, cutaneous lesions were the first manifestation (74%). Systemic therapy was necessary for 72% of patients; the dermatologist managed many of these cases. Oral glucocorticoids were the most commonly used systemic medication (92%). The mean number of systemic drugs used was 1.98 per patient. LimitationsInsufficient data in medical records. ConclusionsThis series highlights the dermatologist role in recognizing and diagnosing cutaneous sarcoidosis, evaluating patients for systemic disease involvement and treating the skin manifestations. Cutaneous sarcoidosis was once considered exceedingly infrequent in Brazil in comparison to infectious granulomatous diseases; however, the present series seems to suggest that the disease is not so rare in this region.

Highlights

MethodsSarcoidosis is a multisystem disease of unknown cause that is characterized by the presence of granulomas in various organs
Skin manifestations can be classified into two categories: specific lesions have histopathological evidence of typical sarcoid granulomas; nonspecific lesions develop as a result of an inflammatory reaction pattern.2,4---9
For patients with isolated cutaneous sarcoidosis or patients with the severe cutaneous disease, a stepwise approach is recommended with local therapies, immunomodulators, and systemic immunosuppressants.[12]

Summary

Introduction

MethodsSarcoidosis is a multisystem disease of unknown cause that is characterized by the presence of granulomas in various organs. Studies show that genetic susceptibility and environmental factors contribute to the development of an exaggerated immune response.[1] The clinical course varies and, in half of the cases, the disease resolves spontaneously within two to five years. Remission is much less likely.[2] Lungs and thoracic lymph nodes are the most commonly affected organs in more than 90% of cases, but nearly every other organ can be affected.[3]. Skin manifestations can be classified into two categories: specific lesions have histopathological evidence of typical sarcoid granulomas; nonspecific lesions develop as a result of an inflammatory reaction pattern.2,4---9. Methods: Clinical data of patients with confirmed cutaneous sarcoidosis were retrospectively reviewed and classified according to gender, ethnicity, age at diagnosis, cutaneous presentation, systemic involvement and treatment. Systemic sarcoidosis was detected in 81% of patients, affecting mainly the lungs and thoracic lymph nodes (97%).

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