Lupus pernio (Besnier-Tenneson syndrome): A rare form of sarcoidosis

Abstract

Lupus pernio (LP) is a chronic non-life threatining type of cutaneous sarcoidosis that can be related to chronic fibrotic sarcoidosis, hyperglobulinemia and hypercalcemia. The aim of this case report is to evaluate the clinical and demographic features of cutaneous sarcoidosis mainly presenting with a rare manifestation of LP. In this paper we report a case of systemic sarcoidosis presenting with LP and a review of the available literature. LP is a rare presentation with infiltrated erythematoviolaceous plaques affecting the nose. We address the main management approach, and possible association with an underlying systemic sarcoidosis. LP is a rare but chronic manifestation of systemic sarcoidosis that needs to be treated in order to prevent cosmetic defects and psychological effects. It is important to recognize such a condition early in order to avoid a delay in treatment and worsening of the condition, both physically and psychologically. Further research regarding the diagnostic approach and management is required to understand this condition thoroughly.

Lupus Pernio in Chronic Sarcoidosis

“…The diagnosis of sarcoidosis is based on physical examination, chest X-ray, and serological tests such as angiotensin-converting enzyme, as it is elevated in 60% of cases of active sarcoidosis and hypercalcemia [ 10 ]. Biopsy of clinically suspicious lesions is the most reliable and accurate tool [ 11 ]. Many treatment options are available in the literature on the treatment of sarcoidosis, but few randomized trials are available dealing with the various treatment options for skin manifestations [ 11 ].…”

Cutaneous Manifestations of “Lupus”: Systemic Lupus Erythematosus and Beyond

“…Treatment of lupus pernio can be challenging given the unpredictable course of disease. Therapeutic options include local, intralesional, and, if needed, systemic corticosteroids, as well as methotrexate, chloroquine, hydroxychloroquine, azathioprine, cyclophosphamide, thalidomide, infliximab, and even laser therapy [144,[146][147][148][149][150]. Lenalidomide has been reported to be successful in one refractory case of lupus pernio [151].…”

“…These lesions can be complicated by nasal ulceration and septal perforation, which can be aggravated further by surgical intervention [ 144 ]. Lupus pernio rarely resolves spontaneously and can result in facial disfigurement as well as nasal obstruction or fibrotic pulmonary complications if the nasal cavity and maxillary sinuses become more extensively involved [ 146 ].…”

“…Histopathology of these lesions demonstrates changes that can be found in all organs affected by sarcoidosis: noncaseating granulomas with a sparse lymphocytic component referred to as “naked granulomas” [ 146 ]. These granulomas are typically found in the dermis but can be subcutaneous.…”

Clinical Presentations, Pathogenesis, and Therapy of Sarcoidosis: State of the Art

“…With the exception of erythema nodosum, which consists of a panniculitis and does not contain granulomas, skin lesions are generally florid areas of granulomatous inflammation, and the diagnosis of sarcoidosis can be readily made by skin biopsy. Erythema nodosum may accompany acute presentations of the disease, is more common in women, and tends to be associated with a favorable prognosis, while manifestations such as lupus pernio [6], often described in African Americans and Puerto Ricans, are associated with chronic disease and worse prognosis.…”