aMiriam L. Vega MD, MPH; bJennifer Abrahams, MD; aMatthew Keller, MD
aThomas Jefferson University, Philadelphia, Pennsylvania; bUniversity of Miami Department of Dermatology, Miami, Florida
Disclosure: The authors report no relevant conflicts of interest.
Abstract
Background: Psoriasiform lesions are an established, but rare, manifestation of sarcoidosis. Only 0.9 percent of patients with cutaneous sarcoidosis develop this form of the disease. Observation: The authors present a case of a 61-year-old woman with a history of pulmonary sarcoidosis who presented to their dermatology clinic with thick plaques resembling psoriasis. Biopsy of one of the lesions revealed sarcoidal granulomas in association with psoriasiform changes. Conclusion: Psoriasiform lesions are a rare manifestation of sarcoidosis. The authors theorize that the co-expression of TNF-? in both entities is a possible explanation of the psoriasiform expression of sarcoidosis. (J Clin Aesthet Dermatol. 2016;9(4):55–57.)
Sarcoidosis is a non-caseating granulomatous disease of unknown etiology, with diverse cutaneous clinical manifestations that range from subtle papules to erythrodermic lesions. The authors describe herein a case of a patient presenting with sarcoidal lesions exhibiting psoriasiform changes, and review the existing literature.
Case
A 60-year-old African-American woman with no prior personal or family history of psoriasis presented with a six-month history of cutaneous lesions over her lower extremities and face. The areas appeared clinically as thick, well-demarcated, erythematous plaques with a silvery scale (Figure 1). The lesions were persistent, but largely asymptomatic, causing mostly cosmetic concerns for the patient. On the left cheek, the patient had an infiltrated edematous erythematous annular plaque without desquamation or scale (Figure 2).
The patient had a previously confirmed history of pulmonary sarcoidosis with a recent computed tomography (CT) scan of the chest revealing groundglass opacities containing superimposed fibrotic changes within bilateral lung bases in addition to bilateral hilar and mediastinal lymphadenopathy, and pulmonary function testing showing severe restrictive and obstructive changes.
Biopsy of a pretibial plaque demonstrated non-caseating epitheliod granulomas in the upper and mid-dermis with thick parakeratotic scale containing microabcesses (Figure 3 and Figure 4). These skin findings were consistent with the diagnosis of psoriasiform sarcoidosis. A cheek lesion was injected with 5mg/cc triamcinolone acetonide at the initial visit, but secondary to poor response at a follow-up visit two weeks later, the patient declined further injections. Triamcinolone 0.1% ointment was instead prescribed twice daily to the cutaneous lesions, and two weeks later the patient reported some improvement and opted to continue with topical therapy at this time.
Discussion
The classic pathologic finding of sarcoidosis is a noncaseating granuloma. It consists of centrally organized collections of macrophages and epithelioid cells encircled by lymphocytes. The typical histopathology of psoriatic lesions presents with intraepidermal collections of neutrophils, hypogranulosis, and parakeratosis.
Psoriasiform lesions of sarcoidosis are an established, but rare cutaneous manifestation of sarcoidosis.[1–3] Only 0.9 percent of sarcoidosis patients develop this form of the disease, and the majority of these cases have been reported in dark-skinned patients. The scaling plaques are seen on the legs and generally heal with scarring.[4 ]
Psoriasis may occur in patients with sarcoidosis, but the concomitant occurrence is rare with only a handful of cases reported in the literature.[2],[5]
Although the exact etiology of sarcoidosis is not yet known, the immunologic response in this disease process has been characterized as: the initial step being the recognition and phagocytosis of a yet unknown antigen by an antigen-presenting cell and its presentation to CD4(+) T cells, which, in turn, elicit a cellular immune response. The inflammatory profile of sarcoidosis is generally characterized by Th1-associated cytokines (including interleukin 12 [IL-12], interferon gamma [IFN-?], IL-15 and IL-18) and molecules associated with chronic granulomatous inflammation (including angiotensin-converting enzyme, tumor necrosis factor alpha [TNF-?], and macrophage inflammatory protein 1,6 all responsible for the generation of the granulomatous inflammation. Similarly, characterization of cells and cytokines in psoriasis have also shown elevated levels of Th1 cytokines including, but not limited to, IFN-?, TNF-?, and IL-12. TNF-? is a critical component in the formation and maintenance of granulomas, and has a crucial role in the pathogenesis of psoriasis.[7–11]
Data on treatment of sarcoidosis has been revealing. The mainstay of treatments for cutaneous sarcoidosis has been largely based off of those used for the pulmonary variant, such as corticosteroids and anti-malarials. However, other options are being explored for patients that are intolerant or resistant to these therapies. Although the antigenic stimulus has yet to be elucidated, the known characteristic overproduction of TNF-? in sarcoidosis has led to a new therapeutic target.
Recent research has suggested a benefit from TNF-? inhibitors, such as thalidomide, infliximab, and adalimumab,[12–16] especially in the subset of patients who remain symptomatic despite the use of steroids and/or cytotoxic agents, and who particularly express high serum levels of TNF-?.[17],[18] The encouraging response to these biologic agents has emphasized the key role that TNF-? plays in the pathogenesis of sarcoidosis and possible immunopathogenic link for the development of psoriasiform sarcoidosis in patients with a psoriatic diathesis.
The details of TNF’s role in the pathogenesis of sarcoidosis and psoriasis require more research, but the authors theorize that the co-expression of TNF-? in both entities may be an explanation for the development of psoriasiform lesions over plaques of sarcoidosis, such as seen in their patient. Further follow-up of these patients may help elucidate whether these patients may be at increased risk of developing psoriasis.
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