Cutaneous sarcoidosis treated with medium-dose UVA1

Abstract

An 82-year-old woman presented with a 2-year history of progressive, itching skin lesions, affecting more than 70% of her body surface. In addition, she had multiple internal diseases such as breast cancer, lung fibrosis after radiation, heart failure, hypertension, and diabetes mellitus. Physical examination revealed multiple, painless, red-brown, elevated, partially confluent papules and plaques disseminated over the whole body (Fig 1). Diascopy of the lesions revealed a grey-yellow infiltrate. All blood tests were within reference limits. Angiotensin-converting enzyme was increased at 43 U/L (normal: 6-32 U/L). Chest radiography; electrocardiography; abdominal sonography; and internal, neurologic, and ophthalmologic examinations revealed no systemic signs of sarcoidosis. Histology of a papule showed noncaseating epitheloid cell granulomas with multinucleated giant cells confirming the diagnosis of cutaneous sarcoidosis. Topical glucocorticosteroids and tacrolimus ointment were used without effect. Oral therapeutic approaches were not induced as a result of absence of systemic involvement of sarcoidosis and the risk of side effects in the patient who is multimorbid. Thus, we treated our patient 4 times a week with medium-dose UVA1 irradiation (Sellamed 2400A, Dr Sellmayer, Sellas, Gevelsberg, Germany), starting with a dose of 20 J/cm2 for the first 3 treatment sessions and 40 J/cm2 for the subsequent 12 sessions. The final dose of 60 J/cm2 was given 35 times. After 50 sessions of medium-dose UVA1 and a total dose of 2640 J/cm2, nearly all lesions disappeared (Fig 2). Adverse effects were not observed during and after UVA1 irradiation. A biopsy specimen 5 months after therapy showed normal skin and a serum angiotensin-converting enzyme level within reference limit. Sarcoidosis is a multisystem disorder characterized histologically by epitheloid cell granulomas without caseating necrosis. Pathophysiologically it has been proposed that formation of sarcoid granulomas occurs as a series of immune events consisting of the triggering of CD4+ T-helper cells by local antigen-presenting cells and the accumulation of immunocompetent cells at sites of disease. It is speculated that accumulated T-helper cells, cooperating with macrophages, contribute to the development of granulomas in sarcoidosis. Diverse therapeutic approaches have been used in the therapy of sarcoidosis. UVA1 phototherapy represents an increasingly used therapeutic modality. Disparate diseases such as atopic dermatitis, urticaria pigmentosa, and lichenoid graft-versus-host disease have been treated with success. Various immunomodulating and immunosuppressive effects of UVA1 are known, which may be responsible for the therapeutic effort in cutaneous sarcoidosis. Long-wave UVA induces apoptosis in T cells.1Morita A Werfel T Stege H Ahrens C Karmann K Grewe M et al.Evidence that singlet oxygen-induced human T helper cell apoptosis is the basic mechanism of UVA radiation phototherapy.J Exp Med. 1997; 186: 1763-1768Crossref PubMed Scopus (237) Google Scholar UVA1 irradiation, similar to UVB, has been shown to induce the expression of immunsuppressive cytokines in human keratinocytes including tumor necrosis factor-α and IL-10.2Krutmann J, Stege H, Morita A. UVA1 phototherapy: indications and mode of action. In: Dermatological phototherapy and photodiagnostic methods. New York: Springer; 2001. p. 261-78Google Scholar Graefe et al3Graefe T Konrad H Barta U Elsner P Successful UVA1 treatment of cutaneous sarcoidosis.Br J Dermatol. 2001; 145: 354-355Crossref PubMed Scopus (32) Google Scholar described an improvement in cutaneous sarcoidosis by a female patient with plaques on her forehead treated with high-dose UVA1. Our patient had generalized disease involving more than 70% of her skin. Medium-dose (ie, up to 60 J/cm2) UVA1 phototherapy was applied, on the basis of our experience and publications on positive effects of medium-dose UVA1 in other cutaneous diseases.4Pinton P.C Capezzera R Zane C De Panfilis G Medium-dose UVA1 therapy for pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica.J Am Acad Dermatol. 2002; 47: 410-414Abstract Full Text Full Text PDF PubMed Scopus (47) Google Scholar Full remission was achieved without any side effects. However, the long-term effects such as carcinogen risk of UVA1 therapy have to be followed up.5Sterenbroigh H.C.J.M van der Leun J.C Tumorgenesis by long wave-length UVA source.Photochem Photobiol. 1990; 51: 325-330Crossref PubMed Scopus (126) Google Scholar