Sarcoidosis expressing as four distinct cutaneous lesions

ARTICLE

Auteur(s) : Hideki Maejima, Chihoko Nakahara, Akira Watarai, Kensei Katsuoka

Department of Dermatology, Kitasato University School of Medicine, 1-15-1, Kitasato University School of Medicine, Sagamihara, Kanagawa, Japan 228-8555

We herein report a patient with sarcoidosis who exhibited four different cutaneous forms and livedo. A 62-year-old Japanese woman had noticed reddish papules on her nose eight years previously (figure 1A). She also had a two year history of uveitis. Asymptomatic nodules appeared in her knee (figure 1B). She noticed several diffuse and indurated erythemas on both hands (figure 1C) and plaque lesions with livedo reticularis on both upper thighs (figure 1B). A skin biopsy specimen from her nose demonstrated dense naked granulomatous infiltrates (figure 1D). The histopathological findings of livedo showed thin-walled, narrowly dilated small arteries and epithelioid granulomas in the lower dermis and subcutaneous tissues (figure 1E). A microscopic examination using polarized light did not reveal the presence of silicon or a foreign body in the granulomas. PAS and Ziehl-Neelsen staining did not demonstrate the presence of bacilli.

Laboratory studies revealed an increased serum lactose dehydrogenase level (276 U/mL), angiotensin I converting enzyme value (34.7 U/mL, normal range within 21.4 U/mL), and sialylated carbohydrate antigen KL-6 level (628 U/mL); other values, including the peripheral blood counts and liver and renal function parameters, were normal. A tuberculin test was negative. Chest radiography showed multiple miliary and infusion shadows (figure 1F). A gallium-67 scan revealed hepato-splenomegaly. The echocardiography results were normal. We diagnosed her as having polymorphous cutaneous sarcoidosis including nodular and lupus pernio, infiltrating scars, plaque lesions involved livedo reticularis and pulmonary involvement.

The cutaneous lesions of sarcoidosis sometimes present in unusual forms [1]. Our case demonstrated four clinical manifestations: nodules, lupus pernio, plaque lesions, and scar infiltrates. Overall, 5.3% of sarcoidosis patients exhibit at least two forms of common cutaneous disease [2]. We retrospectively investigated 80 patients with sarcoidosis (17 men and 63 women). Two forms of cutaneous disease were observed in 28 patients, and three forms were observed in two; only the presently reported patient exhibited four different clinical forms of cutaneous disease. Livedo in association with sarcoidosis is rare [3]. However, seven patients with these conditions have been treated at our clinic. Most patients with sarcoidosis and livedo are female, and they often present with paralysis and/or ocular or pulmonary involvement [3]. Thus, sarcoidosis with systemic vascular involvement often presents with nervous and/or pulmonary diseases [4]. In our hospital, six of the seven patients with sarcoidosis and livedo were female, and all the patients had pulmonary involvement or bilateral lymphadenopathy.

The incidence of nervous system involvement was also higher among patients with livedo and sarcoidosis (2/7: 28.6%) than among patients with only sarcoidosis (4/57: 7.0%). Livedo in association with sarcoidosis might be a clinical marker of nervous system or pulmonary involvement and bilateral lymphadenopathy. Our patient had a rare clinical presentation with four forms of cutaneous sarcoidosis and livedo reticularis, and the presence of cutaneous vascular involvement in patients with sarcoidosis may be important.

Acknowledgements

References

2 Niels KV, Dorrit S, Holger B. Cutaneous sarcoidosis in Caucasians. J Am Acad Dermatol 1987; 16: 534-40.

3 Motoko T, Seichiro K, Haratoh R, et al. A case of sarcoidosis with livedo. Japanese Journal of Clinical Dermatology 2003; 57: 712-4; (in Japanese).

4 Takemura T, Matsui Y, Oritsu M, et al. Pulmonary vascular involvement in sarcoidosis: granulomatous angiitis and microangiopathy in transbronchial lung biopsies. Virchows Arch A Pathol Anat Histopathol 1991; 418: 361-8.