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Sarcoidosis with subcutaneous lesions

  • Author(s): Heller, Marissa
  • Soldano, Anthony C
  • et al.
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Sarcoidosis with subcutaneous lesions
Marissa Heller MD, Anthony C Soldano MD
Dermatology Online Journal 14 (5): 1

Department of Dermatology, New York University

Abstract

A 30-year-old man presented for evaluation of multiple, hypopigmented macules and erythematous papules on his upper and lower lips and upper extremities. He subsequently developed firm, non-tender, subcutaneous, mobile nodules on the left forearm, left calf, and right thigh. Clinical and histopathologic features were consistent with cutaneous and subcutaneous sarcoidosis. Systemic evaluation showed no evidence of extracutaneous involvement. Subcutaneous sarcoidosis is a rare form of nodular cutaneous sarcoidosis. Typical skin findings are painless, firm, mobile nodules without overlying epidermal involvement and with a predilection for the trunk and extremities. Histopathologic alterations include epilthelioid cell tubercles in the subcutaneous fat. As this is a diagnosis of exclusion, other etiologies of granulomatous inflammation must be ruled out with tests and special stains. The mainstay of treatment is with oral glucocorticoids.



Clinical synopsis

A 30-year-old man presented to the New York Campus of the Veterans Affairs New York Harbor Healthcare System for the treatment of lesions on his lips and forearms. He subsequently developed several, painless subcutaneous nodules on his trunk and extremities. There were no systemic features. There is no personal or family history of autoimmune conditions. Past medical history included chronic sinusitis, and a computed tomography scan showed extensive mucosal disease within the paranasal sinuses for which the patient is being followed by an otolaryngologist. The patient takes no systemic medications. The patient was treated with hydroxychloroquine, which was discontinued due to lack of compliance. Therefore, the patient has been maintained on topical glucocorticoids, which included clobetasol 0.05 percent ointment twice daily to the erythematous papules on his upper extremities, hydrocortisone 2.5 percent ointment twice daily to the erythematous papules on his lips, and intralesional injections of triamcinolone, 5mg/cc, to the subcutaneous nodules on his trunk and extremities. A punch biopsy of an erythematous papule on the right forearm as well as two incisional biopsies of subcutaneous nodules on the right leg were performed.


Physical examination

Figure 1Figure 2

Multiple, 1- to-2-mm, hypopigmented macules and erythematous papules were present on the upper and lower lips and upper extremities. Four, firm, 3- to-6-cm, non-tender, subcutaneous, mobile nodules were palpable on the ventral aspect of the left forearm, posterior aspect of the left calf, and right thigh. An ophthalmologic examination was normal.


Laboratory data

A complete blood count, basic metabolic panel, liver function tests, urinalysis, and pulmonary function tests were normal. A chest radiograph showed bilateral hilar fullness. Human immunodeficiency virus, hepatitis B antigen and antibody, and hepatitis C antibody tests were negative. A rapid plasma reagin test was nonreactive. A purified protein derivative skin test was negative.


Histopathology

Figure 3

A complete blood count, basic metabolic panel, liver function tests, urinalysis, and pulmonary function tests were normal. A chest radiograph showed bilateral hilar fullness. Human immunodeficiency virus, hepatitis B antigen and antibody, and hepatitis C antibody tests were negative. A rapid plasma reagin test was nonreactive. A purified protein derivative skin test was negative


Comment

Subcutaneous sardoidosis is a rare, specific subtype of nodular cutaneous sarcoidosis that was originally described by Darier and Roussy in 1904. Although these nodules are often referred to as Darier-Roussy sarcoid, this is actually a term that is used to describe many subcutaneous inflammatory disorders and is therefore nonspecific. Hence, this eponym has been largely abandoned in exchange for the more accurate term subcutaneous sarcoidosis [1]. The diagnosis of subcutaneous involvement in sarcoidosis is made by the histopathologic identification of noninfectious sarcoidal or epithelioid granulomas with multi-nucleated giant cells and with lymphocytic inflammation involving predominantly the panniculus [1, 2]. This diagnosis is associated with extra-cutaneous systemic disease involvement, especially bilateral hilar adenopathy. However, subcutaneous sarcoidosis also has been described in the absence of systemic disease. Subcutaneous sarcoidosis presents as painless, firm, mobile nodules without overlying epidermal involvement [3]. These nodules have a predilection for the trunk and extremities, but also occur on the face. The range of the number of lesions is 1 to 100, and the average size of these lesions is 0.5 to 2 cm [4]. A large, retrospective case series examining the clinical and pathologic characteristics of patients with subcutaneous sarcoidosis, using the criteria outlined above, found 33 cases described in the literature and added 21 new cases from their own institutional database [5]. This series noted a peak incidence of subcutaneous sarcoidosis in the fourth decade of life. In addition, there was a slightly higher incidence in female patients as compared to male patients. Finally, there was an association between subcutaneous sarcoidosis and autoimmune diseases, which has also been noted in the literature. Associations include vitiligo, pernicious anemia, and autoimmune thyroiditis [5, 6]. Another, smaller retrospective review that included 10 patients with subcutaneous sarcoidosis also found a predominance in female patients. However, in this study, the peak incidence was observed in the fifth and sixth decades of life [7]. Histopathologically, subcutaneous sarcoidosis shows epilthelioid cell tubercles in the subcutaneous fat [2]. The nodules arise in the deep dermis and adipose tissue and may rarely become calcified. All forms of cutaneous sarcoidosis, which includes the subcutaneous type, are diagnoses of exclusion. Appropriate tests and special stains always should be performed to rule out additional etiologies of these granulomas. The mainstay of treatment for subcutaneous sarcoidosis is with oral glucocorticoids. Traditional dosages range from 20 to 40 mg daily, with responses noted within 4 to 8 weeks after initiation of therapy. [5] Patients also have been treated with nonsteroidal anti-inflammatory drugs, clofazamine, methotrexate, hydroxychloroquine, intralesional glucocorticoids, dapsone, allopurinol, minocycline, and potassium iodide with various responses [5, 8, 9, 10]. However, because subcutaneous sarcoid is so uncommon, many of these treatment options only are described as single case reports.

References

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2. Shapiro PE. Noninfectious granulomas. In: Elder D, et al, eds. Lever's Histopathology of the Skin. 8th edition. Philadelphia: Lippincott-Raven, 1997: 323

3. Howard A, White CR. Non-infectious granulomas. In: Bolognia JL, et al, eds. Dermatology. New York:Mosby, 2003:1455

4. Kalb RF, et al. Sarcoidosis with subcutaneous nodules. Am J Med 1988;85:731

5. Ahmed I, Harshad S. Subcutaneous sarcoidosis: is it a specific subset of cutaneous sarcoidosis frequently associated with systemic disease? J Am Acad Dermatol 2006;54:55

6. Barnadas MA, et al. Subcutaneous sarcoidosis associated with vitiligo, pernicious anemia and autoimmune thyroiditis. Clin Exp Dermatol 2000;25:55.

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9. Bachelez H, et al. The use of tetracyclines for the treatment of sarcoidosis. Arch Dermatol 2001;137:69

10. Fichtel J, et al. Subcutaneous sarcoidosis presenting after treatment of Cushing's disease. J Am Acad Dermatol 2006;54:360.

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