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Subcutaneous Sarcoidosis in a Melanoma

Lotika Singh, MD; Sina Aboutalebi, MD; Jennifer Smith, MD

Cutaneous sarcoidosis often has been referred Case Report to as the great imitator because A 49-year-old woman presented with 3 firm sub- can present with various morphologies. Skin cutaneous nodules at the border of a melanoma lesions may be the only site of involvement or scar on her left upper arm. The lesions appeared may accompany systemic . Occasion- over 5 months and were first noticed approximately ally, sarcoidosis also may infiltrate from 10 months after the melanoma excision. She denied prior trauma, , or . We report a any associated pain, pruritus, or irritation. The nod- case of subcutaneous sarcoidosis limited to a ules were 3 to 5 mm in diameter and developed at the melanoma scar without any other cutaneous or proximal margin of a linear scar; there was no visible systemic involvement. Familiarity with and proper epidermal change (Figure 1). On examination there diagnosis of cutaneous sarcoidosis can allow for was no lymphadenopathy or hepatosplenomegaly. appropriate systemic screening and timely man- Our initial differential diagnosis included suture agement of the disease. versus recurrent melanoma. A punch Cutis.CUTIS 2011;87:234-236. biopsy of a nodule revealed naked noncaseating granulomas with multinucleated giant cells within the subcutaneous . A few asteroid bodies and a rim arcoidosis is an inflammatory condition with of lymphocytes also were noted around the granulo- noncaseating granulomas that may affect mul- mas. The overlying and showed no S tiple systems. Involvement of the skin is histologic changes (Figure 2). The findings were most seen in approximately 25% of cases, either as the consistent with cutaneous sarcoidosis. onlyDo site affected or accompanying Not systemic disease.1 On reviewCopy of systems, the patient reported occa- Cutaneous sarcoidosis often has been referred to as sional sharp chest pains and recurrent pneumonia the great imitator because skin lesions may exhibit with a history of chronic obstructive pulmonary dis- various morphologies.2 Specific manifestations may ease. She also admitted to smoking 35 to 40 packs of include ; nodules; plaques; and lichenoid, verrucous, ichthyosiform, and ulcerative lesions.2-4 Skin lesions also may be the first signs of sarcoidosis, preceding systemic spread of the disease. Recognition of cutaneous sarcoidosis often can be challenging and may be overlooked until systemic involvement is apparent. Proper diagnosis can allow for appropriate systemic screening and timely treatment.

Dr. Singh is from Texas Tech University Health Sciences Center, Amarillo. Drs. Aboutalebi and Smith are from the Department of , Texas Tech University Health Sciences Center, Lubbock. The authors report no conflict of interest. Figure 1. A healed scar from a melanoma excision on Correspondence: Jennifer Smith, MD, Texas Tech University Health the left arm with no epidermal changes. There is mild Sciences Center, 3601 4th St, MS 9400, Lubbock, TX 79430 crusting at the proximal margin of the scar at the ([email protected]). biopsy site.

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B

CUTISA C Figure 2. No histologic changes were demonstrated in the epidermis; well-demarcated naked noncaseating granu- lomas were noted in the subcutaneous fat (A and B)(H&E; original magnifications 340 and 3200, respectively). An asteroid body within a multinucleated giant cell was revealed (C)(H&E, original magnification 3400).

cigarettesDo yearly. A chest radiograph Not was obtained and of life.2,4Copy The lesions commonly present as firm, showed a patchy right upper lobe infiltrate with no nontender, flesh-colored to violaceous nodules hilar lymphadenopathy. Laboratory testing revealed located mainly on the extremities or trunk. They a slightly elevated angiotensin-converting enzyme generally are asymptomatic or mildly tender and are level of 72 U/mL (reference range, 9–67 U/mL) therefore thought to be underreported. Although and a serum total calcium level within reference uncommon, subcutaneous sarcoidosis is strongly asso- range. Other hematologic tests including a complete ciated with the presence of systemic disease and blood cell count, liver function tests, and renal func- lesions often may be the first manifestations of sar- tion were all within reference range. Although the coidosis.2 For this reason, thorough examination with patient declined treatment of the nodules, she was appropriate systemic screening is warranted, even in referred to her primary care provider for further sys- the absence of other clinical symptoms. Subcutaneous temic evaluation. sarcoidosis also is known as Darier-Roussy sarcoid; however, this term has fallen out of favor because it Comment also has been used to refer to other forms of granulo- Cutaneous involvement is common in sarcoidosis, matous including tuberculosis. with lesions classified as either specific or nonspe- Cases of sarcoidosis infiltrating preexisting scars cific. Specific lesions contain the more characteristic from mechanical trauma, infection, and tattoos are sarcoidal granulomas on biopsy, while nonspecific well-documented in the literature.5,6 Although the lesions vary in their histologic appearance. Subcu- clinical presentation of cutaneous lesions can vary taneous sarcoidosis is a rare subset of specific cuta- considerably, the majority feature some degree of neous lesions seen more frequently in females than epidermal involvement.3 One study examining males, with peak incidence in the fourth decade the histologic appearance of cutaneous lesions of

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sarcoidosis noted epidermal changes in 79% of The presentation of subcutaneous sarcoidosis in cases (49/62).7 Lesions with scar sarcoidosis showed our patient is unique in that there were no epidermal focal , acanthosis, and more commonly changes, the lesions were limited to a preexisting epidermal or . Scar sarcoid- melanoma scar, and there was no other cutaneous or osis or cicatricial sarcoidosis also has been linked systemic involvement. Additionally, our patient did to interferon alfa treatment in some patients.5 The not undergo interferon therapy. appearance of cutaneous sarcoidosis in patients with melanoma following interferon alfa treatment has REFERENCES been described. However, the appearance of lesions 1. Ahmed I, Harshad SR. Subcutaneous sarcoidosis: is it a of sarcoidosis remained discretely separate from the specific subset of cutaneous sarcoidosis frequently associated melanoma excision site.6,8 with systemic disease? J Am Acad Dermatol. 2006;54:55-60. Subcutaneous sarcoidosis portends an excel- 2. Tchernev G. Cutaneous sarcoidosis: the “great imitator.” lent prognosis, with complete remission seen in Am J Clin Dermatol. 2006;7:375-382. 86% (12/14) of patients as demonstrated by one 3. Mangas C, Fernández-Figueras MT, Fité E, et al. Clinical study.1 Primary treatment consists of oral corticoste- spectrum and histological analysis of 32 cases of specific roids for systemic disease or intralesional triamcino- cutaneous sarcoidosis. J Cutan Pathol. 2006;33:772-777. lone for limited cutaneous lesions. Other reported 4. Marcoval J, Maña J, Moreno A, et al. Subcutaneous therapies include hydroxychloroquine sulfate, metho- sarcoidosis—clinicopathological study of 10 cases. Br J trexate sodium, clofazimine, dapsone, or nonsteroidal Dermatol. 2005;153:790-794. anti-inflammatory drugs. These agents have been 5. Selim A, Ehrsam E, Atassi MB, et al. Scar sarcoidosis: a used alone or in combination to effectively treat sub- case report and brief review. Cutis. 2006;78:418-422. cutaneous sarcoidosis.1 6. Perez-Gala S, Delago-Jimenez Y, Goiriz R, et al. Cutane- ous sarcoidosis limited to scars following pegylated inter- Conclusion feron alfa and ribavirin therapy in a patient with chronic Despite a good prognosis, subcutaneous lesions of hepatitis C. J Eur Acad Dermatol Venereol. 2007;21:393-394. sarcoidosis merit careful exploration and workup 7. Okamoto H. Epidermal changes in cutaneous lesions of because of their strong correlationCUTIS with systemic sarcoidosis. Am J Dermatopathol. 1999;21:229-233. disease. Because these lesions often are the present- 8. Alonso-Pérez A, Ballestero-Díez M, Fraga J, et al. Cuta- ing symptoms of a multisystem disorder, recognition neous sarcoidosis by interferon therapy in a patient and timely treatment can slow or arrest progression of with melanoma. J Eur Acad Dermatol Venereol. 2006;20: systemicDo spread. Not 1328-1329.Copy

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