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Annular Atrophic Plaques on the Forearm

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A 57-year-old woman presented with several lesions on the left extensor forearm of 10 years’ duration. A single annular indurated lesion with central atrophy initially developed near a prior surgical site. The lesions were pruritic with no associated pain or bleeding. Over 5 years, similar lesions had developed extending up the arm. No benefit was seen with low-potency topical steroid application. Biopsy for histopathologic examination was performed to confirm the diagnosis.

What's the diagnosis?

granuloma annulare

leprosy

necrobiosis lipoidica

sarcoidosis

tumid lupus erythematosus

Sarcoidosis is a systemic noncaseating granuloma­tous disease of unknown etiology. The skin is the second most common location for disease mani­festation following the lungs.1 Cutaneous sarcoidosis is present in 35% of patients with sarcoidosis and may be further subtyped by its morphologic characteristics (eg, hyperpigmented, papular, nodular, atrophic, ulcer­ative, psoriasiform). Cutaneous sarcoidosis has an increased tendency to occur at areas of prior injury such as surgeries or tattoos.2 Although sarcoidosis affects all races and sexes, it is more prevalent in women and in the black population.3

The clinical presentation of sarcoidosis is difficult due to its morphologic variation, allowing for a wide differential diagnosis. With our patient’s presentation of atrophic plaques, the differential diagnosis included granuloma annulare, necrobiosis lipoidica, tumid lupus erythematosus, leprosy, and sarcoidosis; however, biopsy is required for definitive diagnosis. The characteristic histopathology for cutaneous sarcoidosis includes nonca­seating granulomas (Figure, A) composed of epithelioid histiocytes with giant cells surrounded by a lympho­cytic infiltrate. Noncaseating granulomas are consid­ered specific to sarcoidosis and are present in 71% to 89% of biopsied lesions.4 Interestingly, our patient pre­sented with a rare subtype of atrophic ulcerative cutane­ous sarcoidosis, necrobiosis lipoidica–like sarcoidosis, which is more common in females and in the black population. It is characterized by pink to violaceous plaques with depressed centers and prominent necrotiz­ing granuloma (Figure, B) on histopathology. In a small case series, all 3 patients with necrobiosis lipoidica–like sarcoidosis were female and had systemic involvement at the time of diagnosis.5

A, Microscopic examination of the biopsied lesion revealed a classic noncaseating granuloma with multinucleated giant cells (H&E, original magnification ×100). B, A palisading necrotizing granuloma consistent with necrobiosis lipoidica–like cutaneous sarcoidosis also was seen (H&E, original magnification ×100).

Sarcoidosis typically is a systemic disease with only a limited number of cases presenting with isolated cutane­ous findings. Therefore, patients require a systemic evalu­ation, which may include a chest radiograph, complete blood cell count, ophthalmologic examinations, thyroid testing, and vitamin D monitoring, as well as an echocar­diogram and electrocardiogram.2

Treatment is guided by the severity of disease. For isolated cutaneous lesions, topical or intralesional high-potency steroids have been shown to be effective.6,7 Several studies also have shown phototherapy and laser therapy as well as surgical excision to be beneficial.8-10 Once cutaneous lesions become disfiguring or systemic involvement is found, systemic corticosteroids or other immunomodulatory medications may be warranted.11 Our patient was started on intralesional and topical high-potency steroids, which failed, and she was transitioned to methotrexate and adalimumab. Unfortunately, even with advanced therapies, our patient did not have notableresolution of the lesions.

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