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Ulcerative sarcoidosis

  • Author(s): Hunt, Raegan D
  • Gonzalez, Mercedes E
  • Robinson, Maria
  • Meehan, Shane A
  • Jr, Andrew G Franks
  • et al.
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Ulcerative sarcoidosis
Raegan D Hunt MD PhD, Mercedes E Gonzalez MD, Maria Robinson MD, Shane A Meehan MD, Andrew G Franks Jr MD
Dermatology Online Journal 18 (12): 29

The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York

Abstract

Sarcoidosis is a multi-system, granulomatous disease, which affects the skin in approximately 20 to 30 percent of cases. Recognition of cutaneous sarcoidosis can be challenging because of the wide range of skin lesion morphologies. Ulcerative sarcoidosis is uncommon. We present a 35-year-old woman with pretibial ulcerative sarcoidosis, indurated tattoos, and hilar lymphadenopathy.



History

A 35-year-old woman presented to the Dermatology Clinic at the Charles C. Harris Skin and Cancer Pavilion for evaluation of painful ulcers of the anterior aspects of her lower legs. She reported that the ulcers developed within erythematous papules and plaques. She also noted tenderness of her tattoos. She denied pruritus. Review of symptoms was unremarkable. She had no significant past medical history. Her sister had erythema nodosum and rheumatoid arthritis, but no other family members had cutaneous or rheumatologic diseases.


Physical examination


Figure 1Figure 2

There were numerous, annular and candle-drip, red-brown papules and plaques with punched-out superficial ulcers on the anterior aspects of the lower legs. The lower legs were edematous. The outlines of her tattoos were mildly erythematous and indurated.


Laboratory data

A complete blood cell count, comprehensive metabolic panel, calcium, gamma-glutamyl transpeptidase, erythrocyte sedimentation rate, and thyroid stimulating hormone were normal. Antinuclear antibody, anti-Ro/SSA, anti-La/SSB, and anti-Scl-70 antibody titers were negative. Hepatitis A, B, and C serologies were negative. Angiotensin converting enzyme level was normal. Chest radiographs demonstrated prominent hila, which were consistent with typical adenopathy that is associated with sarcoidosis. Cultures of a skin biopsy specimen for bacteria, mycobacteria, and fungi were negative.


Histopathology


Figure 3

There is a superficial and deep, perivascular and interstitial infiltrate that is composed primarily of epithelioid histiocytes, many of which are present as small aggregates. Focally, there are numerous neutrophils. Periodic acid-Schiff and diastase and Gram stains for fungi, bacteria, and acid-fast bacilli fail to show microorganisms.


Discussion

Sarcoidosis is an idiopathic, granulomatous disease. It has the potential to involve multiple organ systems, but most commonly affects the lungs [1]. Cutaneous manifestations occur in approximately 20 to 30 percent of patients and can be divided into specific sarcoidal skin lesions that exhibit non-caseating granulomas and reactive, non-specific eruptions that lack granulomatous inflammation (e.g., erythema nodosum) [2]. Specific sarcoidal skin lesions classically present as red-brown-to-violaceous papules, plaques, and nodules without epidermal surface changes that are distributed symmetrically on the head, neck, upper trunk, and upper extremities [3]. Diascopy demonstrates a classic, yellow-brown, apple jelly hue [4]. Sarcoidal lesions also develop at sites of trauma, within scars, and within tattoos [5, 6, 7]. Less frequent clinical presentations include atrophic, verrucous, hypopigmented, and ulcerative sarcoidal lesions. Acquired icthyosis, erythroderma, and focal alopecia are less common presentations [2].

Ulcerative sarcoidosis, which is an uncommon cutaneous disease variant, shows a predilection for young adults and most often affects women and individuals of African or Japanese descent [8, 9]. Among Caucasian patients, the incidence of ulcerative disease is estimated to be near 1 percent [10]. Ulcers tend to arise within existing papulonodules on the lower legs but may occur on previously normal skin and in a generalized distribution. The majority of ulcerative sarcoidosis patients have signs and/or symptoms of sarcoidosis that involve other organs [8, 9, 11]. Biopsy specimens of the ulcerative sarcoidosis lesions demonstrate sarcoidal granulomas. Inasmuch as ulcerative sarcoidosis can mimic venous ulcers, a biopsy may be helpful in cases of chronic leg ulcers that are refractory to treatment [12].

No definitive diagnostic test for sarcoidosis exists. Establishing the diagnosis requires compatible clinical findings, evidence of granulomatous inflammation in one or more organ systems, and exclusion of other diseases [13]. Non-caseating epithelioid granulomas are the hallmark histopathologic feature but are not specific. Polarized light microscopy, special stains for microorganisms, and tissue cultures must be performed to exclude a foreign-body reaction and infection with mycobacteria or fungi [3].

Decisions regarding treatment of cutaneous sarcoidosis are influenced by the severity of skin disease, evidence of concurrent internal organ involvement, and the patient’s treatment goals. Standard therapeutic options include topical, intralesional and oral glucocorticoids, methotrexate, and antimalarial drugs [14]. Thalidomide and leflunomide have also been reported to improve cutaneous sarcoidosis [15, 16]. For treatment of ulcerative sarcoidosis, case reports and small case series support the use of oral glucocorticoids, oral glucocorticoids in combination with antimalarial drugs, and methotrexate [8, 17]. More recently, adalimumab treatment improved ulcerative sarcoidosis in a patient who failed to respond to prior treatment with prednisolone and hydroxychloroquine [18].

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18. Philips MA, et al. Ulcerative cutaneous sarcoidosis responding to adalimumab. J Am Acad Dermatol 2005; 53: 917 [PubMed]

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