|Ahead of print publication
Reactivation of a 35-year-old scar!
Eeshaan Ranjan1, Sandeep Arora1, Navya Donaparthi1, Safia Ahmed2, Arpitha Pemmaraju3
1 Department of Dermatology, Command Hospital Air Force, Bengaluru, Karnataka, India
2 Department of Chest Diseases, Command Hospital Air Force, Bengaluru, Karnataka, India
3 Department of Pathology, Command Hospital Air Force, Bengaluru, Karnataka, India
|Date of Submission||22-Jul-2019|
|Date of Acceptance||23-Sep-2019|
|Date of Web Publication||12-Sep-2020|
Department of Skin, Command Hospital Air Force, Bengaluru - 560 007, Karnataka
Source of Support: None, Conflict of Interest: None
Sarcoidosis is a systemic inflammatory disorder of unknown etiology characterized by the presence of noncaseating granulomas. Scar sarcoidosis refers to the infiltration of old scars with noncaseating epithelioid cell granulomas. This reactivation of old scars, manifesting as cutaneous sarcoidosis, is highly specific for sarcoidosis and very unusual. This report describes an old scar with an unusually long history of quiescence and its reactivation after 35 years along with manifestations of pulmonary sarcoidosis.
Keywords: Foreign-body granuloma, sarcoidosis, scar sarcoidosis
| Introduction|| |
Sarcoidosis is a systemic inflammatory disorder of unknown etiology. It is characterized by the presence of noncaseating granulomas. The incidence of this multisystem disease is 10–15 cases per 100,000 each year. Scar sarcoidosis refers to the infiltration of old scars with noncaseating epithelioid cell granulomas. Reactivation of old scars, manifesting as cutaneous sarcoidosis, is highly specific for sarcoidosis, but very unusual. Clinicians may tend to dismiss it if unaware of the significance of this odd sign. We report the case of a 46-year-old female who presented with a recent 15-day onset of swelling of a 35-year-old scar over her left eyebrow, diagnosed as a case of scar sarcoidosis with pulmonary involvement.
| Case Report|| |
A 46-year-old female presented with a recent swelling of a 35-year-old scar over her left eyebrow, which was originally acquired by trauma due to fall onto a kitchen sink at the age of 11. There was no history of cough, breathlessness, or hemoptysis. On examination, a linear, skin-colored, indurated scar was present over her left eyebrow [Figure 1]. The patient was also found to have signs of earlier lower motor neuron facial palsy on the left side (historically of 5-year duration). There was no evidence of excessive scarring or keloid formation at the site of previous healed injuries elsewhere in the body. Systemic examination including respiratory system examination did not reveal any significant finding.
Hematological and biochemical parameters including serum calcium were within the normal limits. The serum angiotensin-converting enzyme was 83.9 U/L (normal range, 16–85 U/L). Mantoux test performed with 5 TU of purified protein derivative was 3 mm after 72 h. Sputum for acid-fast bacilli (AFB) was negative on three consecutive occasions. Noncontrast computed tomography of the chest revealed multiple lymph nodes in the right and left paratracheal, subcarinal, and right hilar stations, with largest measuring 2 cm in the right paratracheal region [Figure 2]. Visualized lung fields appeared normal. Endobronchial biopsy revealed granulomatous lymphadenitis of the paratracheal lymph nodes with absent necrosis and AFB. Ophthalmologic examination, electrocardiogram, ultrasound abdomen, and magnetic resonance imaging brain were normal. Pulmonary function tests revealed a normal ventilatory pattern.
Skin histopathology revealed variably acanthotic epidermis with vacuolar degeneration of basal cell layer. Deep dermis and subcutis showed multiple discrete granulomas consisting of epithelioid histiocytes with numerous interspersed Langhans and foreign-body giant cells. Few lymphocytes were seen rimming the periphery. No caseous necrosis was present [Figure 3]. Occasional giant cells had refractile bodies, which showed up under polarized filter [Figure 4]. Special stains for AFB and fungi were negative. She was treated with intralesional triamcinolone acetonide for scar sarcoidosis and advised 3 monthly follow-up for hilar lymphadenopathy (Siltzbach sarcoidosis Stage I) without medications in the absence of systemic symptoms.
|Figure 3: Multiple discrete granulomas consisting of epithelioid histiocytes with numerous interspersed Langhans and foreign-body giant cells with few lymphocytes rimming the periphery (H and E, ×100)|
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|Figure 4: Giant cells with refractile bodies (H and E, ×400), which showed up under polarized filter polarized filter 300 nm|
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| Discussion|| |
Sarcoidosis is a chronic multisystemic granulomatous disease of unknown etiology. The incidence of cutaneous sarcoidosis is higher in women than men. All organs of the body may be involved, but most frequently affected are the lungs followed by eyes, liver, lymph nodes, and skin. Cutaneous involvement occurs in 10%–35% of patients with systemic sarcoidosis. Scar sarcoidosis is rare, albeit a specific form in which old scars become infiltrated with noncaseating epithelioid cell granulomas representing an exaggerated immune response to various antigens including the posttraumatic material deposits. Its true incidence is not known, but even larger series of cutaneous sarcoidosis have only a few cases of scar sarcoidosis listed. The earlier belief that the presence of foreign material (such as the birefringent material in our case) causes only a foreign-body reaction has been challenged. Clinical signs of reactivation may be itching, erythema, elevation, induration, or increase in size of an older quiet scar. Changes in scars in sarcoidosis patients may also indicate exacerbation of systemic disease [Table 1]. Other than reactivation of scars acquired from old wounds, it has also been reported at the sites of previous tattoo scars, intramuscular injections, blood donation puncture sites, and scars of herpes zoster and at the sites of allergen extracts for desensitization and following hyaluronic acid injection and laser surgery.,
Management of scar sarcoidosis is similar to cutaneous sarcoidosis with recommended use of Class I ultrapotent topical corticosteroids or intralesional triamcinolone for limited involvement (discreet papules and plaques as in scars) till resolution. Systemic agents such as oral corticosteroids, hydroxychloroquine, methotrexate, thalidomide, or tumor necrosis factor alpha inhibitors may only be indicated in widespread or disfiguring lesions not responding to local therapy.
Our case had reactivation of her 35-year-old scar possibly in response to the refractile foreign deposit, presenting later as scar sarcoidosis. She was treated with intralesional triamcinolone as is recommended. In view of this presentation, it is essential that patients presenting with inflammation or any other changes listed above at the sites of preexisting scars of any duration should be examined and investigated for the scar sarcoidosis as well as systemic sarcoidosis. Asymptomatic coexistent pulmonary involvement of Stage 0 or I may be kept on regular follow-up without medication as was done in our case.
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Conflicts of interest
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]