The Clinical Picture Giulia Rech, MD Riccardo Balestri, MD Federico Bardazzi, MD Internal Medicine, Aging and Nephrologic Internal Medicine, Aging and Nephrologic Internal Medicine, Aging and Nephrologic Department, Division, Disease Department, Dermatology Division, Disease Department, Dermatology Division, Ospedale Sant’Orsola-Malpighi, Università degli Ospedale Sant’Orsola-Malpighi, Università degli Ospedale Sant’Orsola-Malpighi, Università degli Studi di Bologna, Italy Studi di Bologna, Italy Studi di Bologna, Italy Bianca Maria Piraccini, MD, PhD AnnalisA Patrizi, MD, PhD Internal Medicine, Aging and Nephrologic Disease Internal Medicine, Aging and Nephrologic Disease Department, Dermatology Division, Ospedale Sant’Orsola- Department, Dermatology Division, Ospedale Sant’Orsola- Malpighi, Università degli Studi di Bologna, Italy Malpighi, Università degli Studi di Bologna, Italy The Clinical Picture reactivation and dry cough

52-year-old woman is referred to our A dermatology clinic by her primary care physician for swelling and redness of seven old . The swelling began 3 months ago. She is on no regular medications. She has never smoked. She underwent liposuction 7 years ago and appendectomy at age 15. Physical examination reveals indurated and painful brownish-red plaques on the thighs and lower trunk, extending beyond the borders of six scars from the liposuction surgery and one scar from the appendectomy (Figure 1). She reports no dyspnea, night sweats, weight loss, fever, or other constitutional symptoms, but she has a dry cough that began 2 months after the onset of the skin symptoms. Her pri- mary care physician has managed the cough symptomatically. Laboratory testing shows mild leukopenia, with a white blood cell count of 3.0 × 109/L (reference range 4.2–9.0). Other routine labo- ratory values are normal, including antinuclear antibody, extractable nuclear antibody, anti- double-stranded DNA, rheumatoid factor, urinalysis, erythrocyte sedimentation rate, C- reactive protein, serum calcium concentration, and liver and renal function tests. FIGURE 1. Indurated and painful brownish-red plaques lo- Chest radiography reveals bilateral hilar calized on old scars: (A) lower trunk, (B) left side, (C) right lymphadenopathy. side. Q: What is the next most appropriate diag- A: All are appropriate at this point. In this nostic procedure? case, skin biopsy and high-resolution CT were □□ Skin biopsy performed. Histopathologic examination of □□ High-resolution computed tomography one of the scars showed multiple well-demar- (CT) of the chest cated, large, noncaseating epitheloid granulo- □□ QuantiFERON-TB Gold test mas with histiocytes and multinucleated giant □□ Ventilatory function tests cells. High-resolution CT confirmed bilateral □□ Serum angiotensin-converting enzyme hilar and mediastinal lymphadenopathy and (ACE) level revealed micronodular densities with a bron- doi:10.3949/ccjm.78a.10132 chovascular and subpleural distribution.

CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 78 • NUMBER 6 JUNE 2011 375 Downloaded from www.ccjm.org on September 29, 2021. For personal use only. All other uses require permission. Scar sarcoidosis

Although sarcoidosis on liposuction scars has not been reported previously, the reactivation of old scars is well known on sites of previous injec- tions, , herpes zoster, and burns.2,3 The finding of is not specific for sarcoidosis (Figure 2). The histologic differen- tial diagnosis of sarcoidosis includes tubercu- losis, atypical mycobacteriosis, fungal infec- tion, reaction to a foreign body, rheumatoid nodules, leishmaniasis, Crohn disease, and lipoidica diabeticorum. The diagnosis of scar sarcoidosis is con- firmed only by excluding other conditions via Figure 2. Noncaseating granuloma seen on a comprehensive evaluation of clinical mani- skin biopsy study. festations, histology, history, and radiologic and laboratory findings. An interferon-gamma-release assay for tu- It has been suggested that the most satisfy- berculosis—QuantiFERON-TB Gold (Cell- ing therapy for the patient and physician in estis, Carnegie, Australia)—was negative. sarcoidosis is no treatment at all,4 and in fact Ventilatory function tests showed a normal sarcoidosis often remits spontaneously. Cur- pattern, while the serum ACE level, elec- rently, the choice of treatment depends on trocardiography, and an eye examination re- the degree of systemic involvement, and the vealed no pathologic findings. oral corticosteroid prednisone remains the first-line treatment. If the condition does not Q: What is the diagnosis? respond, the use of other systemic agents has □□ been reported, but their effectiveness has not □□ Scar sarcoidosis been evaluated in controlled clinical trials. Treatment: □□ Paraneoplastic sign Recurrence is common after the suspen- oral prednisone □□ Dermatofibrosarcoma protuberans sion of treatment; therefore, treatment may □□ Tubercolosis need to be continued for several years, with 1 mg/kg/day frequent checkups. for 6 weeks, A: Based on the data outlined above, we Skin lesions are a visible clue to the di- then tapered made the diagnosis of scar sarcoidosis with agnosis. Reactivation of old scars may be the involvement of hilar and mediastinal lymph single manifestation of cutaneous sarcoidosis, until skin and nodes. The patient began systemic treatment but it may also precede or accompany systemic hilar lesions with oral prednisone 1 mg/kg/day for 6 weeks, involvement, often representing the main sign which was then gradually withdrawn, until of an exacerbation or a relapse of systemic sar- resolved the skin and hilar lesions resolved completely. coidosis, as in our patient.5 ■

■■ scar sarcoidosis ■■ REFERENCES

1. Marchell RM, Judson MA. Chronic cutaneous lesions of Sarcoidosis is a multisystem disorder of un- sarcoidosis. Clin Dermatol 2007; 25:295–302. known cause characterized by the formation 2. Tchernev G. Cutaneous sarcoidosis: the "great imitator": etiopathogenesis, morphology, differential diagnosis, of noncaseating in the affected and clinical management. Am J Clin Dermatol 2006; organs. Patients may present with symptoms 7:375–382. related to the specific organ affected, but they 3. Fernandez-Faith E, McDonnell J. Cutaneous sarcoidosis: differential diagnosis. Clin Dermatol 2007; 25:276–287. may have no symptoms or only general symp- 4. Baughman RP, Lower EE, du Bois RM. Sarcoidosis. Lancet toms such as fever or general malaise. 2003; 361:1111–1118. The skin is involved in 25% of cases and 5. Sorabjee JS, Garje R. Reactivation of old scars: inevitably presents so many polymorphous manifesta- sarcoid. Postgrad Med J 2005; 81:60–61.

tions that sarcoidosis has become known as ADDRESS: Riccardo Balestri, MD, Via Massarenti 1, Clinica 1,2 one of the “great imitators” in dermatology. Dermatologica, 40138 Bologna, Italy; e-mail [email protected].

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