LETTERS TO THE EDITOR
Scar Sarcoidosis: A Rare Entity Diagnosed by Fine-Needle Aspiration Cytology Dear Dr. Bedrossian: Scar sarcoidosis is a rare, uncommon but specific cutaneous manifestation of sarcoidosis, occurring in 2.9–29% of overall cases of sarcoidosis.1 It is one of the great dermatologic masqueraders and most surgeons who deal with scars might not be aware of this condition and its different types of appearance. Therefore, appropriate staging and treatment might be missed or at least delayed. It occurs after mechanical harms of the skin like cuts, but even a venous puncture or a tattoo seem to be sufficient to cause it.2–5 Although skin biopsy, which is the most common diagnostic procedure performed in these cases, is usually a safe procedure, complications such as bleeding, infection, and scarring may occasionally be encountered.6 Fine-needle aspiration cytology (FNAC) can be extremely useful as it does not lead to these complications. Also, in case of scar sarcoidosis, further investigation on systemic involvement must be performed as systemic involvement is seen in most patients with scar sarcoidosis.1 To our knowledge, there are not enough reports emphasizing the role of FNAC in diagnosis.
Case Presentation We present the case of a 60 years old lady with asymptomatic, well-defined skin colored swelling, hard in consistency, increasing in size, on right side of forehead for last 1 year (Fig. 1A). The lesion had developed on a previous traumatic scar in recent time. The clinical differential diagnosis was scar sarcoidosis, hypertrophic scar, or benign skin tumour. Patient was reluctant to undergo excision or incision biopsy so FNAC was done from the
*Correspondence to: Dr Bhawna Jha, M.D.; B 10, Chattarpur Enclave, Phase II, New Delhi 110074, India. E-mail: [email protected] Conflict of interest: Nothing to report Received 17 June 2014; Revised 18 July 2014; Accepted 6 October 2014 DOI: 10.1002/dc.23215 Published online 24 October 2014 in Wiley Online Library (wileyonlinelibrary.com).
438
Diagnostic Cytopathology, Vol. 43, No 5
lesion. The smears were composed of epitheloid granulomas with no evidence of necrosis (Fig. 2). To rule out an infective lesion Ziehl–Neelson stain for leprae and tubercular organism was done, and they were negative. Keeping in view the clinical details and the morphology, a cytological diagnosis of “Scar Sarcoidosis” was given. Further investigations were done to rule out tubercular etiology or any systemic involvement. Mantoux test was negative, Erythrocyte sedimentation rate, C-reactive protein, Chest X ray, Angiotensin-converting enzyme, and calcium levels were normal. Intralesional steroids 2 weeks apart led to complete resolution of the swelling (Fig. 1B). No recurrence was seen 6 months after injections. Patient, however, presented after 6 months with history of cough. X ray and CT chest did not reveal any abnormality. She is being closely monitored for systemic involvement.
Discussion Sarcoidosis presents with many clinical patterns; bilateral hilar lymphadenopathy, lung involvement, eye lesions, and cutaneous involvement. The diagnosis is usually that of exclusion. Disordered immune regulation in the genetically susceptible individuals, who are usually females (M:F, 1:5) is said to be the pathological basis. A cell mediated response to an unidentified antigen causes the noncaseating granulomas.7 Proposed antigens fall into three categories that include infections, environmental, and autoantigens.8 Evidence of genetic influences are familial and racial clustering of cases and association with certain Human Leucocyte Antigen (HLA) types (e.g., HLA-A1 and HLA B8).7 Cutaneous manifestations are relatively uncommon (20–35%). Some authors divide these into specific and nonspecific lesions. Scar sarcoidosis is considered to be highly specific for skin sarcoidosis.9 Pulmonary involvement is known to be more frequent in patients with lupus perino and scar sarcoidosis than with other skin sarcoidosis variants.1 C 2014 WILEY PERIODICALS, INC. V
Diagnostic Cytopathology DOI 10.1002/dc
SCAR SARCOIDOSIS, FINE NEEDLE ASPIRATION CYTOLOGY
not leave any scar, is not very painful and helps to start an early treatment.
Conclusion
Fig. 1. A and B: Nodule on the forehead, before treatment and after treatment with intralesional steroids. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
In case of a suspicious and/or progressive swelling, a definite diagnosis should be achieved by fine needle aspiration within a short time to enable proper treatment. A rapidly increasing scar should arouse a suspicion for a scar sarcoidosis and FNAC should be considered as a diagnostic procedure before a biopsy is done to clinch the diagnosis, as skin biopsy may leave another scar which may increase the scar sarcoidosis lesion. Diagnosis of these lesions by fine needle aspiration cytology can escalate the diagnosis making and initiation of therapy of scar sarcoidosis without leaving any scar. If scar sarcoidosis is proven, further investigation is necessary to exclude a systemic involvement. A surgical treatment of the swelling is not indicated. Hence, diagnosis of these lesions by fine needle aspiration cytology appears possible with reasonable accuracy. Shivani Sharma, D.C.P., D.N.B. Bhawna Jha, M.D.* Mridula Guleria, M.D. Department of Lab Medicine, Medanta, The Medicity, Sector 38, Gurgaon, Delhi NCR, India Naresh Jain, M.D. Department of Dermatology, Fortis Memorial Research Institute, Sector 44, Gurgaon, India
References Fig. 2. Microphotograph of the aspirate from the skin nodule showing granuloma on Geimsa stain at 403 magnification. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
Scar sarcoidosis has been a great imitator with many differential diagnosis most important being infections like—tuberculosis, leprosy, and spirochete infection, chronic lesions like keloid, foreign body granuloma, crohn’s, and rosacea. FNAC is easily available, handy, and minimally invasive technique which helps in diagnosing the scar sarcoidosis; if a good clinical suspicion is kept. Skin lesions are easily visible and accessible for the procedure. Special stains done on FNA smears help to rule out most of the infections. The agony of a minor Operation Theatre (OT) procedure (biopsy) which is quiet painful is avoided. The irony is that a skin biopsy shall leave another scar which may exacerbate the scar sarcoidosis lesion.6 FNAC does
1. Yanardag H, Pamuk ON, Karayel T. Cutaneous involvement in sarcoidosis: Analysis of the features in 170 patients. Respir Med 2003; 97:978–982. 2. Marchell RM, Judson MA. Chronic cutaneous lesions of sarcoidosis. Clin Dermatol 2007;25:295–302. 3. Fernandez-Faith E, McDonnell J. Cutaneous sarcoidosis: Differential diagnosis. Clin Dermatol 2007;25:276–287. 4. Selim A, Ehrsam E, Atassi MB, Khachemoune A. Scar sarcoidosis: A case report and brief review. Cutis 2006;78;18–422. 5. Rech G, Balestri R, Bardazzi F, Piraccini BM, Patrizi A. Scar reactivation and dry cough. Cleve Clin J Med 2011;78:375–376. 6. Nischal U, Nischal KC, Khopkar U. Techniques of skin biopsy and practical considerations. J Cutan Aesthet Surg 2008;1:107–111. 7. Zissel G, Prasse A, Muller-Quernheim J. Sarcoidosis-immunopathogenetic concepts. Semin Respir Crit Care Med 2007;28:3. 8. Katchar K, Soderstrom K, Wahlstrom J, et al. Characterisation of natural killer cells and CD561 T-cells in sarcoidosis patients. Eur Respir J 2005;26:77–85. 9. Chudomirova K, Velichkova L, Anavi B, Arnaduova M. Recurrent sarcoidosis in skin scars accompanying systemic sarcoidosis. J Eur Acad Dermatol Venereol 2003;17:360–361.
Diagnostic Cytopathology, Vol. 43, No 5
439
Scar Sarcoidosis: A Rare Entity Diagnosed by Fine-Needle Aspiration Cytology Dear Dr. Bedrossian: Scar sarcoidosis is a rare, uncommon but specific cutaneous manifestation of sarcoidosis, occurring in 2.9–29% of overall cases of sarcoidosis.1 It is one of the great dermatologic masqueraders and most surgeons who deal with scars might not be aware of this condition and its different types of appearance. Therefore, appropriate staging and treatment might be missed or at least delayed. It occurs after mechanical harms of the skin like cuts, but even a venous puncture or a tattoo seem to be sufficient to cause it.2–5 Although skin biopsy, which is the most common diagnostic procedure performed in these cases, is usually a safe procedure, complications such as bleeding, infection, and scarring may occasionally be encountered.6 Fine-needle aspiration cytology (FNAC) can be extremely useful as it does not lead to these complications. Also, in case of scar sarcoidosis, further investigation on systemic involvement must be performed as systemic involvement is seen in most patients with scar sarcoidosis.1 To our knowledge, there are not enough reports emphasizing the role of FNAC in diagnosis.
Case Presentation We present the case of a 60 years old lady with asymptomatic, well-defined skin colored swelling, hard in consistency, increasing in size, on right side of forehead for last 1 year (Fig. 1A). The lesion had developed on a previous traumatic scar in recent time. The clinical differential diagnosis was scar sarcoidosis, hypertrophic scar, or benign skin tumour. Patient was reluctant to undergo excision or incision biopsy so FNAC was done from the
*Correspondence to: Dr Bhawna Jha, M.D.; B 10, Chattarpur Enclave, Phase II, New Delhi 110074, India. E-mail: [email protected] Conflict of interest: Nothing to report Received 17 June 2014; Revised 18 July 2014; Accepted 6 October 2014 DOI: 10.1002/dc.23215 Published online 24 October 2014 in Wiley Online Library (wileyonlinelibrary.com).
438
Diagnostic Cytopathology, Vol. 43, No 5
lesion. The smears were composed of epitheloid granulomas with no evidence of necrosis (Fig. 2). To rule out an infective lesion Ziehl–Neelson stain for leprae and tubercular organism was done, and they were negative. Keeping in view the clinical details and the morphology, a cytological diagnosis of “Scar Sarcoidosis” was given. Further investigations were done to rule out tubercular etiology or any systemic involvement. Mantoux test was negative, Erythrocyte sedimentation rate, C-reactive protein, Chest X ray, Angiotensin-converting enzyme, and calcium levels were normal. Intralesional steroids 2 weeks apart led to complete resolution of the swelling (Fig. 1B). No recurrence was seen 6 months after injections. Patient, however, presented after 6 months with history of cough. X ray and CT chest did not reveal any abnormality. She is being closely monitored for systemic involvement.
Discussion Sarcoidosis presents with many clinical patterns; bilateral hilar lymphadenopathy, lung involvement, eye lesions, and cutaneous involvement. The diagnosis is usually that of exclusion. Disordered immune regulation in the genetically susceptible individuals, who are usually females (M:F, 1:5) is said to be the pathological basis. A cell mediated response to an unidentified antigen causes the noncaseating granulomas.7 Proposed antigens fall into three categories that include infections, environmental, and autoantigens.8 Evidence of genetic influences are familial and racial clustering of cases and association with certain Human Leucocyte Antigen (HLA) types (e.g., HLA-A1 and HLA B8).7 Cutaneous manifestations are relatively uncommon (20–35%). Some authors divide these into specific and nonspecific lesions. Scar sarcoidosis is considered to be highly specific for skin sarcoidosis.9 Pulmonary involvement is known to be more frequent in patients with lupus perino and scar sarcoidosis than with other skin sarcoidosis variants.1 C 2014 WILEY PERIODICALS, INC. V
Diagnostic Cytopathology DOI 10.1002/dc
SCAR SARCOIDOSIS, FINE NEEDLE ASPIRATION CYTOLOGY
not leave any scar, is not very painful and helps to start an early treatment.
Conclusion
Fig. 1. A and B: Nodule on the forehead, before treatment and after treatment with intralesional steroids. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
In case of a suspicious and/or progressive swelling, a definite diagnosis should be achieved by fine needle aspiration within a short time to enable proper treatment. A rapidly increasing scar should arouse a suspicion for a scar sarcoidosis and FNAC should be considered as a diagnostic procedure before a biopsy is done to clinch the diagnosis, as skin biopsy may leave another scar which may increase the scar sarcoidosis lesion. Diagnosis of these lesions by fine needle aspiration cytology can escalate the diagnosis making and initiation of therapy of scar sarcoidosis without leaving any scar. If scar sarcoidosis is proven, further investigation is necessary to exclude a systemic involvement. A surgical treatment of the swelling is not indicated. Hence, diagnosis of these lesions by fine needle aspiration cytology appears possible with reasonable accuracy. Shivani Sharma, D.C.P., D.N.B. Bhawna Jha, M.D.* Mridula Guleria, M.D. Department of Lab Medicine, Medanta, The Medicity, Sector 38, Gurgaon, Delhi NCR, India Naresh Jain, M.D. Department of Dermatology, Fortis Memorial Research Institute, Sector 44, Gurgaon, India
References Fig. 2. Microphotograph of the aspirate from the skin nodule showing granuloma on Geimsa stain at 403 magnification. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
Scar sarcoidosis has been a great imitator with many differential diagnosis most important being infections like—tuberculosis, leprosy, and spirochete infection, chronic lesions like keloid, foreign body granuloma, crohn’s, and rosacea. FNAC is easily available, handy, and minimally invasive technique which helps in diagnosing the scar sarcoidosis; if a good clinical suspicion is kept. Skin lesions are easily visible and accessible for the procedure. Special stains done on FNA smears help to rule out most of the infections. The agony of a minor Operation Theatre (OT) procedure (biopsy) which is quiet painful is avoided. The irony is that a skin biopsy shall leave another scar which may exacerbate the scar sarcoidosis lesion.6 FNAC does
1. Yanardag H, Pamuk ON, Karayel T. Cutaneous involvement in sarcoidosis: Analysis of the features in 170 patients. Respir Med 2003; 97:978–982. 2. Marchell RM, Judson MA. Chronic cutaneous lesions of sarcoidosis. Clin Dermatol 2007;25:295–302. 3. Fernandez-Faith E, McDonnell J. Cutaneous sarcoidosis: Differential diagnosis. Clin Dermatol 2007;25:276–287. 4. Selim A, Ehrsam E, Atassi MB, Khachemoune A. Scar sarcoidosis: A case report and brief review. Cutis 2006;78;18–422. 5. Rech G, Balestri R, Bardazzi F, Piraccini BM, Patrizi A. Scar reactivation and dry cough. Cleve Clin J Med 2011;78:375–376. 6. Nischal U, Nischal KC, Khopkar U. Techniques of skin biopsy and practical considerations. J Cutan Aesthet Surg 2008;1:107–111. 7. Zissel G, Prasse A, Muller-Quernheim J. Sarcoidosis-immunopathogenetic concepts. Semin Respir Crit Care Med 2007;28:3. 8. Katchar K, Soderstrom K, Wahlstrom J, et al. Characterisation of natural killer cells and CD561 T-cells in sarcoidosis patients. Eur Respir J 2005;26:77–85. 9. Chudomirova K, Velichkova L, Anavi B, Arnaduova M. Recurrent sarcoidosis in skin scars accompanying systemic sarcoidosis. J Eur Acad Dermatol Venereol 2003;17:360–361.
Diagnostic Cytopathology, Vol. 43, No 5
439
