|Year : 2012 | Volume
| Issue : 4 | Page : 123
Darier-Roussy sarcoidosis with lung involvement: One of the very rare cases in the world
Department of Pulmonary Medicine, Osmania Medical College, AP Chest Hospital, Hyderabad, Andhra Pradesh, India
|Date of Web Publication||19-Sep-2014|
Department of Pulmonary Medicine, Osmania Medical College, AP Chest Hospital, Hyderabad - 500 038, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Kandi S. Darier-Roussy sarcoidosis with lung involvement: One of the very rare cases in the world. J Acad Med Sci 2012;2:123
Sarcoidosis (flesh-like process) also called sarcoid or Besnier-Boeck disease is a multisystem disorder of unknown etiology, which is characterized by noncaseating granulomas (small inflammatory nodules) mainly involving the lungs (90%), lymph nodes, eyes, and skin. The first reported case bearing the clinicopathological features of subcutaneous sarcoidosis was described by Darier and Roussy in 1904. Subcutaneous sarcoidosis also known as "Darier-Roussy sarcoid" is a cutaneous condition characterized by numerous 0.3-0.5 cm deep-seated nodules on the trunk and extremities. 
There is an acute presentation of sarcoidosis called Lofgren syndrome, associated with hilar adenopathy, Bell's palsy, fever, and erythema nodosum. Erythema nodosum is another cutaneous manifestation of sarcoidosis.
A case of Darier-Roussy sarcoid mimicking breast cancer was reported in the literature. Sarcoidosis and breast carcinoma are two distinct diseases that affect women.  They can present either at the same time or in a sequence. Both diseases can present with pulmonary nodules, which mimic metastasis misleading the diagnosis in case of sarcoid. Subcutaneous lesions in systemic sarcoidosis are well-known, but uncommon. Rarely, subcutaneous sarcoidosis occurs in patients without evidence of systemic disease. ,
Chen et al.  reported three cases of breast cancer with coexistent pulmonary nodules which on biopsy revealed pulmonary sarcoidosis. Tolaney et al.  also had the same clinical situation with pulmonary nodules in advanced breast cancer, which proved to be sarcoid lesions on tissue biopsy. An unfortunate consequence of the presence of both entities in the same patient is the risk of misdiagnosis and incorrect treatment. To avoid such mishaps it is always better to obtain a tissue biopsy of the suspicious metastatic lesions.
According to Indian literature, Sharma and Mohan.  mentioned that sarcoidosis is an underdiagnosed disease in India. However, now it is being diagnosed more frequently, and sarcoidosis usually a benign disease except in rare conditions. Sharma et al.  mentioned 54 rare manifestations of sarcoidosis, which includes various systems of the body. Sharma and Mohan.  also reviewed various rare manifestations of the sarcoidosis, which includes two patients with Grade IV esophageal varices, one of them succumb to massive hematemesis, another one successfully treated with endoscopic sclerotherapy.
The definitive diagnosis can be made after histopathological examination. Treatment will be same as per other forms of sarcoidosis, that is, observation or corticosteroids or immunosuppressasnts. Sharma et al.,  also mentioned various manifestations of sarcoidosis, in addition to steroid response and relapses. The independent predictors of relapse found to be history of malaise, presence of crepitations, wheezing, peripheral eosinophilia and forced expiratory volume in 1 second/forced vital capacity% <65%.
| References|| |
|1.||James WD, Berger TG. Andrews' Diseases of the Skin: Clinical Dermatology. Philadelphia PA: Saunders Elsevier; 2006. |
|2.||Chen W, Miller RA, Hebbe KA, Guron GK. Sarcoidosis and breast carcinoma: Three case reports and a review. J Clin Oncol 2004;22 Suppl:867. |
|3.||Mingins C, Williams MR, Cox NH. Subcutaneous sarcoidosis mimicking breast carcinoma. Br J Dermatol 2002;146:924-5. |
|4.||Whittington R, Lazarus A, Nerenstone S, Martin A. Sarcoidosis developing during therapy for breast cancer. Chest 1986;89:762-3. |
|5.||Tolaney SM, Colson YL, Gill RR, Schulte S, Duggan MM, Shulman LN, et al. Clin Breast Cancer 2007;7:804-10. |
|6.||Sharma SK, Mohan A. Sarcoidosis: Global scenario and Indian perspective. Indian J Med Res 2002;116:221-47. |
|7.||Sharma SK, Soneja M, Sharma A, Sharma MC, Hari S. Rare manifestations of sarcoidosis in modern era of new diagnostic tools. Indian J Med Res 2012;135:621-9. |
|8.||Sharma SK, Mohan A. Uncommon manifestations of sarcoidosis. J Assoc Physicians India 2004;52:210-4. |
|9.||Sharma SK, Mohan A, Guleria JS. Clinical characteristics, pulmonary function abnormalities and outcome of prednisolone treatment in 106 patients with sarcoidosis. J Assoc Physicians India 2001;49:697-704. |