Subcutaneous Sarcoidosis in a Nigerian female

Introduction Sarcoidosis is a chronic multisystem disorder of unknown etiology characterized by formation of non caseating epitheloid granulomas and derangement of normal tissue architecture (1,2). It can affect all organs of the body but intrathoracic involvement (pulmonary and hilar nodes) occurs in about 90% of cases (3). It is a world wide disease with regional variation in incidence, affecting both genders with slight female preponderance (4). All age groups are affected with bimodal peaks between 25-35 years and 45-65 years (4). In the West, the disease is quite common in persons of black descent, the Scandinavians, and the Irish. African Americans (especially women) are more severely affected than whites (5). Sarcoidosis is said to be rare in indigenous Africans, but there are now well validated reports of many clinical cases in Africa (6,7). Africans are thought to present most commonly with extrapulmonary sarcoidosis albeit in late stages. Skin sarcoidosis can arise in isolation or as a component of a systemic disease. There has not been any report on sarcoidosis from our center. This case is being reported to highlight this unusual skin presentation.


Introduction
Sarcoidosis is a chronic multisystem disorder of unknown etiology characterized by formation of non caseating epitheloid granulomas and derangement of normal tissue architecture (1,2). It can affect all organs of the body but intrathoracic involvement (pulmonary and hilar nodes) occurs in about 90% of cases (3). It is a world wide disease with regional variation in incidence, affecting both genders with slight female preponderance (4). All age groups are affected with bimodal peaks between 25-35 years and 45-65 years (4). In the West, the disease is quite common in persons of black descent, the Scandinavians, and the Irish. African Americans (especially women) are more severely affected than whites (5). Sarcoidosis is said to be rare in indigenous Africans, but there are now well validated reports of many clinical cases in Africa (6,7). Africans are thought to present most commonly with extrapulmonary sarcoidosis albeit in late stages. Skin sarcoidosis can arise in isolation or as a component of a systemic disease.
There has not been any report on sarcoidosis from our center. This case is being reported to highlight this unusual skin presentation.

Case Report
A 65 year old married woman presented with a swelling on the anterior surface of her right ankle joint of six Subcutaneous Sarcoidosis in a Nigerian female Summary Sarcoidosis is relatively uncommon in indigenous black Africans, especially along the West African coast. There has not been any report of isolated cutaneous sarcoidosis from our center. We present a patient who was reviewed with an isolated ganglion-like subcutaneous swelling on the ankle region. Excision biopsy revealed a non caseating granulomatous lesion. Systemic evaluation was not remarkable and the lesion was negative for tuberculosis and fungi. The operative wound healed with the use of of a combination therapy with oral chloroquine, prednisolone, and methotrexate. We conclude that cutenous sarcoidosis can occur in the absence of systemic disease and definitive diagnosis is by histological finding of a non caseating granuloma, when other potential causes such as tuberculosis are excluded. Hydroxychloroquine or chloroquine has been proved to be effective in selected cases of chronic skin sarcoidosis; similarly, cytotoxic agents such as methotrexate, cyclophosphamide, and azathioprine can be used (11).
To achieve rapid control of the disease, and reduce the probable complications of prolonged single agent therapy, combination of therapies were used successfully in our patient.
Sarcoidosis can be self limiting or life threatening. The overall prognosis of cutaneous sarcoidosis is primarily dependent on the degree of systemic involvement (1).
Mortality is about 5% and results from pulmonary (progressive pulmonary fibrosis, pulmonary hypertension and right ventricular failure), cardiac (arrhythmias and cardiac failure) and neurologic involvement. We intend to keep following up our patient for any evidence of systemic involvement (10).
In conclusion, a high index of suspicion is necessary for diagnosis of cutaneous sarcoidosis after excluding other causes of noncaseating, non sarcoid lesions. A combination therapy is often helpful in controlling the disease and preventing reccurrence.