|Year : 1993 | Volume
| Issue : 4 | Page : 190-193
RS Mishra, C Indira, U Saxena, A Mukherjee
R S Mishra
Source of Support: None, Conflict of Interest: None
Two cases of sarcoidosis with cutaneous lesions as presenting complaints are reported. Clinicians should have a high index of suspicion for sarcoidosis since it mimics other granulomatous skin conditions.
Keywords: Sarcoidosis, Lupus pernio
|How to cite this article:|
Mishra R S, Indira C, Saxena U, Mukherjee A. Cutaneous sarcoidosis. Indian J Dermatol Venereol Leprol 1993;59:190-3
| Introduction|| |
Sarcoidosis, a multi-system granulomatous disorder of unknown aetiology, is seen more commonly in women than men with a predilection for coloured races. The granulomatous process can involve one or more organs simultaneously. The signs and symptoms depend mainly on the clinical stage of disease, organ involved and activity of disease. The commonest mode of presentation is with chest signs and symptoms. Sarcoidosis is thought to be in eclipse where diseases like tuberculosis and leprosy are prevalent  as in India. Only 300 cases of sarcoidosis have been reported in India till 1986. 
| Case Reports|| |
Case 1 (patient A) : A 60-year-old lady presented with asymptomatic skin lesions on the face, neck and body of two and half years duration. Lesions on the nose were progressive and the other lesions were relatively stationary. No aggravating or relieving factors. Patient had not received any treatment for her skin condition. No history of fever, cough with expectoration or nasal discharge. She had a diffuse swelling of the thyroid gland since childhood without any history suggestive of thyroid dysfunction. Pain and swelling of small joints of both hands were present since past 5 years and were relieved with antiinflammatory drugs. She was not a known hypertensive or diabetic. She developed post-traumatic blindness in her right eye and underwent an operation for senile cataract in her left eye seven years ago. Family history and personal history were non-contributory.
Cutaneous examination revealed multiple, erythematous papules and nodules on the face, neck, front and back of chest. A large erythematous plaque was present on the nose without involvement of the septum or nasal mucosa. Lesions of the skin had no accompanying sensory impairment or peripheral nerve thickening. Mucosae, hair and nails were normal. Two lymph nodes belonging to the deep cervical group were enlarged, mobile, discrete and non-tender. Swelling and restriction of movement was seen in all the small joints of the hands, especially in the right 3rd and 5th proximal interphalangeal joints and the left 4th interphalangeal joint. No clinical signs of thyroid disease were evident. Ophthalmological examination revealed a painless right eye and a corneal opacity in the left eye. The other systems were normal on examination.
Routine urine examination, haemogram, blood urea and sugar, serum electrolytes and calcium were within normal limits. ESR was raised- 45 mm in 1st hour. Slit skin smears for AFB and LD bodies were negative. Mx test with 10 tuberculin units was negative. 24 hour urinary calcium was markedly raised - 990mg (normal-200mg). X-ray chest showed calcified mass in superior mediastinum with interstitial fibrosis in lung. X-ray hands showed cystic lesions in terminal phalanges and metacarpals corresponding to the joint involvement clinically. X-ray feet-osteoarthritic changes in the ankle joint. Barium swallow- no obstruction to oesophagus; ultrasound study of abdominal viscera was normal. Thyroid function tests- WNL.
Biopsy of skin lesion on the neck and lymph node on histopathological examination showed numerous focal epitheloid granulomas with fibrous network, demonstrated with reticulin stains.
Case 2 (patient B) : A 50-year-old lady presented with mildly pruritic, slowly progressive skin lesions of insidious onset of two years duration. There was no history of fever, cough with expectoration and joint pains. The patient was not a known diabetic or hypertensive. Before coming to us she had been biopsied twice for her skin condition, which were reported as lupus vulgaris and granuloma annulare respectively, for which she had received treatment with anti-tubercular drugs and topical steroids with little response. Cutaneous examination revealed multiple erythematous papules and nodules on the face, scalp, back and dorsae of hands with no sensory deficit or peripheral nerve thickening. No nail or mucosal involvement. Scalp lesions showed loss of hair and fine scaling. Other systems were normal. We repeated a skin biopsy which showed involvement with sarcoid granuloma. Other investigations supporting the diagnosis were a raised ESR of 38 mm 1st hour and 24 hour urine calcium of 1184 mg. Radiological involvement of lung and bone was present. X-ray chest showed bilateral hilar lymphadenopathy with nodular opacities in both lung fields with areas of cavitation and of hands showed cystic lesions in terminal phalanges of both hands.
| Comments|| |
Cutaneous involvement in sarcoidosis can be specific where a sarcoid granuloma is demonstrable and non-specific where a granuloma is absent. The specific types of lesions are lupus pernio, small and large nodules, small and large plaques and scar infiltrations. Lupus pernio is the most characteristic of sarcoid skin lesion and is seen as a chronic, persistent bluish indurated plaque with a predilection for nose, cheek with nasal mucosal and septal involvement.
Patient A had the classical lupus pernio lesion on the nose with no septal or mucosal involvement and patient B had the small nodular type of infiltration. Lupus pernio is commonly associated with eye and bone involvement. Bone involvement was seen in patient A but blindness in one eye and opacity in the other eye could not be attributed to the disease. Bone involvement is usually symptomless unless sought specifically and classically involves the small joints of hands and feet causing cystic changes. In both the patients bone lesions were seen. In patient A, the thyroid swelling was unrelated to her disease as dysfunction which is a signal of gland involvement was absent. Hypercalcaemia and hypercalciurea are usually seen associated with activity of the disease and are reversible with treatment. In patient B, the 24 hour urinary calcium levels dropped from 1184 mg to 274 mg with 3 months of therapy.
Both the patients revealed histopathologically focal granulomas surrounded by peri-granulomatous network in patient A deep cervical lymph node also revealed a non-caseating granuloma. In the histological diagnosis lupus vulgaris, tuberculoid leprosy, foreign body reaction and granuloma annulare may pose difficulties. The diagnosis of sarcoidosis has been made wrongly several times in cases of leprosy 3 and cases of sarcoidosis were erroneously treated for leprosy , Sarcoidal reaction is seen in skin diseases like tuberculoid leprosy, dermal leishmaniasis, non-ulcerating fungal infections and papular and nodular syphilids. In patient B due to lack of index of suspicion for sarcoidosis the histopathology was initially reported as lupus vulgaris. A repeat biopsy was performed due to lack of response to treatment and a review with clinical and radiological data established the diagnosis of sarcoidosis. Kveim's test, where available, helps in the reproduction of the epitheloid granuloma at the injected site but reversal of biochemical parameters following treatment is confirmatory of the disease.
In both the patients steroid therapy resulted in flattening and clearing of skin lesions within eight weeks. Relapses are common, both the patients relapsed after a remission of three months and needed a maintenance dose of 5mg of prednisolone on alternate day. Chloroquine phosphate has been reported to be a successful alternative therapy for persistent skin lesions and lupus pernio,  but regular ophthalmological examinations are mandatory to detect retinopathy.
| References|| |
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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]