Year : 2009 | Volume
: 54 | Issue : 5 | Page : 80--82
Cutaneous sarcoidosis without systemic manifestations
Rajesh Mohanty1, SN Singh2, AB Bhattamishra2,
1 Department of Pathology, Tata Main Hospital, Jamshedpur, Jharkhand, India
2 Department of Dermatology, Tata Main Hospital, Jamshedpur, Jharkhand, India
12, New TC Colony, Road No. 1, Kadma, Jamshedpur - 831 005
A 38-year-old lady, housewife by profession presented with mildly pruritic polymorphic lesions on various parts of her body. The lesions appeared as smooth, violaceous and round papules. Histopathological examination showed classical features of cutaneous sarcoidosis. She had no systemic involvement. Cutaneous sarcoidosis has many morphological presentations and often mimics other dermatologic diseases. Treatment options are reviewed.
|How to cite this article:|
Mohanty R, Singh S N, Bhattamishra A B. Cutaneous sarcoidosis without systemic manifestations.Indian J Dermatol 2009;54:80-82
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Mohanty R, Singh S N, Bhattamishra A B. Cutaneous sarcoidosis without systemic manifestations. Indian J Dermatol [serial online] 2009 [cited 2021 Sep 21 ];54:80-82
Available from: https://www.e-ijd.org/text.asp?2009/54/5/80/45467
Sarcoidosis is a systemic disease that can involve almost any organ system. Infiltration with noncaseating granulomas is the hallmark of the disease, and it may result in various clinical manifestations. The underlying cause of sarcoidosis remains unknown. Cutaneous involvement occurs in 20 to 35 percent of patients with systemic sarcoidosis but sarcoidosis confined to skin only is quite uncommon. ,,, Because lesions assume a vast array of morphologies, cutaneous sarcoidosis is known as one of the 'great imitators' in dermatology. Involvement may be mild to severe, self-limited or chronic, and limited or wide-ranging in extent.
A 38-year-old housewife presented with a three months history of few small, slightly pruritic lesions on the nape of her neck, face, ears, waist and legs. The lesions gradually increased in size and number. She denied history of pain or burning sensation. The lesions were skin colored, erythematous, violaceous, lichenoid, shiny papules and plaques. Some of the papules were umbilicated. The papules ranged in size from 2mm to 5mm. In some areas, the papules had coalesced into linear streaks [Figure 1].
There was no history of fever, chill, arthralgia, weight loss, cough or visual disturbances. There was no history of insect bite, trauma or any other systemic complaints.
Past and family history was not contributory. There was no significant drug history. General and systemic examinations revealed no abnormality.
A complete blood count with differential analysis, liver function tests, renal function tests, serum calcium, HIV, VDRL, skin slit smear for acid fast bacilli, routine urine and stool examinations were normal. Erythrocyte sedimentation rate (westergren method) was 28mm in first hour. The total cholesterol and low-density lipoprotein were mildly elevated at 219mg/dl and 151mg/dl respectively. Serum ACE and bronchoalveolar lavage for CD4/CD8 ratio were not done due to non availability of facilities. Radiological examination of chest, hands and feet, ophthalmologic examination, pulmonary function tests revealed no abnormalities.
Histopathological examination of the skin lesion showed noncaseating epitheloid granulomas and a mild infiltrate of lymphocytes arranged in a band like pattern in the papillary dermis. The granulomas were composed of epitheloid cells, langhans type of giant cells and lymphocytes. The overlying epidermis was stretched but intact [Figure 2],[Figure 3]. No caseation necrosis seen. Acid fast stain was negative. Periodic acid Schiff stain for fungal granuloma was negative. A final diagnosis of cutaneous sarcoidosis was made.
The patient was first treated with topical Clobetasol cream twice daily for one month and similarly with Flucinolone acetonide 0.15 for another month without much relief.
She was treated with intralesional steroid triamcinolone acetonide injection in a dosage of 40mg/ml. Visible improvement with reduction in size and color of the papules was observed after the first injection. This therapy was repeated three times at two-week intervals and most of the lesions improved significantly. Her face and neck lesions responded to only one injection while her waist and leg lesions required a minimum of two to a maximum of three sittings.
Sarcoidosis commonly affects the lungs, lymph nodes, liver, eyes, bones and skin. In patients with systemic disease, the skin is involved in 20 to 30 percent of cases. However, cutaneous lesions are the sole manifestation of sarcoidosis in approximately 10 percent of patients. 
Sarcoid is a great imitator of other dermatologic diseases because the cutaneous manifestations are quite variable and occur in both localized as well as generalized forms. Among the numerous reported morphological presentations are papules, micopapules, plaques, subcutaneous nodules,  scar sarcoidosis, lupus pernio, erythema nodosum, ulcer and alopecia. Follicular, verrucous, ichthyosiform, hypomelanotic, psoriasiform and annular lesions have also been described.  Moreover, the epidermis may be relatively unaffected in subcutaneous nodules of sarcoid or it may be thin, hyperplastic, discolored, telangiectatic, or scaly.  Sarcoid lesions are typically asymptomatic, but pruritus is present in approximately 10-15 percent of patients. 
Although there are numerous therapeutic options for sarcoid, little data exists regarding which therapy is best suited for the various cutaneous manifestations. Systemic glucocorticoids are generally beneficial for all patients with sarcoid, although long-term use of high doses is not recommended. Intralesional injections of triamcinolone acetonide is suited for limited skin disease,  so also is phonophoresis.  In case of extensive skin involvement, chloroquine and hydroxychloroquine have been used, with 75 percent of patients responding either partially or completely.  Methotrexate in doses of 7.5-25mg weekly has been efficacious in treating sarcoid. However, it has been reported that methotrexate induced hepatitis occurs in 15 percent of patients with sarcoidosis. 
Cutaneous sarcoidosis has been reported occasionally from India and in most of these reports, lesions were associated with some form of systemic involvement.  Therefore patients presenting with disease confined to skin alone, should be followed-up regularly for the probable risk of developing systemic manifestations at a later date. We asked her to attend chest, eye and skin clinics every three months. Considering the hazards of frequent exposure to radiation we planned her X-ray chest to be done if she complained of respiratory symptoms for more than 15 days at a stretch.
This case has been reported for the unusual presence of itching, though mild, and the rather rare appearance of umbilicated papules.
|1||Samtsov AV. Cutaneous sarcoidosis. Int J Dermatol 1992;31:385-91.|
|2||Mishra RS, Indira C, Saxena U, Mukherjee. A. Short communication: Cutaneous sarcoidosis. Indian J Dermatol Venereol Leprol 1993;59:4.|
|3||Singh SK, Singh S, Pandey SS. Cutaneous sarcoidosis without systemic involvement: Response to intralesional corticosteroid. Indian J Dermatol Venereol Leprol 1996;62:4.|
|4||Mahajan VK, Sharma NL, Sharma RC, Sharma VC. Cutaneous sarcoidosis: Clinical profile of 23 Indian patients. Indian JDermatol Venereol Leprol 2007;73:1.|
|5||Sarcoidosis. Odom RB, et al , editors. In : Andres' diseases of the skin, 9 th ed. Philadelphia: WB Saunders Company; 2000. p. 896.|
|6||Gangopadhyay AK. Subcutaneous sarcoidosis: A case report. Indian J Dermatol 1999;44:4.|
|7||Fujii K, Okamoto H, Onuki M, Horio T. Recurrent follicular and lichenoid papules of sarcoidosis. Eur J Dermatol 2000;10:303-5.|
|8||Okamoto H. Epidermal changes in cutaneous lesions of sarcoidosis. Am J Dermatopathol 1999;21:229-33.|
|9||Bachelez H, Senet P, Cadranel J, Kaoukhov A, Dubertret L. The use of tetracyclines for the treatment of sarcoidosis. Arch Dermatol 2001;137:69-73.|
|10||Gogstetter DS, Goldsmith LA. Treatment of cutaneous sarcoidosis using phonophoresis. J Am Acad Dermatol. 1999; 40:767-9|
|11||Zie JA, Horowitz DH, Arzubiaga C, King LE Jr. Treatment of cutaneous sarcoidosis with chloroquine. Arch Dermatol 1991;127:1034-40.|
|12||Webster GF, Razsi LK, Sanchez M, Shupack JR. Methotrexate therapy in cutaneous sarcoidosis. Ann Intern Med 1989;111:538-9.|
|13||Singh G, Sangolli P, Dash K, Grove S. Cutaneous sarcoidosis without systemic involvement. Indian J Dermatol Venereol Leprol 2000;66:43-4.|