A 50 year-old African-American female presented with a chief complaint of lesions around the nose and eyes. She had a 17-year history of sarcoidosis. The initial diagnosis was based on the presence of sarcoid iritis, and her course included pulmonary and upper respiratory tract involvement. Upon examination, she appeared healthy with multiple 1-2 mm nontender, shiny, smooth and violaceous papules bilaterally along the epicanthal folds and larger papules on the nasal rim.(Figure 1) These lesions were firm, nonpruritic and nonulcerating. DIAGNOSING THE CONDITION Criteria for diagnosis of sarcoidosis include: • compatible clinical and/or radiologic picture; • histologic evidence of noncaseating granuloma; and • negative special stains and cultures for other disease entities.1 Cutaneous sarcoidosis occurs in 20 to 25% of patients and presents most often at the early onset of the disease process.2,3,4 Lupus pernio is characterized by cutaneous lesions involving the nose, cheeks, lips, forehead and ears. Three quarters of individuals with lupus pernio exhibit chronic fibrotic respiratory tract disease and warrant a complete ENT examination. A nasal lesion is strongly associated with granulomatous infiltration of the nasal mucosa and respiratory tract.1 Additionally, about 43% of lupus pernio patients have granulomas in bones, most frequently located in the fingers, which results in a sausage-shaped digit.5 Although lupus pernio and cutaneous sarcoidosis may occur without systemic involvement, studies indicate a high predilection to develop systemic features in the future. Patients should receive periodic screening. We recommend that individuals undergo a thorough evaluation, including a chest x-ray, pulmonary function tests, diffusion capacity of carbon monoxide testing (or an ABG) and tuberculosis skin testing. Also, include a further work up of serum calcium levels, renal function and hepatic function tests and EKG, slitlamp and fundoscopic examination. Additionally, X-rays for bony involvement or a neurological evaluation may be indicated in specific symptomatic patients. TREATMENT Various cutaneous sarcoidosis treatment modalities have been reported as beneficial. Lupus pernio typically does not spontaneously remit. Young et al. recently recommended the following order for initiation of treatment modalities:2 1. Topical steroid (mid-strength) 2. Pulse ultrapotent topical corticosteroid (week ends only) 3. Daily class II topical steroid 4. Antimalarial 5. Methotrexate or prednisone 6. Other therapy based on anecdotal success At the time of presentation, the patient’s pulmonologist felt she did not require systemic corticosteroids for her internal disease. Given the localized nature of the perinasal involvement, intralesional corticosteroid injection was planned. Before the injection, however, the lesions responded to a 10-day treatment with a combination of topical clobetasol propionate and topical tacrolimus 0.1% ointment.(Figure 2)
SARCOIDOSIS/LUPUS PERNIO
A 50 year-old African-American female presented with a chief complaint of lesions around the nose and eyes. She had a 17-year history of sarcoidosis. The initial diagnosis was based on the presence of sarcoid iritis, and her course included pulmonary and upper respiratory tract involvement. Upon examination, she appeared healthy with multiple 1-2 mm nontender, shiny, smooth and violaceous papules bilaterally along the epicanthal folds and larger papules on the nasal rim.(Figure 1) These lesions were firm, nonpruritic and nonulcerating. DIAGNOSING THE CONDITION Criteria for diagnosis of sarcoidosis include: • compatible clinical and/or radiologic picture; • histologic evidence of noncaseating granuloma; and • negative special stains and cultures for other disease entities.1 Cutaneous sarcoidosis occurs in 20 to 25% of patients and presents most often at the early onset of the disease process.2,3,4 Lupus pernio is characterized by cutaneous lesions involving the nose, cheeks, lips, forehead and ears. Three quarters of individuals with lupus pernio exhibit chronic fibrotic respiratory tract disease and warrant a complete ENT examination. A nasal lesion is strongly associated with granulomatous infiltration of the nasal mucosa and respiratory tract.1 Additionally, about 43% of lupus pernio patients have granulomas in bones, most frequently located in the fingers, which results in a sausage-shaped digit.5 Although lupus pernio and cutaneous sarcoidosis may occur without systemic involvement, studies indicate a high predilection to develop systemic features in the future. Patients should receive periodic screening. We recommend that individuals undergo a thorough evaluation, including a chest x-ray, pulmonary function tests, diffusion capacity of carbon monoxide testing (or an ABG) and tuberculosis skin testing. Also, include a further work up of serum calcium levels, renal function and hepatic function tests and EKG, slitlamp and fundoscopic examination. Additionally, X-rays for bony involvement or a neurological evaluation may be indicated in specific symptomatic patients. TREATMENT Various cutaneous sarcoidosis treatment modalities have been reported as beneficial. Lupus pernio typically does not spontaneously remit. Young et al. recently recommended the following order for initiation of treatment modalities:2 1. Topical steroid (mid-strength) 2. Pulse ultrapotent topical corticosteroid (week ends only) 3. Daily class II topical steroid 4. Antimalarial 5. Methotrexate or prednisone 6. Other therapy based on anecdotal success At the time of presentation, the patient’s pulmonologist felt she did not require systemic corticosteroids for her internal disease. Given the localized nature of the perinasal involvement, intralesional corticosteroid injection was planned. Before the injection, however, the lesions responded to a 10-day treatment with a combination of topical clobetasol propionate and topical tacrolimus 0.1% ointment.(Figure 2)
A 50 year-old African-American female presented with a chief complaint of lesions around the nose and eyes. She had a 17-year history of sarcoidosis. The initial diagnosis was based on the presence of sarcoid iritis, and her course included pulmonary and upper respiratory tract involvement. Upon examination, she appeared healthy with multiple 1-2 mm nontender, shiny, smooth and violaceous papules bilaterally along the epicanthal folds and larger papules on the nasal rim.(Figure 1) These lesions were firm, nonpruritic and nonulcerating. DIAGNOSING THE CONDITION Criteria for diagnosis of sarcoidosis include: • compatible clinical and/or radiologic picture; • histologic evidence of noncaseating granuloma; and • negative special stains and cultures for other disease entities.1 Cutaneous sarcoidosis occurs in 20 to 25% of patients and presents most often at the early onset of the disease process.2,3,4 Lupus pernio is characterized by cutaneous lesions involving the nose, cheeks, lips, forehead and ears. Three quarters of individuals with lupus pernio exhibit chronic fibrotic respiratory tract disease and warrant a complete ENT examination. A nasal lesion is strongly associated with granulomatous infiltration of the nasal mucosa and respiratory tract.1 Additionally, about 43% of lupus pernio patients have granulomas in bones, most frequently located in the fingers, which results in a sausage-shaped digit.5 Although lupus pernio and cutaneous sarcoidosis may occur without systemic involvement, studies indicate a high predilection to develop systemic features in the future. Patients should receive periodic screening. We recommend that individuals undergo a thorough evaluation, including a chest x-ray, pulmonary function tests, diffusion capacity of carbon monoxide testing (or an ABG) and tuberculosis skin testing. Also, include a further work up of serum calcium levels, renal function and hepatic function tests and EKG, slitlamp and fundoscopic examination. Additionally, X-rays for bony involvement or a neurological evaluation may be indicated in specific symptomatic patients. TREATMENT Various cutaneous sarcoidosis treatment modalities have been reported as beneficial. Lupus pernio typically does not spontaneously remit. Young et al. recently recommended the following order for initiation of treatment modalities:2 1. Topical steroid (mid-strength) 2. Pulse ultrapotent topical corticosteroid (week ends only) 3. Daily class II topical steroid 4. Antimalarial 5. Methotrexate or prednisone 6. Other therapy based on anecdotal success At the time of presentation, the patient’s pulmonologist felt she did not require systemic corticosteroids for her internal disease. Given the localized nature of the perinasal involvement, intralesional corticosteroid injection was planned. Before the injection, however, the lesions responded to a 10-day treatment with a combination of topical clobetasol propionate and topical tacrolimus 0.1% ointment.(Figure 2)
