A 37-year-old African American woman with a history of biopsy-proven cutaneous sarcoidosis presented with what she described as a “rash” on her nose. The rash was red, tender, and mild in severity, and it had been present for 7 years. The patient denied any joint aches, recent infections, fevers, chills, and blisters, and she was not on any new medications or personal care products. She also was not on any current treatments for this condition.
Physical examination showed an erythematous plaque and papules on the nasal tip.
Answer: Lupus pernioSee the full case at Consultant360
Based on her history and clinical presentation, the woman received a diagnosis of lupus pernio.
Lupus pernio is a cutaneous variant of sarcoidosis, a disease in which abnormal groupings of inflammatory cells (granulomas) can form in multiple organs of the body. While the lungs are the most common site of infection, skin manifestations occur in approximately 25% of patients.1 Sarcoidosis is predisposed in individuals 20 to 40 years of age and in those of Northern European or African American descent.2 However, lupus pernio specifically is much more common among African American women.3,4
The condition is characterized by indurated red, brown, and purple papules around the nose, eyelids, cheeks, lips, ears, and fingers. Such lesions are indicative of chronic sarcoidosis and may mark the spread of the disease to other locations throughout the body. Nasal and oral mucosa, the larynx, pharynx, and pulmonary parenchyma are all possible sites of dissemination.5 Additionally, lupus pernio can result in further disfigurement of the face with invasive destruction of underlying cartilage and bone.6
Treatment of patients with lupus pernio includes topical and intralesional injection of corticosteroids, which can effectively treat inflammation and granuloma formation.7 A trial of oral minocycline or doxycycline may be considered in patients with skin involvement only, without systemic manifestations.8 Immune-modifying agents such as methotrexate, azathioprine, tetracyclines, hydroxychloroquine, and infliximab can be recommended when chronic or severe disease warrants their use.9 Nasal skin excision followed by flap reconstruction can lead to dramatic improvement in severe cases of lupus pernio.10 In some cases, laser therapy may be beneficial.11
While cutaneous sarcoidosis may remit spontaneously without treatment, spontaneous remission of lesions that have been present for more than 2 years is rare. Achieving effective therapy may require prolonged treatment.
Outcome of the case
Previous treatment with a topical corticosteroid proved beneficial for her cutaneous sarcoidosis lesions. Prior to subsequent treatment, a thorough workup was completed, including a comprehensive metabolic panel, a complete blood count, plasma angiotensin-converting enzyme levels, and chest radiography to screen for systemic involvement and the potential need for treatment with a systemic agent.
William Scharpf, BS, is a student at the Campbell University Jerry M. Wallace School of Osteopathic Medicine in Lillington, North Carolina.
Laura F. Sandoval, DO, is a dermatology resident at Sampson Regional Medical Center in Clinton, North Carolina.
Jonathan S. Crane, DO, is director of the dermatology residency program at Sampson Regional Medical Center in Clinton, North Carolina.
1. Wilson NJE, King CM. Cutaneous sarcoidosis. Postgrad Med J. 1998;74(877):649-652.
2. Durić B, Secen N. Sarcoidosis activity markers [in Croatian]. Med Pregl. 1991;44(5-6):259-262.
3. Cox CE, Davis-Allen A, Judson MA. Sarcoidosis. Med Clin North Am. 2005;89(4):817-828.
4. Cozier YC, Berman JS, Palmer JR, Boggs DA, Serlin DM, Rosenberg L. Sarcoidosis in black women in the United States: data from the Black Women’s Health Study. Chest. 2011;139(1):144-150.
5. James DG. Lupus pernio. Lupus. 1992;1(3):129-131.
6. Neville E, Carstairs LS, James DG. Sarcoidosis of bone. Q J Med. 1977;46(182):215-227.
7. Johns CJ, Michele TM. The clinical management of sarcoidosis: a 50-year experience at the Johns Hopkins Hospital. Medicine (Baltimore). 1999;78(2):65-111.
8. Bachelez H, Senet P, Cadranel J, Kaoukhov A, Dubertret L. The use of tetracyclines for the treatment of sarcoidosis. Arch Dermatol. 2001:137(1):69-73.
9. Stagaki E, Mountford WK, Lackland DT, Judson MA. The treatment of lupus pernio: results of 116 treatment courses in 54 patients. Chest. 2009;135(2):468-476.
10. Smith R1, Haeney J, Gulraiz Rauf Kh. Improving cosmesis of lupus pernio by excision and forehead flap reconstruction. Clin Exp Dermatol. 2009:34(5):e25-e27.
11. Brauer JA, Gordon Spratt EA, Geronemus RG. Laser therapy in the treatment of connective tissue diseases: a review. Dermatol Surg. 2014;40(1):1-13.