Treatment of angiolupoid sarcoidosis done with tofacitinib 2
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Sarcoidosis is an inflammatory disease that is characterized by the formation of granulomas in affected tissues. Sarcoidosis can occur in nearly any organ; approximately one-third of patients have skin involvement, which is occasionally the only site of disease. Angiolupoid sarcoidosis is a clinical variant that favors the central face, especially the nasal bridge and is characterized by prominent telangiectasia formation overlying inflammatory lesion.

A 50-year-old female with a history of sarcoidosis for five years presented for evaluation and discussion of treatment options. She had a history of a T1a melanoma five years prior, treated with surgery alone. Her sarcoidosis was incidentally discovered during her melanoma workup.

An abnormal chest X-ray led to a chest CT which showed hilar and mediastinal lymphadenopathy with peribronchovascular and subpleural opacities. The findings were deemed consistent with sarcoidosis and have been followed and remained stable. Two years later she developed plaques on her face and arms. A biopsy from the upper arm showed sarcoidal granulomas.

For her cutaneous disease, the patient had previously been treated with triamcinolone 0.1% cream for up to two weeks at a time and was ineffective. Topical tacrolimus 0.1% ointment was also ineffective. A trial of doxycycline 50 mg twice daily and later methotrexate 15 mg weekly were both ineffective after 3 month trials of each. She was intolerant of hydroxychloroquine(dizziness). Given her history of melanoma and personal preference, TNF-alpha inhibition was not pursued.

Physical examination (while taking methotrexate) showed indurated, slightly infiltrated pink-red plaques on the nasal dorsum, tip, alae, and glabella. There were prominent telangiectasias overlying the lesions on the nose. For her facial involvement, the Cutaneous Sarcoidosis Activity and Morphology Instrument (CSAMI) (9) activity score was 15. Given the emerging experience with oral JAK inhibition in sarcoidosis doctors elected to proceed with compounded tofacitinib 2% ointment twice daily.

After 10 weeks of topical tofacitinib, improvement in erythema and induration was noted; however, the telangiectasia did not improve. At this time pulsed dye laser (PDL) was initiated, for three treatments over five months with improvement in the telangiectasia. Tofacitinib was continued through the PDL and has been used continuously for a total of 18 months. The patient has experienced ongoing improvement and have not developed new telangiectasias. At her most recent follow-up her CSAMI activity score was 8; the lesions on the face were judged to be minimally active and their distribution had not changed. There was no adverse effects from therapy.