Sarcoidosis is a multisystemic disease of unknown etiology characterized by the presence of epithelioid granulomas without caseous necrosis in the organs involved. Sarcoidosis cutaneous lesions can be severe. There is no recommendation for the treatment of cutaneous sarcoidosis. A recent study highlights the potential efficacy of mTOR inhibitors in the treatment of sarcoidosis granulomas. The hypothesis is that sirolimus could be effective for sarcoidosis treatment, especially for cutaneous lesions.

The main objective of this study is to evaluate sirolimus efficacy on cutaneous sarcoidosis of the face.

The main evaluation criteria is the percentage of patients with a significant clinical response (relative decrease in "facial SASI" ≥ 25%) at week 16 of treatment.

Inclusion Criteria:

• Age ≥ 18 years old <75 years old (men and women)
• Cutaneous sarcoidosis of the face (diagnosed according to the following criteria : compatible clinical appearance showing erythemato-purple, brownish or yellowish macules or papules or nodules and compatible histological appearance with a facial or extra facial skin biopsy confirming the diagnosis of sarcoidosis showing epithelioid and giganto-cellular granuloma without caseous necrosis) moderate to severe defined by: "Facial SASI" Score (Sarcoidosis Area and Severity Index) ≥ 2 and PGA (Physician's Global Assessment,0 to 10 scale) ≥ 5
• Health insurance plan coverage
• Patients who never had a systemic treatment or who had at least one classical systemic treatment failure for sarcoidosis treatment
• For women of childbearing age (unless post-menopausal or sterile), pregnancy test with βHCG negative. Effective contraception should be used during sirolimus treatment and for 12 weeks after stopping sirolimus
• Patients who have signed a written consent

Exclusion Criteria:

• Severe hepatic failure (Cytolysis (ALAT)> 3N and / or Cholestase (PAL)> 3N)
• Allergy or intolerance to sirolimus or at one of its excipients
• Allergy to peanut or soybeans
• Patient with a pulmonary or hepatic graft
• General corticotherapy or immunosuppressive treatment (methotrexate, azathioprine, mycophenolate mofetil, cyclophosphamide, ciclosporin) in the month before the inclusion
• Intra-lesional corticotherapy for less than 3 months
• Biotherapy (anti-TNFa, anti-IL12/23, anti-IL17A) within 3 months preceding the inclusion
• Thalidomide or other -imide treatment for less than 3 months
• Cyclins treatment for less than 1 month
• Topical corticosteroids or topical tacrolimus for less than 1 week
• Sarcoidosis involvement of at least one organ requiring systemic treatment other than sirolimus (oral corticosteroid or systemic immunosuppressive treatment)
• Cholesterolemia> 300 mg/ dl or triglyceridemia> 400 mg/dl
• Administration of strong CYP3A4 inhibitors or inducers such as rifampicin, ketoconazole, voriconazole, telithromycin , diltiazem, verapamil, erythromycin, clarythromycin, ciclosporin
• Pregnancy or breastfeeding
• Active infection including tuberculosis disease
• Non-controlled arterial hypertension (TAS> 150 mmHg and / or TAD> 100 mmHg)
• Patient under guardianship or curatorship, patients deprived of freedom, under safeguarding of justice, receiving psychiatric care, under the constraint, admitted in a health or social institution for purposes other than those of research
• Patient with cancer (except cutaneous basal cell carcinoma or in situ cervical cancer)
• Risk of patient bad compliance
• Grapefruit or grapefruit juice consumption during the treatment duration
• Patients with fructose intolerance, galactose intolerance, glucose-galactose malabsorption, insufficiency in sucrase-isomaltase or Lapp lactase