PMC10277483_01
(A): Several follicular ostia, mild sebaceous hyperplasia, superficial dermal telangiectatic vessels, as well as perivascular and perifollicular inflammation. Perivascular granulomas were also detected. (Hematoxylin-eosin stain; x10). (B): Pathological examination shows an unusual proliferation of abundant elastic fibers and perivascular infiltration by inflammatory cells. (Hematoxylin-eosin stain; x10). (A, B) The excisional biopsy of the patient 1 support a diagnosis of Morbihan disease. (C, D): case 1 before Tofacitinib treatment: diffuse erythema and redness on his cheeks, and his left eyelid displayed evident erythema, swelling, and ptosis. (E, F): case 1 after 6 months' Tofacitinib treatment: erythema and redness on his cheeks has subsided, there was a noticeable improvement in the swelling of the left eyelid compared to before.
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Patient 1 ( Figure 1 ) was a 43-year-old male who presented to the clinic with a 12-month history of progressive pain and swelling in his left face and left upper eyelid. Furthermore, perivascular granulomas were detected in the biopsy ( Figure 1A, B ), leading to the diagnosis of Morbihan disease. We conducted a follow-up on patient 1 and after receiving a six-month treatment of Tofacitinib, the swelling and redness on the left cheek of the patient has disappeared ( Figure 1E, F ), and there has been a significant improvement in the swelling of the left eyelid compared to before.
The timeline of laboratory investigations and ongoing treatments. (case1).
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Subsequently, we halved the dosage of Tofacitinib (5mg, po once daily) ( Figure 2 ).
PMC10277483_02
(A): Patient 2 excision biopsy [left face]: lymphocyte infiltration in the superficial vessels of dermis and some accessories. (HEx10). (B): case 2 before Tofacitinib treatment: Solid persistent eyelid and facial edema could be found on the left side face. (C): case 2 after Tofacitinib treatment:The swelling of the left eyelid and redness of the left cheek have subsided.
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Patient 2 ( Figure 3B ) was a 29-year-old Chinese female patient who came to the clinic with a 2-year history of progressive left-sided facial edema. The patient was recently admitted to the dermatological department, and an excision biopsy revealed lymphocyte infiltration in the superficial vessels of the dermis ( Figure 3A ). After one-week treatment, facial swelling was assessed as moderate by her doctor ( Figure 3C ).
The timeline of laboratory investigations and ongoing treatments. (case2).
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Subsequently, the patient continued to use tofacitinib for one month, and during the subsequent six-month follow-up, there was no recurrence of cutaneous lesions ( Figure 4 ).