A Case of Alopecia Totalis with Lichen Planus Hypertrophicus Responded Excellently to Oral Tofacitinib-An Observation

Dear Editor,

Alopecia areata is a chronic, relapsing, immune-mediated inflammatory disorder characterized by patchy or diffuse, non-cicatricial alopecia which in severe cases may lead to total loss of scalp hair (alopecia totalis) or both scalp and body hair (alopecia universalis). Alopecia areata has been frequently seen to be associated with a number of immunologically mediated cutaneous disorders, lichen planus being one of them.[1] The scope of use of Janus kinase (JAK) inhibitors in dermatologic conditions continues to broaden as multiple conditions seem to respond to treatment with these agents. While the use of a JAK 1/3 inhibitor, tofacitinib in alopecia areata is supported by multiple case reports and case series, it is increasingly being used in lichen planus too with favourable response.[2,3]

A 23-year-old female presented to our clinic with a history of multiple, severely itchy, warty lesions over both legs for the last 8 years. The patient also reported asymptomatic loss of scalp hair of 6 months duration. There was no history of trauma or drug intake relevant to her condition. The family history of the patient was non-contributory. The patient reported following multiple treatment regimens previously with no satisfactory results. Cutaneous examination of the patient revealed multiple, hyperkeratotic, hyperpigmented papules and plaques with central hypopigmentation involving both the lower legs and ankles in a linear fashion. Examination of the scalp and face revealed diffuse, non-cicatricial alopecia. The Severity of Alopecia Tool (SALT) score was used for the assessment of hair loss. Histopathological examination of a lesion on leg revealed epidermis with marked hyperkeratosis and irregular hyperplasia, dense lymphocytic infiltrate at the dermo-epidermal junction admixed with melanophages and vascular prominence [Figures 1 and 2]. A skin biopsy from the scalp revealed features suggestive of alopecia areata. Based on clinical examination and histopathology of scalp and lower limb lesions respectively, the diagnosis of alopecia totalis and lichen planus hypertrophicus (LPH) was made. The patient was put on oral cyclosporine at a dosage of 5 mg/kg/day. However, subsequent to insufficient therapy response at 6 months, cyclosporine was stopped, and tofacitinib was initiated at the dosage of 5 mg twice daily. Before commencement of tofacitinib therapy, baseline laboratory evaluation including complete blood cell count, liver function tests, lipid profile, screening for latent tubercular infection, HIV, hepatitis B, and C viruses were carried out. All other possible autoimmune associations were ruled out with appropriate investigations. The patient was followed up and assessed monthly. Adverse effects were recorded at each visit.

Figure 1.

Epidermis shows marked hyperkeratosis and irregular hyperplasia (H&E, 40x)

Figure 2.

Dense lymphocytic infiltrate at the dermo-epidermal junction admixed with melanophages. Vascular prominence is also seen here (H&E, 100x)

Hair regrowth over the scalp and face started from the second month of treatment with tofacitinib whereas lesions on the legs started improving clinically after two and half months of therapy. A significant improvement in both the conditions was noted at 9 months of treatment [Figures 3 and 4]. Tofacitinib was well tolerated by the patient and no serious adverse effects were noted. The patient was further put on methotrexate therapy for maintenance after the stoppage of tofacitinib. We used only topical moisturizer for LPH lesions.

Figure 3.

Alopecia totalis, before and after tofacitinib therapy

Figure 4.

Lichen planus hypertrophicus, before and after tofacitinib therapy

Co-occurrence of alopecia areata and lichen planus has commonly been reported and may represent a common pathogenic mechanism in these CD8+T cell-mediated cutaneous disorders. However, the concurrence of alopecia totalis with lichen planus hypertrophicus, to the best of our knowledge, has rarely been reported. Tofacitinib is a JAK 1/3 inhibitor and is emerging as a promising therapy for severe cases of alopecia areata (alopecia totalis and alopecia universalis).[4] On the other hand, the efficacy of tofacitinib in lichen planus especially lichen planus hypertrophicus has been mentioned in very few reports.[5] Tofacitinib acts by inhibiting the JAK-STAT pathway thereby attenuating the inflammatory cascade in immune mediated cutaneous disorders like alopecia areata and lichen planus [Figure 5]. Adverse effects of tofacitinib in the form of heart-related adverse events, increased risk of blood clots, cancer and death appear to be more in patients taking higher doses (10 mg twice daily).[6] As observed from Figure 5, IFNγ/CXCL 10 axis via the JAK-STAT pathway, plays a key role in the inflammatory cascade involved in the pathogenesis of lichen planus and alopecia areata hence proving the role of JAK inhibitors in the management of both conditions. Our case highlights the efficacy of tofacitinib in severe alopecia areata and lichen planus hypertrophicus, both being resistant and difficult-to-treat conditions. As the outcome in our case was quite encouraging, tofacitinib could be considered an effective treatment option in patients with severe disease or those not responding to the conventional therapy.

Figure 5.

Common signalling pathway involved in pathogenesis of alopecia areata and lichen planus and role of JAK-STAT pathway

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