Alopecia totalis

Alopecia areata (AA) is an autoimmune disease characterized by T cell-mediated attack on the hair follicle. Although there are a wide range of therapies, the majority of them are not satisfactory due to side effects, pain due to intralesional injections or limited efficacy. In this study, we sought to evaluate the efficacy, influence factors, and safety of 308 nm excimer lamp used in a monthly basis in a double-stacked pulse manner for the treatment of AA.

This was a prospective study, using 308 nm excimer lamp in a double-stacked pulse therapy for AA. The primary endpoint was the improvement in SALT score.

A total of 40 patients with AA were enrolled in this study. Forty (100%) patients achieved clinical response. Hyperpigmentation and erythema occurred on the treated alopecic areas of all patients but they were considered tolerable. Patients of younger age or with a smaller area of affection had a better overall treatment response.

308 nm excimer lamp therapy is an excellent option for single or multiple AA because it achieves a good clinical response with less adverse effects than other therapies. This therapy may be useful for low-income countries where new JAK inhibitors are not available, however, for patients with extensive hair loss, it is not as effective and thus, it may be unfit for patients with alopecia totalis and alopecia universals.

STING-associated vasculopathy with onset in infancy (SAVI) is a rare type I interferonopathy caused by heterozygous variants in the STING gene. In SAVI, STING variants confer a gain-of-function which causes overactivation of type I interferon (IFN) signaling leading to autoinflammation and various degrees of immunodeficiency and autoimmunity.

We report the case of a 5 year old child and his mother, both of whom presented with systemic inflammatory symptoms yet widely varying organ involvement, disease course and therapeutic response. Genetic testing revealed a heterozygous STING variant, R281Q, in the child and his mother that had previously been associated with SAVI. However, in contrast to previously reported SAVI cases due to the R281Q variant, our patients showed an atypical course of disease with alopecia totalis in the child and a complete lack of lung involvement in the mother.

Our findings demonstrate the phenotypic breadth of clinical SAVI manifestations. Given the therapeutic benefit of treatment with JAK inhibitors, early genetic testing for SAVI should be considered in patients with unclear systemic inflammation involving cutaneous, pulmonary, or musculoskeletal symptoms, and signs of immunodeficiency and autoimmunity.

Alopecia areata is an autoimmune form of non-scarring hair loss. It is usually characterized by limited areas of hair loss. However, the disease may progress to complete scalp and body hair loss (alopecia totalis, alopecia universalis). In patients with alopecia areata hair loss significantly impacts the quality of life. Children and adolescents with alopecia areata often experience bullying, including physical aggression. The disease severity evaluation tools used in clinical practice are: the Severity of Alopecia Tool (SALT) score and the Alopecia Areata Scale (AAS). A SALT score equal to or greater than 20 constitutes a commonly accepted indication for systemic therapy in alopecia areata. When using the AAS, moderate to severe alopecia areata should be considered a medical indication for systemic treatment. Currently, the only two EMA-approved medications for alopecia areata are baricitinib (JAK 1/2 inhibitor) for adults and ritlecitinib (JAK 3/TEC inhibitor) for individuals aged 12 and older. Both are EMA-approved for patients with severe alopecia areata. Other systemic medications used off-label in alopecia areata include glucocorticosteroids, cyclosporine, methotrexate and azathioprine. Oral minoxidil is considered an adjuvant therapy with limited data confirming its possible efficacy. This consensus statement is to outline a systemic treatment algorithm for alopecia areata, indications for systemic treatment, available therapeutic options, their efficacy and safety, as well as the duration of the therapy.