We present a case of myasthenia gravis that occurred after alopecia areata and evaluate the literature on the comorbidity of alopecia and myasthenia gravis. A 41-year-old male noticed hair loss on his scalp 4 months ago, and his hair loss progressed with drooping of both upper eyelids and diplopia. Physical and laboratory examination identified the comorbidity of myasthenia gravis and alopecia areata. Prednisolone 10 mg/day and tacrolimus 3 mg/day were administered for 7 days following hospitalization, which served to improve diplopia and ptosis. Following methylprednisolone therapy, hair loss in alopecia areata improved without the enlargement of bald areas. Based on the literature review, a total of 29 cases with alopecia and myasthenia gravis including our case have been reported. Among them, seven cases of myasthenia gravis developed after alopecia. The average time for the onset of myasthenia gravis after alopecia was 16.6 months. Four cases showed other autoimmune disease comorbidity, such as vitiligo, lichen planus, cutaneous lupus erythematosus, and pemphigus foliaceus, suggesting the involvement of Th1-significant immunological states in these patients.
We demonstrated that there were abundant neutrophil extracellular traps (NETs) in the skin biopsies from various types of pyoderma gangrenosum (PG), based on the observation of extended and compact areas of immunolabeling of MPO and Cit H3 proteins. We suggest that neutrophils could undergo an aberrant NET formation in the lesions of PG patients, in the vast majority of idiopathic PG. We did not detect NETs in the skin ulcers of an antiphospholipid syndrome patient with a similar appearance to classical ulcerative PG, while rich NETs were found in the various types of PG. These findings suggest that the presence of NETs in skin tissues could serve as a marker for making differential diagnoses of various types of PG from other similar conditions.
This study aimed to evaluate long-term changes in nailfold videocapillaroscopy (NVC) findings in dermatomyositis patients with antinuclear matrix protein 2 (NXP-2) antibody (Ab). All four patients with anti-NXP-2 Ab presented irregularly enlarged and reduced capillaries and hemorrhages at the initial assessment. After disease stabilization, irregularly enlarged capillaries and hemorrhages disappeared within the mean observation period of 6 months. These early improvements were not observed in patients with anti-TIF1 Ab. The results of this study show that long-term changes in NVC findings should be assessed using myositis-specific Ab information.
Prurigo-type atopic dermatitis (AD) is an AD variant characterized by excoriated papules, indurated nodules, and intense itching associated with type 2 cytokine responses.1 Recently, upadacitinib, an oral selective Janus kinase (JAK) 1 inhibitor, was found to be efficacious and safe in treating moderate-to-severe AD in patients aged >12 years in a clinical trial.2 However, few reports have demonstrated evidence of upadacitinib in prurigo-type AD. Here, we present four Japanese patients with prurigo-type AD who received upadacitinib.
Our cases included two male and two female patients with moderate-to-severe AD (Table S1). The patients fulfilled the AD criteria.3 Cases 3 and 4 were also confirmed AD pathologically. They were treated with 15 or 30 mg upadacitinib, topical corticosteroid, and moisturizers once a day. Case 1: a 49-year-old female with widespread areas of erythema, nodules, and itching on her legs (Figure 1A). We initiated 30 mg of upadacitinib. After 8 weeks, she achieved Eczema Area and Severity Index (EASI)-90 and was itch-free (Figure 1B). Case 2: a 50-year-old female. She applied corticosteroid ointments on her refractory nodules for decades (Figure 1C). She had difficulty throughout her life due to insomnia caused by itching. We initiated 30 mg of upadacitinib; she was pruritus-free after 2 weeks, achieving EASI-90 after 4 weeks (Figure 1D). Case 3: a 64-year-old male with multiple pruritic nodules on his trunk and extremities (Figure 1E). We first initiated 30 mg of upadacitinib for his severe itchiness. After 4 weeks, his skin lesions and itching improved noticeably. Thus, we decreased the upadacitinib dose to 15 mg. After 12 weeks, his skin lesions almost disappeared (Figure 1F). Case 4: a 66-year-old male. He had multiple nodules on his neck and arms, forming plaque (Figure 1G). Although we diagnosed him with severe AD, we prescribed 15 mg of upadacitinib because of his age. After 12 weeks, he achieved EASI-90, and his skin lesions almost disappeared (Figure 1H). No adverse events were observed in the patients.
Figure 1I–K shows the transition of EASI, patient-oriented eczema measure (POEM), and pruritus numerical rating scale (NRS). A rapid decrease was seen in all categories from baseline to Week 4, and this effectiveness lasted for 12 weeks. Thymus and activation-regulated chemokine and immunoglobulin E in the serum were decreased from baseline to Week 4; however, some cases increased at Week 12 (Figure 1L,M).
The effectiveness of dupilumab and baricitinib against prurigo-type AD was recently reported.4, 5 Dupilumab showed a significantly lower achievement rate of EASI-50 at 2 months in the prurigo compared with the non-prurigo group.4 These results indicate the importance of suppressing not only IL-4 and IL-13 but also other cytokines, including IL-31 and thymic stromal lymphopoi
Bullous pemphigoid (BP) is a representative autoimmune subepidermal blister disease, and approximately 20% of cases recognized the absence of a typical blister. Those cases result in delayed diagnosis and appropriate therapeutic selection results, leading to an increased mortality rate. Herein, we present a case of initial misdiagnosed as persistent eczematous eruption, which was finally confirmed as nonbullous pemphigoid by repeated histological examinations