The nose as a predilection site of pemphigus
The nose as a predilection site of pemphigus
复制标题
鼻子是天疱疮的好发部位
DOI：
10.1111/ced.13275
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发表时间：
2018
影响因子：
4.1
通讯作者：
Nakagawa H.
中科院分区：
文献类型：
--
作者：
Aizawa N.;Asahina A.;Ishii N.;Hashimoto T.;Nakagawa H.
The patient was therefore diagnosed with LABD. Complete blood count showed neutrophilia (neutrophils 94%; normal range 41.8–70.8%). Other laboratory studies, including liver and renal function, antinuclear antibodies and IgG/IgA/IgM levels, were within normal limits. The patient was started on prednisone 20 mg daily. Complete clearing of the cutaneous lesions was achieved within 1 month and the dosage of prednisolone was tapered gradually to 5 mg daily with no clinical recurrence of LABD during a follow-up of more than 2 years.The aetiology of ALS is currently not fully understood. Immunological factors and increased susceptibility to the toxic effects of glutamate in the nervous system may be involved in the pathogenesis of ALS. 2 Bullous pemphigoid (BP), a vesicobullous disease similar to LABD, has been reported in association with ALS. 2, 3 The massive accumulation of intermediate filaments in motor neurons in patients with ALS could induce a crossreactive immune response, which attacks not only the neuronal antigens but also the BP antigens in the skin, leading to the development of BP. 3 Unlike the well-established association between neurological diseases and BP, the association with such diseases and LABD is unclear. We hypothesize that in our patient, the mechanism of LABD is similar to BP in association with ALS. The predominant antigenic targets in patients with LABD partially overlap with the BP antigens, such as bullous pemphigoid (BP) 180 and BP230. 1 This supports our hypothesis, and may explain the BP-like cutaneous manifestations in our patient. Another possible explanation for LABD coexisting with ALS is laminin-c1, whose overexpression has been found in both the neurological system and the skin of patients with ALS. 4, 5 In addition, some studies have demonstrated IgA reactivity to various antigens, including laminin-c1, in patients with LABD. 6 These studies might indicate shared autoimmunity between LABD and ALS. The possibility of druginduced LABD resulting from riluzole is unlikely because the patient had used the drug for 4 years without any skin eruption, and it was continued during the development and resolution of LABD. To our knowledge, this is the first reported case of LABD in association with ALS. Further studies are required to clarify whether LABD may be associated with other neurological disorders, as had been demonstrated in BP.
