Skin involvement in amyotrophic lateral sclerosis

Kolde, G.; Bachus, R.; Ludolph, Albert C.

doi:10.1016/s0140-6736(96)90737-0

Cited by 58 publications

(38 citation statements)

References 8 publications

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“…14 Cutaneously, light and electron microscopic alterations of skin connective tissue have recently been described in 7 patients with ALS, 22 with a cutaneous deposition of ␤-amyloid protein. Moreover, 1 study showed a decreased type IV collagen immunoreactivity of the basement membrane of the skin, which was even more substantial in patients with disease of longer duration.…”

Section: Casementioning

confidence: 99%

Bullous Pemphigoid and Amyotrophic Lateral Sclerosis

Chosidow¹,

Doppler²,

Bensimon³

et al. 2000

Arch Dermatol

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Section: Casementioning

confidence: 99%

Bullous Pemphigoid and Amyotrophic Lateral Sclerosis

Chosidow¹,

Doppler²,

Bensimon³

et al. 2000

Arch Dermatol

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“…Light and electron microscope alterations in skin connective tissue were previously described in patients with ALS (15,16). BP in ALS may be under-recognized; however, more importantly, it is treatable with steroid therapy.…”

Section: Discussionmentioning

confidence: 96%

A Potential Link between Amyotrophic Lateral Sclerosis and Bullous Pemphigoid: Six New Cases and a Systematic Review of the Literature

et al. 2016

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Objective Bullous pemphigoid in amyotrophic lateral sclerosis (BP-ALS) is rare and poorly understood. We herein assessed the association between ALS and BP using clinical and biological findings. Methods The clinical features of six new BP-ALS cases were described and collated with cases from a systematic literature review. Results Our six cases were combined with three other published cases. The mean disease duration (from ALS onset to the occurrence of BP) was 5.6±3.1 years. All patients had limb-onset ALS. Four of the 9 patients received riluzole, with the use of riluzole ranging from a few days to 3 years. When BP occurred, the status of the ALS patients was paretic and/or bedridden in all cases. BP occurred throughout the body, and we confirmed that the bullous lesions were located not only at the compression site, but also at the anterior part of the chest, abdomen, and limbs. Treatment for BP was successful, as oral prednisone and/or local corticosteroids were effective in 8 cases. Conclusion These six new cases, in combination with previous cases, expand our knowledge of the relationship between dermatological lesions and ALS. The pathogenesis of BP-ALS is poorly understood, however, some immunological aberrance is likely.

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“…[1][2][3]7,[10][11][12][13] Under light microscopy, collagen bundles in ALS dermis were reduced in number and size. 10 Electron microscopy showed collagen fibrils of small diameter in the dermis, and there was a negative correlation between the duration of illness and diameter of the collagen fibrils in patients with ALS.…”

Section: Discussionmentioning

confidence: 99%

“…7 We have analyzed change in the serum concentrations of type IV collagen, a major component of the basement membrane, 6 during the various stages of sporadic ALS to clarify whether the metabolism of the basement membrane is affected in the disease process of ALS.…”

mentioning

confidence: 99%