Alopecia as a rare but distinct manifestation of pemphigus vulgaris

Veraitch, Ophelia; Ohyama, Manabu; Yamagami, Jun; Amagai, Masayuki

doi:10.1111/j.1468-3083.2011.04363.x

Cited by 24 publications

(31 citation statements)

References 20 publications

(29 reference statements)

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“…[4] Indeed, also in our patient, a skin swab cultured S. aureus , which was also cultured in one previous pemphigus vulgaris and alopecia case. [4]…”

Section: Discussionmentioning

confidence: 56%

“…It has been suggested that antibody-mediated acantholysis between outer root sheath keratinocytes is responsible for the hair loss, but the fact that alopecia is not seen in most pemphigus patients implies that an additional secondary factor is needed for hair-loss induction. [4] One such proposed factor is secondary infection, which was evident in all cases of a recently published case series where skin swabs were taken. [4] Indeed, also in our patient, a skin swab cultured S. aureus , which was also cultured in one previous pemphigus vulgaris and alopecia case.…”

Section: Discussionmentioning

confidence: 99%

“…[4] One such proposed factor is secondary infection, which was evident in all cases of a recently published case series where skin swabs were taken. [4] Indeed, also in our patient, a skin swab cultured S. aureus , which was also cultured in one previous pemphigus vulgaris and alopecia case. [4]…”

Section: Discussionmentioning

confidence: 99%

“…[1] Although the scalp is frequently involved in pemphigus vulgaris,[2] associated hair loss has only rarely been described. [3456] We report a rare case of pemphigus vulgaris involving the scalp that leads to hair loss.…”

Section: Introductionmentioning

confidence: 99%

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Pemphigus vulgaris with loss of hair on the scalp

et al. 2013

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Pemphigus vulgaris is an autoimmune disease, which leads to flaccid bullae and erosions. Although the scalp is commonly involved, hair loss is rarely seen. We herein report a case of 32-year-old female patient with a 2-year history of pemphigus vulgaris, involving the skin and mucosa. Although clinical improvement was achieved with oral corticosteroids and mycophenolate mofetil, the patient developed tender plaques on her scalp, accompanied by hair loss. Histopathology and direct immunofluorescence were consistent with pemphigus vulgaris. Skin swab cultured Staphylococcus aureus. Treatment with oral and topical corticosteroids combined with mycophenolate mofetil resulted in clinical remission, with regrowth of scalp hair. Alopecia is an unusual clinical manifestation of pemphigus vulgaris. Our case supports the hypothesis that hair loss is induced by antibody-mediated outer root sheath keratinocyte acantholysis, in combination with skin infection, that together lead to the observed hair loss.

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“…[4] Indeed, also in our patient, a skin swab cultured S. aureus , which was also cultured in one previous pemphigus vulgaris and alopecia case. [4]…”

Section: Discussionmentioning

confidence: 56%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Pemphigus vulgaris with loss of hair on the scalp

et al. 2013

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“…2 We acknowledge that we incorrectly stated that alopecia is frequently observed in pemphigus patients in our manuscript, which should have read infrequently, as indicated in the cited reference. 4 In their letter, Drs. Grando and Pittelkow claim that our results, in conjunction with previous animal studies, failed to substantiate that Dsg3 is the principal desmosomal cadherin that holds keratinocytes together.…”

Section: From the Department Of Immunology Duke University Medical Cmentioning

confidence: 96%

Authors' Reply

Kountikov

Fujisawa

Tedder

2015

The American Journal of Pathology

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We are grateful that Drs. Grando and Pittelkow 1 have chosen to highlight our recent paper in their Letter to the Editor, where they argue that mouse models of desmoglein 3 (Dsg3) deficiency lack the phenotype of human pemphigus, thereby providing support for a multigenic theory of pemphigus. Our studies focused exclusively on the genetic mapping and phenotype of a spontaneous mouse mutation within the Dsg3 gene that resulted in a unique squeaky phenotype characterized by immunodeficiency and a wasting disease. 2 Squeaky mice share phenotypic similarities with Dsg3-deficient mice, but they also exhibit unique characteristics due to their hypomorphic expression of a truncated Dsg3 protein. Nonetheless, pemphigus results from autoantibodies, not congenital genetic alterations in their target molecules.We appreciate this opportunity to reiterate several key points of our paper to diminish any possible over-or misinterpretation of the data presented. Squeaky mice have a dramatic phenotype that includes severe immunodeficiency and inspiratory stridor (squeaking) due to primary pathological changes within the larynx and airway obstruction. Hallmarks of pathology include significant hyperplasia of the epiglottis that results in its thickening and deformation. This pathology extends to lesions on the back of the tongue as described for Dsg3-deficient mice. 3 There is no evidence of acantholysis in the tongue or other sites. Thereby, hypomorphic Dsg3 protein expression may support intercellular adhesion and prevent spontaneous acantholysis in Dsg3 sqk/sqk mice, but it appears insufficient to prevent epithelial sloughing and lesions resulting from mechanical damage or stresses, such as chewing on solid food or snout abrasions. The histology of these oral lesions and snout erosions was substantiated in Figure 6. 2 We acknowledge that we incorrectly stated that alopecia is frequently observed in pemphigus patients in our manuscript, which should have read infrequently, as indicated in the cited reference. 4 These mice have been made available to the research community through the Mutant Mouse

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