Limits...
Dyshidrosiform pemphigoid with Parkinsonism in a nonagenarian Maharashtrian female.

Behlim T, Sharma YK, Chaudhari ND, Dash K - Indian Dermatol Online J (2014)

Bottom Line: Dyshidrosiform pemphigoid is a rare variant of bullous pemphigoid localized to the hands and feet whose characteristic subepidermal blisters develop as a result of binding of the IgG autoantibodies to intracellular plaque and extracellular face of the hemidesmosome recognizing a 230-kDa plakin molecule (BP230, BPAg1or BPAg1e) and a 180-kDa transmembrane protein.Neurodegenerative processes (viz., stroke, dementia, Parkinsonism, epilepsy, etc) uncover BPAg1-n, an alternatively spliced form of BPAg1-e that stabilizes the cytoskeleton of sensory neurons, generating autoantibodies that may subsequently lead to BP by cross-reacting with BPAg1-e.We present a patient with Parkinsonism who later developed blisters, erosions and crusts localized to the palms and soles, confirmed histopathologically as bullous pemphigoid.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Dr. D. Y. Patil Medical College and Hospital, Pimpri, Pune, Maharashtra, India.

ABSTRACT
Dyshidrosiform pemphigoid is a rare variant of bullous pemphigoid localized to the hands and feet whose characteristic subepidermal blisters develop as a result of binding of the IgG autoantibodies to intracellular plaque and extracellular face of the hemidesmosome recognizing a 230-kDa plakin molecule (BP230, BPAg1or BPAg1e) and a 180-kDa transmembrane protein. Neurodegenerative processes (viz., stroke, dementia, Parkinsonism, epilepsy, etc) uncover BPAg1-n, an alternatively spliced form of BPAg1-e that stabilizes the cytoskeleton of sensory neurons, generating autoantibodies that may subsequently lead to BP by cross-reacting with BPAg1-e. We present a patient with Parkinsonism who later developed blisters, erosions and crusts localized to the palms and soles, confirmed histopathologically as bullous pemphigoid. To the best of our knowledge, ours is the first case report from India wherein Parkinsonism-generated autoantibodies led to the development of dyshidrosiform pemphigoid due to their cross-reactivity with BPAg1-e.

No MeSH data available.


Related in: MedlinePlus

Healed lesions with hyperpigmentation in between fingers of right hand and over right palm
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC4228647&req=5

Figure 1: Healed lesions with hyperpigmentation in between fingers of right hand and over right palm

Mentions: A debilitated, non-ambulatory female aged 91 years, known case of Parkinsonism since 2 years, presented to us with the history of appearance of multiple blisters over palms and soles since one month. Local itching and burning preceded their onset. There was no significant antecedent drug intake. There was no icterus, pallor, lymphadenopathy, clubbing or organomegaly. Neurological examination revealed mask like facies, glabellar tap, pill rolling tremors of the right hand, cogwheel rigidity and hypertonia of limbs. Lateral and palmoplantar aspects of hands [Figure 1] and feet [Figure 2] showed dark brown encrusted erosions and a few tense clear bullae with negative Nikolsky- and Asboe-Hansen signs. Histopathological examination findings of subepidermal bulla containing eosinophilic infiltrate [Figure 3] in the lesional skin and IgG at dermoepidermal junction (DEJ) on direct immunofluorescense [Figure 4] confirmed our clinical suspicion of BP. Peripheral blood smear and ultrasonography abdomen were normal. Advanced imaging studies could not be carried out due to kyphoscoliosis, chest X-ray evidence of scoliosis to the right and her confinement to the wheelchair. Advanced confirmational studies viz., immunoblot/immunoprecipitation could not be afforded by the patient. Treatment with tetracycline, nicotinamide and dapsone showed appreciable improvement after a fortnight. She insisted to go home on discharge and passed away a month later due to gastrointestinal bleeding.


Dyshidrosiform pemphigoid with Parkinsonism in a nonagenarian Maharashtrian female.

Behlim T, Sharma YK, Chaudhari ND, Dash K - Indian Dermatol Online J (2014)

Healed lesions with hyperpigmentation in between fingers of right hand and over right palm
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4228647&req=5

Figure 1: Healed lesions with hyperpigmentation in between fingers of right hand and over right palm
Mentions: A debilitated, non-ambulatory female aged 91 years, known case of Parkinsonism since 2 years, presented to us with the history of appearance of multiple blisters over palms and soles since one month. Local itching and burning preceded their onset. There was no significant antecedent drug intake. There was no icterus, pallor, lymphadenopathy, clubbing or organomegaly. Neurological examination revealed mask like facies, glabellar tap, pill rolling tremors of the right hand, cogwheel rigidity and hypertonia of limbs. Lateral and palmoplantar aspects of hands [Figure 1] and feet [Figure 2] showed dark brown encrusted erosions and a few tense clear bullae with negative Nikolsky- and Asboe-Hansen signs. Histopathological examination findings of subepidermal bulla containing eosinophilic infiltrate [Figure 3] in the lesional skin and IgG at dermoepidermal junction (DEJ) on direct immunofluorescense [Figure 4] confirmed our clinical suspicion of BP. Peripheral blood smear and ultrasonography abdomen were normal. Advanced imaging studies could not be carried out due to kyphoscoliosis, chest X-ray evidence of scoliosis to the right and her confinement to the wheelchair. Advanced confirmational studies viz., immunoblot/immunoprecipitation could not be afforded by the patient. Treatment with tetracycline, nicotinamide and dapsone showed appreciable improvement after a fortnight. She insisted to go home on discharge and passed away a month later due to gastrointestinal bleeding.

Bottom Line: Dyshidrosiform pemphigoid is a rare variant of bullous pemphigoid localized to the hands and feet whose characteristic subepidermal blisters develop as a result of binding of the IgG autoantibodies to intracellular plaque and extracellular face of the hemidesmosome recognizing a 230-kDa plakin molecule (BP230, BPAg1or BPAg1e) and a 180-kDa transmembrane protein.Neurodegenerative processes (viz., stroke, dementia, Parkinsonism, epilepsy, etc) uncover BPAg1-n, an alternatively spliced form of BPAg1-e that stabilizes the cytoskeleton of sensory neurons, generating autoantibodies that may subsequently lead to BP by cross-reacting with BPAg1-e.We present a patient with Parkinsonism who later developed blisters, erosions and crusts localized to the palms and soles, confirmed histopathologically as bullous pemphigoid.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Dr. D. Y. Patil Medical College and Hospital, Pimpri, Pune, Maharashtra, India.

ABSTRACT
Dyshidrosiform pemphigoid is a rare variant of bullous pemphigoid localized to the hands and feet whose characteristic subepidermal blisters develop as a result of binding of the IgG autoantibodies to intracellular plaque and extracellular face of the hemidesmosome recognizing a 230-kDa plakin molecule (BP230, BPAg1or BPAg1e) and a 180-kDa transmembrane protein. Neurodegenerative processes (viz., stroke, dementia, Parkinsonism, epilepsy, etc) uncover BPAg1-n, an alternatively spliced form of BPAg1-e that stabilizes the cytoskeleton of sensory neurons, generating autoantibodies that may subsequently lead to BP by cross-reacting with BPAg1-e. We present a patient with Parkinsonism who later developed blisters, erosions and crusts localized to the palms and soles, confirmed histopathologically as bullous pemphigoid. To the best of our knowledge, ours is the first case report from India wherein Parkinsonism-generated autoantibodies led to the development of dyshidrosiform pemphigoid due to their cross-reactivity with BPAg1-e.

No MeSH data available.


Related in: MedlinePlus