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Skin Type: 5

Pemphigus Vulgaris

Author: Shahnawaz Towheed MS2, Y, Miller-Monthrope, Dermatologist/Dermatopathologist, Toronto, Canada, 2024

Definition: Pemphigus Vulgaris is an autoimmune blistering disorder affecting the skin and mucous membranes. 

Epidemiology: It has an incidence of approximately 1-5 cases per million people per year and predominantly affects middle-aged and older adults, with a higher prevalence in certain ethnic groups, such as the Ashkenazi Jewish population and patients of East Indian descent. 

Etiology: The condition is caused by autoantibodies against desmogleins, which are essential proteins in skin cell adhesion. 

Signs/ Symptoms: Clinically, it presents with flaccid blisters and erosions, particularly in the mouth, and can progress to involve the skin. 

Diagnosis: Diagnosis is confirmed through biopsies of involved skin and a biopsy for direct immunofluorescence of uninvolved skin, which shows IgG antibodies against the keratinocyte cell surface in a “chicken wire pattern”. 

Treatment: The treatment typically requires high-dose corticosteroids, and immunosuppressive agents like azathioprine, and rituximab.

Complications: The prognosis is generally favourable with appropriate management, though the condition can be chronic and require long-term treatment. Corticosteroids first used for PV in the 1950s reduced the mortality from 75% to 30%. This was further reduced to below 5% with the addition of other immunosuppressive agents introduced in the 1980s. 

References:

1.     Alpsoy E, Akman-Karakas A, Uzun S. Geographic variations in epidemiology of two autoimmune bullous diseases: pemphigus and bullous pemphigoid. Arch Dermatol Res. 2015;307(4):291-298. doi:10.1007/s00403-014-1531-1

2.     Russo I, De Siena FP, Saponeri A, Alaibac M. Evaluation of anti-desmoglein-1 and anti-desmoglein-3 autoantibody titers in pemphigus patients at the time of the initial diagnosis and after clinical remission. Medicine (Baltimore). 2017;96(46):e8801. doi:10.1097/MD.0000000000008801

3.     Kridin K, Sagi SZ, Bergman R. Mortality and Cause of Death in Patients with Pemphigus. Acta Derm Venereol. 2017 May 8;97(5):607-611. doi: 10.2340/00015555-2611. PMID: 28093595.

4.     Joly P, Maho-Vaillant M, Prost-Squarcioni C, et al. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial. Lancet. 2017;389(10083):2031-2040. doi:10.1016/S0140-6736(17)30070-3

Pemphigus Vulgaris

Author: Shahnawaz Towheed MS2, Y, Miller-Monthrope, Dermatologist/Dermatopathologist, Toronto, Canada, 2024

Definition: Pemphigus Vulgaris is an autoimmune blistering disorder affecting the skin and mucous membranes. 

Epidemiology: It has an incidence of approximately 1-5 cases per million people per year and predominantly affects middle-aged and older adults, with a higher prevalence in certain ethnic groups, such as the Ashkenazi Jewish population and patients of East Indian descent. 

Etiology: The condition is caused by autoantibodies against desmogleins, which are essential proteins in skin cell adhesion. 

Signs/ Symptoms: Clinically, it presents with flaccid blisters and erosions, particularly in the mouth, and can progress to involve the skin. 

Diagnosis: Diagnosis is confirmed through biopsies of involved skin and a biopsy for direct immunofluorescence of uninvolved skin, which shows IgG antibodies against the keratinocyte cell surface in a “chicken wire pattern”. 

Treatment: The treatment typically requires high-dose corticosteroids, and immunosuppressive agents like azathioprine, and rituximab.

Complications: The prognosis is generally favourable with appropriate management, though the condition can be chronic and require long-term treatment. Corticosteroids first used for PV in the 1950s reduced the mortality from 75% to 30%. This was further reduced to below 5% with the addition of other immunosuppressive agents introduced in the 1980s. 

References:

1.     Alpsoy E, Akman-Karakas A, Uzun S. Geographic variations in epidemiology of two autoimmune bullous diseases: pemphigus and bullous pemphigoid. Arch Dermatol Res. 2015;307(4):291-298. doi:10.1007/s00403-014-1531-1

2.     Russo I, De Siena FP, Saponeri A, Alaibac M. Evaluation of anti-desmoglein-1 and anti-desmoglein-3 autoantibody titers in pemphigus patients at the time of the initial diagnosis and after clinical remission. Medicine (Baltimore). 2017;96(46):e8801. doi:10.1097/MD.0000000000008801

3.     Kridin K, Sagi SZ, Bergman R. Mortality and Cause of Death in Patients with Pemphigus. Acta Derm Venereol. 2017 May 8;97(5):607-611. doi: 10.2340/00015555-2611. PMID: 28093595.

4.     Joly P, Maho-Vaillant M, Prost-Squarcioni C, et al. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial. Lancet. 2017;389(10083):2031-2040. doi:10.1016/S0140-6736(17)30070-3

Epidermal Nevus

Author: Kawaroreet Karwal MS2, Y, Miller-Monthrope, Dermatologist/Dermatopathologist, Toronto, Canada, 2024

Definition: Epidermal nevus is a benign skin condition characterized by the overgrowth of epidermal cells, resulting in the formation of warty or verrucous plaques. 

Etiology: These lesions typically present at birth or develop early in childhood and can persist throughout life.

Epidemiology: Epidermal nevi are rare, occurring in approximately 1 in 1,000 live births. They are most commonly found on the trunk, limbs, and neck. 

Signs: The lesions are often linear or segmental, following the lines of Blaschko, and can vary in colour from flesh-toned to brown.

Symptoms: Symptoms associated with epidermal nevus include pruritus and irritation, particularly if the lesions are inflamed or become secondarily infected. In rare cases, epidermal nevi can be part of broader syndromes, such as epidermal nevus syndrome, which may involve neurological, skeletal, and ocular abnormalities.

Treatment: Treatment for epidermal nevus is primarily aimed at managing symptoms and improving cosmetic appearance. Options include topical treatments, such as keratolytics and retinoids, and surgical interventions like excision, laser therapy, or cryotherapy. The choice of treatment depends on the size, location, and symptoms associated with the nevus.

References:

1.     Hafner, C., & Vogt, T. (2008). Sebaceous Gland Disorders. In Harper’s Textbook of Pediatric Dermatology (Vol. 2, pp. 235-246). Wiley-Blackwell.

2.     Happle, R. (2000). The group of epidermal nevus syndromes part I. Well-defined phenotypes. Journal of the American Academy of Dermatology, 42(2), 171-183.

3.     Patel, S., Zirwas, M., & English, J. C. (2010). Treatment of epidermal nevi: a systematic review. Journal of the American Academy of Dermatology, 62(1), 19-28.

Epidermal Nevus

Author: Kawaroreet Karwal MS2, Y, Miller-Monthrope, Dermatologist/Dermatopathologist, Toronto, Canada, 2024

Definition: Epidermal nevus is a benign skin condition characterized by the overgrowth of epidermal cells, resulting in the formation of warty or verrucous plaques. 

Etiology: These lesions typically present at birth or develop early in childhood and can persist throughout life.

Epidemiology: Epidermal nevi are rare, occurring in approximately 1 in 1,000 live births. They are most commonly found on the trunk, limbs, and neck. 

Signs: The lesions are often linear or segmental, following the lines of Blaschko, and can vary in colour from flesh-toned to brown.

Symptoms: Symptoms associated with epidermal nevus include pruritus and irritation, particularly if the lesions are inflamed or become secondarily infected. In rare cases, epidermal nevi can be part of broader syndromes, such as epidermal nevus syndrome, which may involve neurological, skeletal, and ocular abnormalities.

Treatment: Treatment for epidermal nevus is primarily aimed at managing symptoms and improving cosmetic appearance. Options include topical treatments, such as keratolytics and retinoids, and surgical interventions like excision, laser therapy, or cryotherapy. The choice of treatment depends on the size, location, and symptoms associated with the nevus.

References:

1.     Hafner, C., & Vogt, T. (2008). Sebaceous Gland Disorders. In Harper’s Textbook of Pediatric Dermatology (Vol. 2, pp. 235-246). Wiley-Blackwell.

2.     Happle, R. (2000). The group of epidermal nevus syndromes part I. Well-defined phenotypes. Journal of the American Academy of Dermatology, 42(2), 171-183.

3.     Patel, S., Zirwas, M., & English, J. C. (2010). Treatment of epidermal nevi: a systematic review. Journal of the American Academy of Dermatology, 62(1), 19-28.

Eczema Herpeticum

Eczema Herpeticum

Author: Kawaroreet Karwal MS2, Y, Miller-Monthrope, Dermatologist/Dermatopathologist, Toronto, Canada, 2024

Definition: Eczema herpeticum (also known as Kaposi varicelliform eruption) is a severe, potentially life-threatening skin infection caused by the herpes simplex virus (HSV) in individuals with atopic dermatitis or other underlying skin conditions. 

Epidemiology: It typically occurs in children and young adults but can affect individuals of any age. 

Symptoms: Eczema herpeticum usually presents with clusters of painful, monomorphic, punched-out erosions that rapidly spread over eczematous skin areas. The affected areas may become swollen, and the lesions can develop into pustules, vesicles, and crusts. Systemic symptoms such as fever, malaise, and lymphadenopathy may also be present. 

Diagnosis: Early diagnosis and treatment are crucial to prevent complications. Antiviral medications, such as acyclovir or valacyclovir, are the primary treatment options and should be administered promptly. In severe cases, hospitalization may be required for intravenous antiviral therapy and supportive care. Corticosteroids and antibiotics may be used to manage concurrent inflammation and bacterial infections.

References:

1.     Eichenfield, L. F., Tom, W. L., Chamlin, S. L., Feldman, S. R., Hanifin, J. M., Simpson, E. L., & Paller, A. S. (2014). Guidelines of care for the management of atopic dermatitis. Journal of the American Academy of Dermatology, 71(1), 116-132.

2.     Wollenberg, A., Oranje, A., Deleuran, M., Simon, D., Szalai, Z., Kunz, B., … & Taïeb, A. (2016). ETFAD/EADV eczema task force position paper on diagnosis and treatment of atopic dermatitis. Journal of the European Academy of Dermatology and Venereology, 30(5), 729-747.

3.     Hebert, A. A., & Rodriguez, L. A. (2016). Management of eczema herpeticum in children. Pediatric Dermatology, 33(4), 357-361.

Eczema Herpeticum

Eczema Herpeticum

Author: Kawaroreet Karwal MS2, Y, Miller-Monthrope, Dermatologist/Dermatopathologist, Toronto, Canada, 2024

Definition: Eczema herpeticum (also known as Kaposi varicelliform eruption) is a severe, potentially life-threatening skin infection caused by the herpes simplex virus (HSV) in individuals with atopic dermatitis or other underlying skin conditions. 

Epidemiology: It typically occurs in children and young adults but can affect individuals of any age. 

Symptoms: Eczema herpeticum usually presents with clusters of painful, monomorphic, punched-out erosions that rapidly spread over eczematous skin areas. The affected areas may become swollen, and the lesions can develop into pustules, vesicles, and crusts. Systemic symptoms such as fever, malaise, and lymphadenopathy may also be present. 

Diagnosis: Early diagnosis and treatment are crucial to prevent complications. Antiviral medications, such as acyclovir or valacyclovir, are the primary treatment options and should be administered promptly. In severe cases, hospitalization may be required for intravenous antiviral therapy and supportive care. Corticosteroids and antibiotics may be used to manage concurrent inflammation and bacterial infections.

References:

1.     Eichenfield, L. F., Tom, W. L., Chamlin, S. L., Feldman, S. R., Hanifin, J. M., Simpson, E. L., & Paller, A. S. (2014). Guidelines of care for the management of atopic dermatitis. Journal of the American Academy of Dermatology, 71(1), 116-132.

2.     Wollenberg, A., Oranje, A., Deleuran, M., Simon, D., Szalai, Z., Kunz, B., … & Taïeb, A. (2016). ETFAD/EADV eczema task force position paper on diagnosis and treatment of atopic dermatitis. Journal of the European Academy of Dermatology and Venereology, 30(5), 729-747.

3.     Hebert, A. A., & Rodriguez, L. A. (2016). Management of eczema herpeticum in children. Pediatric Dermatology, 33(4), 357-361.