Case 1.
A 90-year-old man presented with blisters on the trunk, arms, skin, and scalp (Figures A-F).
a. Bullous lupus erythematosus
b. Dermatitis herpetiformis
c. Bullous pemphigoid
d. Linear IgA dermatosis
Case 2.
A young man with a complicated medical history had a prolonged stay in the intensive care unit. He had multiple ulcerated lesions of the buttock. There is a superficial ulcer that, in part, is likely of traumatic-based etiology (Figures A-D).
a. Coma blister
b. Fixed drug eruption
c. Bullosis diabeticorum
d. Epidermolysis bullosa acqusita
Case 3.
This specimen from a 40-year-old man with a scalp lesion shows a circumscribed nodule composed of monomorphous appearing basaloid islands invested by condensed eosinophilic stroma (Figures A-C).
a. Cylindroma
b. Spiradenoma
c. Chondroid syringoma
d. Trichoblastoma
To learn the answers, go to page 2
QUIZ ANSWERS
Case 1 — C
Bullous Pemphigoid
Bullous pemphigoid (BP) is an autoimmune vesiculobullous disorder whereby the pathogenesis is caused by autoantibodies to components of the basement membrane of the skin. These are BP antigen 180 (type XVII collagen and the most frequent antigenic target) and BP antigen 230 (component of the hemidesmosome).
Case 2 — A
Coma Blister
The fundamental process in the skin is one of acute necrosis of the eccrine apparatus. This is a finding characteristic for the coma blister and related iatrogenic/exogenous drug-induced insults with attendant local complement activation and vascular injury, namely Nicolau syndrome and with local installation of certain analgesic compounds. The histopathology does not support a diagnosis of atypical hemolytic uremic syndrome, Degos disease, or any type of endogenous syndrome related to endothelial cell injury. One might question whether the changes on all 3 biopsies relate to agents, specifically barbiturates. The actual pathophysiologic events that lead to the development of skin lesions that fall under the general umbrella of coma blister likely include local and generalized hypoxia and the direct toxic effects of the drug along with the ability of certain drugs to potentially activate complement deposition. In this case, the most striking complement component is deposited in C4d; however, complement activation is a temporal event and not all components of complement activation are specifically present in this biopsy.
Case 3 — A
Cylindroma
Most cylindromas present as single papules of the head and neck in middle-aged or elderly individuals. Multiple disfiguring tumors of the scalp have been described and likened to a “turban tumor.” Multiple tumors can be seen in the setting of CYLD tumor suppressor gene mutations. Characteristic features of a cylindroma include monomorphous appearing basaloid islands, eosinophilic hyaline-like globules, thickened eosinophilic basement membrane surrounding the lobules, and a jigsaw puzzle-like pattern of growth. It is not uncommon to encounter tumors showing features of both cylindroma and spiradenoma.
Dr Magro is the director of dermatopathology at Weill Cornell Medicine in New York, NY. For more information, please visit www.weillcornelldermpath.com.
Disclosure: The author reports no relevant financial relationships.
Case 1.
A 90-year-old man presented with blisters on the trunk, arms, skin, and scalp (Figures A-F).
a. Bullous lupus erythematosus
b. Dermatitis herpetiformis
c. Bullous pemphigoid
d. Linear IgA dermatosis
Case 2.
A young man with a complicated medical history had a prolonged stay in the intensive care unit. He had multiple ulcerated lesions of the buttock. There is a superficial ulcer that, in part, is likely of traumatic-based etiology (Figures A-D).
a. Coma blister
b. Fixed drug eruption
c. Bullosis diabeticorum
d. Epidermolysis bullosa acqusita
Case 3.
This specimen from a 40-year-old man with a scalp lesion shows a circumscribed nodule composed of monomorphous appearing basaloid islands invested by condensed eosinophilic stroma (Figures A-C).
a. Cylindroma
b. Spiradenoma
c. Chondroid syringoma
d. Trichoblastoma
To learn the answers, go to page 2
QUIZ ANSWERS
Case 1 — C
Bullous Pemphigoid
Bullous pemphigoid (BP) is an autoimmune vesiculobullous disorder whereby the pathogenesis is caused by autoantibodies to components of the basement membrane of the skin. These are BP antigen 180 (type XVII collagen and the most frequent antigenic target) and BP antigen 230 (component of the hemidesmosome).
Case 2 — A
Coma Blister
The fundamental process in the skin is one of acute necrosis of the eccrine apparatus. This is a finding characteristic for the coma blister and related iatrogenic/exogenous drug-induced insults with attendant local complement activation and vascular injury, namely Nicolau syndrome and with local installation of certain analgesic compounds. The histopathology does not support a diagnosis of atypical hemolytic uremic syndrome, Degos disease, or any type of endogenous syndrome related to endothelial cell injury. One might question whether the changes on all 3 biopsies relate to agents, specifically barbiturates. The actual pathophysiologic events that lead to the development of skin lesions that fall under the general umbrella of coma blister likely include local and generalized hypoxia and the direct toxic effects of the drug along with the ability of certain drugs to potentially activate complement deposition. In this case, the most striking complement component is deposited in C4d; however, complement activation is a temporal event and not all components of complement activation are specifically present in this biopsy.
Case 3 — A
Cylindroma
Most cylindromas present as single papules of the head and neck in middle-aged or elderly individuals. Multiple disfiguring tumors of the scalp have been described and likened to a “turban tumor.” Multiple tumors can be seen in the setting of CYLD tumor suppressor gene mutations. Characteristic features of a cylindroma include monomorphous appearing basaloid islands, eosinophilic hyaline-like globules, thickened eosinophilic basement membrane surrounding the lobules, and a jigsaw puzzle-like pattern of growth. It is not uncommon to encounter tumors showing features of both cylindroma and spiradenoma.
Dr Magro is the director of dermatopathology at Weill Cornell Medicine in New York, NY. For more information, please visit www.weillcornelldermpath.com.
Disclosure: The author reports no relevant financial relationships.
Case 1.
A 90-year-old man presented with blisters on the trunk, arms, skin, and scalp (Figures A-F).
a. Bullous lupus erythematosus
b. Dermatitis herpetiformis
c. Bullous pemphigoid
d. Linear IgA dermatosis
Case 2.
A young man with a complicated medical history had a prolonged stay in the intensive care unit. He had multiple ulcerated lesions of the buttock. There is a superficial ulcer that, in part, is likely of traumatic-based etiology (Figures A-D).
a. Coma blister
b. Fixed drug eruption
c. Bullosis diabeticorum
d. Epidermolysis bullosa acqusita
Case 3.
This specimen from a 40-year-old man with a scalp lesion shows a circumscribed nodule composed of monomorphous appearing basaloid islands invested by condensed eosinophilic stroma (Figures A-C).
a. Cylindroma
b. Spiradenoma
c. Chondroid syringoma
d. Trichoblastoma
To learn the answers, go to page 2
QUIZ ANSWERS
Case 1 — C
Bullous Pemphigoid
Bullous pemphigoid (BP) is an autoimmune vesiculobullous disorder whereby the pathogenesis is caused by autoantibodies to components of the basement membrane of the skin. These are BP antigen 180 (type XVII collagen and the most frequent antigenic target) and BP antigen 230 (component of the hemidesmosome).
Case 2 — A
Coma Blister
The fundamental process in the skin is one of acute necrosis of the eccrine apparatus. This is a finding characteristic for the coma blister and related iatrogenic/exogenous drug-induced insults with attendant local complement activation and vascular injury, namely Nicolau syndrome and with local installation of certain analgesic compounds. The histopathology does not support a diagnosis of atypical hemolytic uremic syndrome, Degos disease, or any type of endogenous syndrome related to endothelial cell injury. One might question whether the changes on all 3 biopsies relate to agents, specifically barbiturates. The actual pathophysiologic events that lead to the development of skin lesions that fall under the general umbrella of coma blister likely include local and generalized hypoxia and the direct toxic effects of the drug along with the ability of certain drugs to potentially activate complement deposition. In this case, the most striking complement component is deposited in C4d; however, complement activation is a temporal event and not all components of complement activation are specifically present in this biopsy.
Case 3 — A
Cylindroma
Most cylindromas present as single papules of the head and neck in middle-aged or elderly individuals. Multiple disfiguring tumors of the scalp have been described and likened to a “turban tumor.” Multiple tumors can be seen in the setting of CYLD tumor suppressor gene mutations. Characteristic features of a cylindroma include monomorphous appearing basaloid islands, eosinophilic hyaline-like globules, thickened eosinophilic basement membrane surrounding the lobules, and a jigsaw puzzle-like pattern of growth. It is not uncommon to encounter tumors showing features of both cylindroma and spiradenoma.
Dr Magro is the director of dermatopathology at Weill Cornell Medicine in New York, NY. For more information, please visit www.weillcornelldermpath.com.
Disclosure: The author reports no relevant financial relationships.
