Kodachromes
Pathophysiology Pearls
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100
What is bullous pemphigoid?
100
Dapsone is thought to help with dermatitis herpetiformis via its effect on this specific cell type.
What is neutrophils? (neutrophil chemotaxis and neutrophil attachment to IgA)
100
Aside from blood, these two broad types of biochemical tests are standard in the evaluation of porphyrias.
What is urine and fecal studies?
100
This grain is safe for people with dermatitis herpetiformis
What is oats?
Rice/corn is also acceptable
100
This medication is most commonly implicated in linear IgA bullous dermatosis.
What is vancomycin?
200
Name 3 porphyrias where patients can have neurovisceral attacks.
What are
1. acute intermittent porphyria (AIP) - most common
2. variegated porphyria (VP)
3. hereditary coproporphyria (HCP)
4. ALA-D deficiency porphyria (ALA-D)?
Pearl important to know that not all porphrias have other systemic manifestations
200
You should screen for this condition if a patient presents with this following a mosquito bite.
What is chronic lymphocytic leukemia?
200
The risk of this cancer is elevated in uncontrolled dermatitis herpetiformis.
What is lymphoma (non-Hodgkins)?
200
Methemoglobinemia, agranulocytosis, and peripheral neuropathy are 3 of the more serious potential side effects of this medication.
What is dapsone?
Methemoglobemia if on >100mg dapsone qd
Agranulocytosis 2-12 weeks (watch for fever/sore throat/infection)
Peripheral neuropathy (high dose 200 mg+ or high cumulative dose (25-500 g)
300
The IgA antibodies of linear IgA bullous dermatosis bind to this antigen.
What is BPAG2 (carboxy terminus) - same target but different part of antigen from bullous pemphigoid
Why clinically it can look similar with tense bullae!
300
Biopsy of a coma blister can differentiate it from a friction blister due to split at this level
What is subepidermal split?
(Also will have sweat gland/follicular necrosis)
300
This therapy (aside from trigger avoidance) can lead to resolution of skin fragility and blistering in porphyria cutanea tarda via its effect on iron stores.
What is phlebotomy?
Phlebotomy reduces iron stores --> reduces iron induced inhibition of uroporphyrinogen decarboxylase
Of note, NOT helpful in acute porphyrias
300
This medication is can trigger tense bullae as seen in the photo below
What is furosemide (although will accept diuretics)?
400
What is bullous diabeticorum
(acral, pauciinflammatory, not typical site of pressure)
400
This organ is where uroporphyrinogen decarboxylase is found to be dysfunctional in the most common type of porphyria cutanea tarda.
What is the liver?
Hence - why it can be triggered by hepatotoxins e.g. alcohol
400
This histology method can help differentiate epidermolysis bullosa acquisita from bullous pemphigoid.
What is salt split skin? (u serrated pattern for EBA)
Pearls: can be done direct or indirect
400
This specific part of the electromagnetic spectrum needs to be covered for patients experiencing pseudoporphyria.
Green, J. J., & Manders, S. M. (2001). Pseudoporphyria. Journal of the American Academy of Dermatology, 44(1), 100-108.
What is UVA?
Hence - need broad spectrum sunscreen
Need also protection need windows/windshields
400
This antiseptic should be avoided if possible for patients with the below condition.
What is povidone iodine?
Reunala, T., Hervonen, K., & Salmi, T. (2021). Dermatitis herpetiformis: an update on diagnosis and management. American Journal of Clinical Dermatology, 22(3), 329-338.
500
What is mucous membrane pemphigoid?
Symblepharon
Pearl: All bullous disorders - look at the eyes!
(this condition overall is poorly defined)
500
This disease also shares the collagen target of epidermolysis bullosa acquista as shown below.
What is bullous SLE?
500
This is the more sensitive serologic test that can be used in the workup (and monitoring) of dermatitis herpetiformis
What is IgA anti-Tg3 antibodies?
anti-Tg2 = intestine only
anti-tg3 = develops later in life (caused by epitope spreading from continued exposure to gliadin) and is responsible for skin component
500
The administration of afamelanotide for EPP is performed via the following method.
What is in the office, subcutaneously 2cm above the ASIS?
500
This class of drugs is a common cause of the below photodistributed lesions in a patient with normal urine and stool studies.
What is NSAIDs?
Gil‐Lianes, J., Luque‐Luna, M., Morgado‐Carrasco, D., & Aguilera‐Peiró, P. (2022). Pseudoporphyria—a diagnostic challenge: A case series and a proposed diagnostic algorithm. Photodermatology, Photoimmunology & Photomedicine, 38(6).