Renal Dermatology

Question 1

derm findings associated with EGPA

Answer 1

*leukocytoclastic vasculitis
*purpura

Question 2

leukocytoclastic vasculitis

Answer 2

*palpable purpura: nonblanching violaceous or dusky papules or plaques, typically seen on lower extremities
*seen in a variety of diseases, including EGPA, GPA, IgA vasculitis (Henoch-Schoenlein)

Question 3

purpura

Answer 3

*extensive ecchymoses and purpura
*can be associated with EGPA, among other conditions

Question 4

nephrogenic fibrosing dermopathy - overview

Answer 4

*aka nephrogenic systemic fibrosis, when involving other organs
*seen in pts with pre-existing kidney disease who are exposed to GADOLINIUM CONTRAST agents
*deposition of circulating fibroblasts
*requires deep skin biopsy b/c fibrosis is located in deep subcutaneous tissue
*treatments are ineffective

Question 5

nephrogenic fibrosing dermopathy - clinical presentation

Answer 5

*exam shows cobblestone appearance to skin, symmetric thick indurated firm plaques on arms and legs
*plaques can be skin colored, red, or hyperpigmented
*patients can have contractures if skin lesions overlie joints

Question 6

nephrogenic fibrosing dermopathy - histology

Answer 6

*deep subcutaneous fat showing proliferation of fibroblasts (spindle-shaped cells) and mucin infiltrating the major septae between the fat

Question 7

calciphylaxis - overview

Answer 7

*aka calcific uremic arteriolopathy (CUA)
*deposition of calcium in the walls of blood vessels in the subcutaneous fat of the skin, commonly seen on the hips, thighs, or legs
*risk factors: obesity, poor nutrition, coumadin use, female, ESRD, elevated phosphorous levels
*60% fatal within 12 months; pts usually die from secondary infections

Question 8

calciphylaxis - clinical presentation

Answer 8

*branching/retiform purpuric lesions on fatty areas with secondary ulceration and eschar
*extremely painful (10 out of 10 pain), causes starvation of tissue and ulceration

Question 9

derm findings associated with GPA

Answer 9

1. ocular findings
2. saddle nose deformity
3. strawberry gums
4. leukocytoclastic vasculitis
5. cutaneous ulcers

Question 10

ocular findings associated with GPA

Answer 10

*ocular involvement occurs in 30-50% of patients
*variety of eye findings, including:
-corneal and/or scleral ulceration
-uveitis
-conjunctivitis
-vasculitis of the optic, retinal, and ciliary vessels

Question 11

saddle nose deformity associated with GPA

Answer 11

*a sunken depression on the bridge of the nose
*occurs due to granulomatous involvement of the nasal septum and destruction of nasal cartilage
*one of the hallmark findings in GPA

Question 12

mucosal findings associated with GPA

Answer 12

*Strawberry Gums - one of the most common oral mucosal findings in GPA
*see hemorrhagic-appearing clusters of small papules or small ulcers along gingival mucosa

Question 13

cutaneous ulcers associated with GPA

Answer 13

*ulcers appear punched out on the legs/ankles
*can mimic pyoderma gangrenosum