Dermatologic Manifestations

of Chronic kidney Disease

Dr. D M J S Dissanayake
Registrar im medicine
 Introduction
• Dermatologic manifestations of renal disease are common findings in
patients with end-stage renal disease (ESRD).

• Cutaneous examination of patients with ESRD has shown that 50-100% of

patients have at least 1 dermatologic condition.

• A high prevalence of cutaneous disorders is expected, because most

patients with ESRD have an underlying disease process with cutaneous
manifestations.
Many factors are involved in the pathogenesis of the cutaneous
manifestations of ESRD,

• including electrolyte imbalance,

• buildup of uremic substances,
• comorbid disease

Some conditions are easily diagnosed but can be difficult to manage.

Others are less straightforward to diagnose, largely because of clinical
similarities with more common diseases, and they are notoriously
difficult to manage.
Dermatologic manifestations of renal disease may be divided into 3
categories:

(1) Dermatologic manifestations of diseases associated with the

development of ESRD,
(2) Dermatologic manifestations of uremia,
(3) Dermatologic disorders associated with renal transplantation.
 Dermatologic Manifestations of Diseases
Associated With ESRD
• Diabetes Mellitus - most common cause of ESRD, responsible for
approximately 45% of all patients on renal replacement therapy.
Hypertension accounts for approximately 30% of cases, and
glomerulonephritis and cystic kidney diseases account for about 20%.

• The remaining causes of ESRD included vasculitis from an infectious or

rheumatologic disease, interstitial nephritis, tumors, cholesterol emboli, and
systemic amyloidosis.

• Infectious causes of glomerulonephritis included streptococcal infections,

human immunodeficiency virus (HIV) infection, and hepatitis viral infections,
both HCV and HBV.
Systemic Disorder Renal Disorder Dermatologic Manifestations

Diabetes mellitus Diabetic nephropathy Diabetic dermopathy

Necrobiosis lipoidica
Acanthosis nigricans
Eruptive xanthomas
Kyrle disease

Systemic lupus erythematosus Glomerulonephritis Purpura

Chronic cutaneous lupus
Nephrotic syndrome Subacute cutaneous lupus
Photosensitivity
Mucosal ulcers
Vasculitis
Systemic lupus erythematosus–
associated neutrophilic dermatosis

Henoch-Schönlein purpura Glomerulonephritis Purpura

Vasculitis
Dermatologic Manifestations of Uremia
• Many of the dermatologic manifestations associated with uremia,
such as pruritus and xerosis, are prevalent in the ESRD population but
are not specific for uremia.

• Other manifestations, especially those related to the dialysis

procedure, (Calcinosis Cutis, Calciphylaxis, Kyrle Disease, Bullous
Disease of Dialysis, Nephrogenic Fibrosing Dermopathy.)
Primary dermatologic manifestations associated with uremia:

• Xerosis
• Pruritus
• Pigmentary alteration
• Half-and-half nails
• Alopecia
• Uremic frost
• Porphyria cutanea tarda
• Arterial steal syndrome
Xerosis

• Significant xerosis occurs for unknown reasons in 50-92% of the

dialysis population.
• Some patients may develop acquired ichthyosis.
• Most commonly seen over the flexor surfaces of the forearm, legs and
thighs.
• The abdomen and chest may show fine scaling.
• Many patients respond to routine use of emollients.
 Pruritus
• Uremia - most common
• Incidence - 50-90% of the dialysis population.
• Development of pruritus has no consistent association with age, sex, race, or
precipitating disease.
• Pruritus may be episodic or constant, localized or generalized, and mild or severe.
forearms and upper back predominately are affected

• Pruritus frequently affects the patient's sleep pattern and psychologic well-being.
• Cutaneous manifestations of pruritus include excoriations, prurigo nodularis, and
lichen simplex chronicus.
Mechanisms - poorly understood and are believed to result from metabolic
disequilibrium. Some causes of uremic pruritus include the following:

• Xerosis
• Decreased transepidermal elimination of pruritogenic factors
• Hyperparathyroidism
• Hypercalcemia
• Hyperphosphatemia
• Elevated histamine levels
• Increased dermal mast cell proliferation
• Uremic sensory neuropathy
• Middle molecule theory
Therapeutic options,

• Emollients to alleviate xerosis,

• augmentation of dialysis efficacy,
• normalization of serum calcium and phosphate levels, and
parathyroidectomy.
For some, sedating antihistamines and sauna therapy may provide
temporary relief. Nonsedating antihistamines and topical steroids
usually are not helpful.

• Ultraviolet (UV) B phototherapy probably is the most effective

therapeutic choice and may have prolonged benefit.
 Pigmentary alteration
• Pigmentary alteration occurs in 25-70% of the dialysis population and
increases over time.
• Before the widespread use of erythropoietin, pallor was common and was
attributed to the significant anemia.
• A brown–to–slate-gray discoloration may occur as a result of hemosiderin
deposition in association with iron overload from excessive transfusions.
• Many patients develop a yellowish hue, which has been attributed to
retained urochromes and carotene, which are subsequently deposited in
the epidermis and subcutaneous tissues.
• A brownish hyperpigmentation is common, mostly in a sun-exposed
distribution. This hyperpigmentation results from an increase in melanin
production because of an increase in poorly dialyzable beta-melanocyte
stimulating hormone.
Half-and-half nails

• Not pathognomonic for renal failure, these nails occur in as many as

40% of patients on dialysis and disappear several months after
successful renal transplantation.
• Half-and-half nails are characterized by a dark distal band of the nail
bed and by a white proximal band.
• The distal dark band may range in color from reddish to brown.
• Although the condition has been observed on toenails, it more
commonly involves fingernails.
Other nail changes

• Koilonychia
• Subungual hyperkeratosis
• Onycholysis
• Splinter haemorrhages
• Brown nail bed arcs
Hair abnormalities / Alopecia
• Alopeca is more common in end-stage renal disease (ESRD) than in
the general population.

• Likely causes of alopecia in ESRD include systemic lupus

erythematosus (SLE) or chronic telogen effluvium.

• Chronic telogen effluvium may be related to the multitude and

severity of illnesses encountered by patients, or it may be related to
commonly used medications (heparin, antihypertensives, or lipid-
lowering agent)
Uremic frost

• Uremic frost is rarely seen in the present day because of early dialytic
intervention.
• When the blood urea nitrogen (BUN) level is adequately high (usually
> 250-300 mg/dL), the concentration of urea in sweat is increased
greatly.
• Evaporation results in the deposition of urea crystals on the skin.
• Uremic frost is commonly found in the beard or on other parts of the
face, neck, and trunk as fine white-to-yellow crystals that dissolve
readily when challenged by a drop of water.
Bullous Disease of Dialysis
• Syndrome of cutaneous fragility and blistering
• Sun-exposed skin mostly –dorsum of hands
• Resembles porphyria
• Plasma porphyrin level normal or minimally elevated
• Mechanical fragility of skin subjected to sunlight and incidental
trauma.
• Treatment –protect from sunlight, avoid trauma
Purpura /Ecchymosis / Easy bruising
Calaiphylaxis / Calcific Uremic Arteriolopathy
Acquired Perforating Dermatosis
 Arterial steal syndrome
• The arterial steal syndrome is an uncommon but highly morbid complication of the
vascular access necessary for hemodialysis. Vascular access typically is placed in
the nondominant upper extremity.

• The arterial steal syndrome may develop if the inevitable proximal shunting of
blood is significant enough to cause hand ischemia.
• Proximal shunting is attributed to the reversal of blood flow through distal arteries,
induced by the low-pressure system produced by the arteriovenous connection.
• Symptoms of arterial steal syndrome include pain and numbness.
• Prolonged ischemia may result in digital gangrene, peripheral neuropathy, or
cutaneous atrophy.
• Individuals at heightened risk for this complication include those with peripheral
vascular disease, especially diabetes mellitus.
Dermatologic Disorders Associated With
Renal Transplantation

• The best therapeutic option for many patients with end-stage renal
disease (ESRD) is renal allograft transplantation. Successful
transplantation results in regression of many of the metabolic and
cutaneous changes of uremia.

• Unfortunately, renal transplantation has its own set of complications,

primarily resulting from the immunosuppressive medications that are
essential for allograft survival
 Medication-related dermatologic disorders

• Cushingoid changes (moon facies, development of a cervical fat pad, striae,

cutaneous atrophy, and telangiectasias).
• Gingival hyperplasia (see the following image)
• Disorders of the pilosebaceous unit, including acne, folliculitis, hypertrichosis,
keratosis pilaris, sebaceous gland hyperplasia, epidermal cysts

A full-blown Cushingoid appearance develops in 55-90% of patients and is associated

with the high doses of corticosteroids used early after transplantation. Changes may
resolve or improve when the corticosteroid dose is reduced,
Gingival hyperplasia, which occurs in approximately one third of patients receiving
cyclosporin A (CyA), tends to occur early and improve over time.
 Immunosuppression-related disorders

• Viral infections, Herpes simplex virus (HSV), VZV, EBV

• Bacterial infections, Staphylococcus aureus, Mycobacterium tuberculosis, and
atypical mycobacteria
• Fungal infections, superficial mycoses (eg, dermatophytes, Pityrosporum species,
candidiasis) and deep fungal infections (eg, Aspergillus, Cryptococcus, Nocardia)
• Parasitic infection, Scabies
• Actinic keratoses
• Malignancies, Squamous cell carcinoma, keratoacanthoma, basal cell carcinoma,
Kaposi sarcoma, melanoma
• Miscellaneous malignancies, lymphoma, Merkel cell carcinoma (MCC)
• Miscellaneous disorders, including transfusion-associated graft-versus-host disease
and porokeratosis
Summary

• A broad range of skin diseases occurs in patients with ESRD, from the
benign and asymptomatic to the physically disabling and life-
threatening. Many of them negatively impact on quality of life. Their
early recognition and treatment are essential in reducing morbidity
and mortality.
• The cutaneous manifestations can be divided into two main
categories: nonspecific and specific. The nonspecific manifestations
are commonly seen and include skin color changes, xerosis, half-and-
half nails, and pruritus. The specific disorders include acquired
perforating dermatosis, bullous dermatoses, metastatic calcification,
and nephrogenic systemic fibrosis
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