Chromonychia: Pigmented Nail Disorders

Nail Anatomy

Nail plate actual nail made of tightly packed onychocytes; DIPJ in close proximity. Proximal Nail Fold Cuticle Nail Matrix Epithelium beneath nail plate; longitudinal grooves (melanoma, splinter hemorrhage); splinter hemorrhage-endocard trichinosis, trauma, PS, vasculitis) Hyponychium epithelum on volar surface of digit Lunula visible portion of distal matrix Lateral digital artery

Nail Anatomy Continued

Proximal portion of nail matrix gives rise to dorsal nail plate Distal portion of nail matrix produces the ventral nail plate Proximal matrix >> pitting(paraker), onychorrhexis, beaus lines Mid-matrix >> leukonychia Distal Matrix .. Onycholysis, thinned nail plate, erythema of the lunula Melanocytes more numerous in distal nail matrix than proximal (melanonychia)

Nail Embryology
Matrix

appears at 10 weeks as a wedge of basal like cells (matrix primordium) Earliest nail plate at 14 weeks Full nail at 17 weeks

Why do we have nails?

Protects

the fingertips and toes Fine touch/increases tactile sensitivity Essential for picking up small objects Toenails contribute to foot biomechanics

Nail Growth Rates

Fingernails 3mm/month; replacement = 6 months Toenails 1mm/month; replacement = 12 18 months Faster Growth Rates Psoriasis, males, youth, onycholysis, summer, periungual inflammation, onychophagia, day, premenstrual, pregnancy, hyperthyroids handedness. Slower growth rates: thumb and 5th finger, viral disease/fever, chemo, immobilization, poor nutrition, yellow nail syndrome

Acquired Melanonychia

Acquired Melanonychia
Case Presentation

80 year old woman presented with longitudinal melanonychia, or longitudinal dark pigmentation of the nails Noticed the pigmentation after a year since the start of hydroxyurea treatment for essential thrombocythemia

Case Presentation (Continued)

Patient

had no other side effects, no other hyperpigmentation, and was not taking any other medications that would have caused nail changes. So how did this happen?

Hydroxyurea Therapy

Hydroxyurea is used to treat a certain type of chronic myelogenous leukemia It is also used to treat ovarian cancer, melanoma, and certain types of head and neck cancer (including cancer of the mouth, cheek, tongue, throat, tonsils, and sinuses). Hydroxyurea is also used to reduce the frequency of painful crises and reduce the need for blood transfusions in patients with sickle cell anemia Hydroxyurea treats cancer by slowing or stopping the growth of cancer cells in your body. Hydroxyurea treats sickle cell anemia by helping to prevent formation of sickle-shaped red blood cells.

So what does this have to do with Melanonychia????

Well...

Melanonychia is a rare SIDE EFFECT of hydroxyurea therapy The differential diagnosis includes:

Subungal melanoma Pigmented squamous cell carcinoma Subungal hematoma Nevus Hyperpigmentation due to other drugs

Cyclophosphamide, doxorubicin, minocycline, and zidobudine

Mechanism of Hydroxyurea Therapy

Hydroxyurea

can cause a severe decrease in the number of blood cells in your bone marrow However, the mechanism of hydroxyurea induced melanonychia is UNKNOWN. Potential causes include:

Toxicity affecting the nail bed or nail matrix Focal stimulation of nail matrix melanocytes Photosensitization

What is Melanonychia

Melanin within the nail plate is usually produced by nail matrix melanocytes Active melanocytes transfer melanin-rich melanosomes by the way of dendrites to differentiating nail matrixderive onychocytes This can lead to a longitudinal streak along the nail plate

Other Causes of Melanonychia

Melanonychia is an ambiguous clinical finding that occurs as a result of benign etiologies:

Nail matrix melanocytic activation Nail matrix melanocytic hyperplasia Nail invasion by melanin producing pathogens

Other nail pathogens can cause NON MELANIC brown to black nail pigmentation

Exogenous substances Subungal hemorrhage Trauma Vitamin Deficiencies

How to Evaluate Melanonychia

NAIL MATRIX SHAVE BIOPSY!!!! Nail plate clipping can be sent for histological evaluation or histological analysis Examination of affected nails w/a dermatoscope Hutchinson sign a sign of subungal melanoma, however it is not the best predictor of disease!

Treatment of Melanonychia
Depends

on the etiology

Discontinue medication/therapy! Multivitamins Time Last two are if problem is not melanonychia, but rather an outcome of another etiology.

See

a Dermatologist

Green Nails

Introduction
A

53 year old intensive care nurse being treated with efalizumab for psoriasis involving her arms, legs and nails. After a few weeks of treatment the patient started to develop a strange discoloration of both thumbnails.

Oh my

Diagnosis
After

careful bacteriologic examination of the nail scrapings it was determined that the strange discoloration was caused by Pseudomonas aeruginosa and Klebsiella pneumoniae. Both organisms were shown to be sensitive to fluoroquinolone

Pseudomonas aeruginosa
A

Gram negative, aerobic, coccobacillum bacterium Commonly found in moist dwellings P. aeruginosa secretes a variety of pigments, including pyocynanin, pyoverdin, and pyorubin.

Pathomechanics of Infection
Psoriasis-induced

onycholysis Patient may have acquired when gardening or at work in the hospital. Frequent hand washing may have created a moist environment for infection.

Treatment
2

week course of topical Nadifloxacin

Others..
Mainly due to obvious cosmetic procedures, topical or oral drugs, or common diseases. None is of particular significance in itself, its presence sometimes aiding in the diagnosis of disease or pointing towards overdose of drugs.

White Nail (Leukonychia)

Most

common color change Two types, but techincally three

True leukonychia, nail plate involvement

Diffraction of light in abnormal keratotic cells

Apparent leukonychia with subungal tissue involvement

Underlying onycholysis and subungal hyperkeratosis Modification of matrix and or nail bed

Pseudoleukonychia, fungal infection involves nail plate

White Nail (Leukonychia) cont..

Causes:

Congenital and or hereditary

Acquired Pseduoleukonychia

Isolated, acrokeratosis, psoriasis, etc.

Apparent leukonychia

Diffuse distal and lateral subungual onychomycosis, superficial white onychomycosis, keratin granulation, etc.

True leukonychia

Anaemia, cancer chemotherapeutic agents, Cirrhosis, Leprosy, etc.

Alkaline metabolic disease, alopecia areata, Gout, Hodgkins disease, Occupational, Sickle cell, Tumors, etc.

Yellow Nail

Nail enamle and hardeners AIDS Carotene Dermatophyte onychomycosis Drugs

Tetracycle fluorsecent lunula Penicillamine Clioquinole (topical) Mepacrine (nail bed) Jaundice

Blue/Blue-Grey Nail
Antimalarials

Argyria
Bleomycin Congenital

Pernicious anaemia Minocycline Phenolphthalein Phenothiazines Wilsons disease

Red/Purple

Angioma Cirsoid aneurysm tumour Glomus tumor Congestive cardiac failure (lunula) Enchondroma Heparin Lichen planus Linear red line

Lupus erythematosus Porphyria (with fluorescence) Rheumatoid arthritis Warfarin

Dariers disease Benign tumours/cysts near proximal matrix

Citations

Baran, R., Julian Barth, and R. P. R. Dawber. Nail Disorders: Common Presenting Signs, Differential Diagnosis, and Treatment. New York: Churchill Livingstone, 1991. Print. Stern, Dana. "Nail Disorders." Dermatology Lecture. The New York College of Podiatric Medicine, New York. 07 Feb. 2013. Lecture. Ranta, Dana MD and Bonmati, Caroline MD. Acquired Melanonychia. New England Journal of Medicine 2009; 361:1188 September 17, 2009. Hengge, Ulrich R. MD and Bardeli, Virginia MD; Green Nails New England Journal of Medicine March 12, 2009

Now youre ready to nail nail pathology!