Cafe-Au-Lait Spots, A Simple Guide To The Condition, Diagnosis, Treatment And Related Conditions

This book describes Cafe-Au-Lait Spots, Diagnosis and Treatment and Related Diseases
Cafe-au-lait spot is a flat, pigmented birthmark often manifesting as a hyper-pigmented skin patch with a sharp border and diameter of > 0.5 cm.
It is also known as circumscribed cafe-au-lait hyper-melanosis, von Recklinghausen spot, or abbreviated as "CALM".
The name cafe au lait is French for "coffee with milk" and refers to their light-brown color.
They are produced by a collection of pigment-forming melanocytes in the epidermis of the skin.
These spots are typically permanent, and may grow or increase in number over time.
Cafe au lait spots are often benign, not harmful but may be linked with syndromes such as neurofibromatosis type 1 and McCune–Albright syndrome.
Cafe-au-lait spots are normally evident at birth (congenital) or occur in early infancy.
They occasionally become apparent later in infancy, particularly after exposure to the sun, which darkens the color.
They may be isolated or linked with systemic diseases such as neurofibromatosis (NF), McCune Albright syndrome, Legius syndrome, and LEOPARD syndrome.

Causes:
The brown color of a cafe-au-lait spot is because of a pigment called melanin, produced in the skin by cells called melanocytes.
Multiple cafe-au-lait spots are related to several genetic syndromes.
Neurofibromatosis type 1 &2
Legius syndrome
McCune Albright syndrome,
LEOPARD syndrome.

Symptoms:
Cafe-au-lait spots:
1.They are light brown in color.
2.The pigment is evenly distributed.
3.They are well demarcated with smooth or irregular border
4.Their shape is often round or oval
Diagnosis
Cafe-au-lait spots are diagnosed visually with measurement of spot size
If significant in number and size, a complete medical examination should be done to find out whether a related syndrome may be present.
Syndromes may be diagnosed from their medical manifestations or by genetic testing

6 or more cafe au lait spots greater than 5 mm in diameter before puberty is a diagnostic feature of neurofibromatosis type I
Skin biopsy may be required for certain syndromes.

Treatment

No medical care is required to treat cafe-au-lait spots.
Cafe au lait spots can be removed with lasers.
Results are inconsistent as the spots are often not entirely removed or can recur after treatment.
Often, a test spot is treated first to help predict the likelihood of treatment success.
Lasers reported to have successfully faded cafe-au-lait spots are:

1.Pulsed-dye laser
2.Er:YAG laser
3.Q-switched Nd:YAG laser
4.Q-switched ruby or alexandrite laser

Results are not consistent.

A study has found lesions with an irregular margin react better than those with a smooth, well-defined border.

Risks for laser surgery are:

1.Transient or permanent hyper-pigmentation,
2.Hypo-pigmentation, and
3.Scarring.
4.Permanent hyper-pigmentation, and
5.Recurrence

Complete disappearance of cafe au liat spots was documented using a pulsed dye laser for 4-14 treatments, with no repeat occurrences at 12 months follow-up
Chinese patients were treated with the Q-switched alexandrite laser and 51.4% patients had good-to-excellent reactions after an average of 3.2 treatments with a low rate of recurrence (10.4%).
The results are normally preferred, and when considerable clearing is obtained, the repeat occurrence rate is low; where clearance is partial, repeat occurrence is as high as 50%.
The risk of post-inflammatory hyper-pigmentation is as high as 50% per treatment.
When this develops, it is essential to wait until it clears before resuming laser treatment.

Treatment of underlying syndromes may be complicated and need multidisciplinary care

TABLE OF CONTENT
Introduction
Chapter 1 Cafe-Au-Lait Spots
Chapter 2 Causes
Chapter 3 Symptoms
Chapter 4 Diagnosis
Chapter 5 Treatment
Chapter 6 Prognosis
Chapter 7 Elephant Man Disease
Chapter 8 Neurofibromatosis
Epilogue