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Graham
Little Piccardi Lassueur syndrome introduction
For dermatologists and clinico-pathologists, scarring alopecia
has been an enigma, a mystery for years. The intriguing role of
genes, abnormal immune responses, abnormal follicular structures,
stem cell depletion and defective hair follicle cycling have aroused
a great deal of interest in hair loss research. A lot of headway
has been made, but not enough, and researchers all over the world
endeavor to unravel the mystery of scarring alopecia.
The complexity of diagnosis with scarring alopecias arises because
often the clinical features of each type of cicatricial alopecia
overlap with other types. Often a conclusive diagnosis can be elusive,
and a scalp biopsy is critical for assessment of the patient. Very
often, alopecia with different manifestations seen in two different
people may be just specific clinical aspects in a broader spectrum
of the same disease. Also, different clinical symptoms other than
those classically documented may be considered variants of a classified
form of alopecia. On the other hand, some forms of hair loss that
look similar in two different people may be completely different
types of scarring alopecia.
Graham Little Piccardi Lassueur syndrome (GLPLS) is an uncommon
condition of adults, and regarded by many authors (but not all)
as a variant of lichen planopilaris. Lichen planopilaris is a kind
of primary lymphocytic alopecia, which exhibits skin flaking and
pruritis (itching) with distinct patches of hair loss. The patches
expand and coalesce over time, in the advanced stages of the disease.
Graham Little Piccardi Lassueur syndrome or Graham Little syndrome,
as it is commonly referred to, is a rare lichenoid dermatosis defined
by scarring alopecia of the scalp, loss of pubic and axillary (armpit)
hairs and progressive development of horny follicular papules or
bumps in various locations. Dermatosis is a broad term that refers
to any disease of the skin, especially one that is not accompanied
by inflammation, and is not to be confused with dermatitis, which
is limited to inflammation of the skin.
Some authors also consider Graham Little Piccardi Lassueur syndrome
a distinctive entity presenting as a scarring patchy alopecia of
the scalp, a non-scarring axillary and pubic hair loss and a chronic
skin eruption characterized by hardening and thickening of the skin
with follicular papules.
Graham Little Piccardi Lassueur syndrome clinical features
Graham Little syndrome presents features similar to lichen planopilaris,
such as small, confluent patches of progressive scarring alopecia.
The clinical presentations that differentiate it from classic lichen
planopilaris include non-cicatricial alopecia of the armpits and
pubic areas. There are infrequent reports of face and eyebrow involvement
as well.
The scalp alopecia may develop at any time during the course of
the disease and the patches on the scalp are atrophic (wasted away – often
seen as a depression in the skin) and hence, indicate follicular
degeneration. In active disease, the patches are scarred in the
center, but erythematous (reddened and inflamed) and scaly around
the edges. This pinpoints to the occurrence of follicular hyperkeratosis
or abnormal keratinization.
Graham Little Piccardi Lassueur syndrome differential diagnosis
The diagnosis of Graham Little Piccardi Lassueur syndrome should
be fairly distinct compared to the classic presentations of other
scarring alopecias. The combination of follicular lichen planus
scarring alopecia of scalp plus non-scarring alopecia of the axilla
and pubis is the diagnostic hallmark of Graham Little syndrome.
However, if the interviewing physician focuses on the scalp alopecia
and does not identify the underarm or pubic hair loss then Graham Little Piccardi Lassueur syndrome may not be diagnosed. The condition
also resembles lichen spinulosus or keratosis pilaris on the trunk
and extremities. Keratosis pilaris is a genetic condition of the
skin in which the hair follicles become plugged with hair and dead
cells from the outermost layer of skin. The follicles redden and
inflame, causing numerous tiny rough pink bumps on the surface of
the skin. Again, if the examination is cursory the keratosis pilaris
may be identified, but the diagnosis of Graham Little Piccardi Lassueur syndrome may not be made if other symptoms are missed. It is the
particular composition of clinical symptoms that identify Graham Little Piccardi Lassueur syndrome, though each of the symptoms can
be seen in other diagnoses.
Graham Little Piccardi Lassueur syndrome pathology
Scalp biopsy is critical to identifying a particular form of scarring
alopecia. Irrespective of the overlapping features, tissue studies
and evaluation under the microscope often help differentiate one
form of scarring alopecia from another and direct the clinicians
to a definitive diagnosis.
When treating a patient with inflammatory scarring alopecia, normally
a two-pronged approach is adopted. Scalp examination is carried
out to view the scarred as well as hair bearing areas, to give valuable
clues to the pathologic condition. Localization of symptoms directs
the clinician to active areas of disease. As the clinical condition
of the Graham Little syndrome overlaps with other forms of scarring
alopecia, scalp biopsy tests are conducted, to confirm clinically
prompted diagnostic impressions. The scalp biopsy is a simple procedure
in which a small area of the scalp is removed after numbing medication
is administered. The site chosen for scalp biopsy is crucial to
the evaluation, as the pathogenic information obtained from a hair
bearing site with active disease is more productive than from bald
or end-stage diseased areas of the scalp. Thereafter, subjecting
the scalp biopsy sample (horizontal and vertical sections) through
the various standard histology tests leads to the proper detection
of the condition. The pathological examination proves the fact that
the histological features of hair follicles in Graham little syndrome
are similar to those seen in lichen planopilaris. However, the absence
of interface dermatitis of the overlying epidermis distinguishes
it from classic lichen planopilaris. This information in combination
with the required set of clinical features should be enough to diagnose
Graham Little Piccardi Lassueur syndrome.
Graham Little Piccardi Lassueur syndrome treatment
To date no effective treatment for properly treating the Graham Little Piccardi Lassueur syndrome has evolved. On the basis of the
limited reports available to us we are aware that topical or systemic
corticosteroids, retinoids or PUVA therapy are treatments which
have had partial and at least temporary effects on the scarring
alopecia condition.
Most often topical, intralesional and systemic corticosteroids
have been used in the treatment of Graham Little syndrome, as typically
done for lichen planopilaris. Corticosteroids are a group of anti-inflammatory
drugs similar to the natural corticosteroid hormones produced by
the cortex of the adrenal glands. The follicular papules presented
in Graham Little syndrome may also respond to topical retinoids,
a class of chemical compounds that are chemically related to vitamin
A. Retinoids slow down keratinocyte cell growth and this can help
reduce the development of the horny plugs that develop in Graham
Little syndrome.
PUVA therapy for Graham Little syndrome has been recommended by
some authors. PUVA is an acronym for Psoralen plus UVA light and
is a special type of phototherapy that combines the use of certain
drugs that sensitize inflammatory cells in the skin with exposure
of the skin to UVA (ultraviolet A) light. The sensitized inflammatory
cells are disrupted by the UVA light and the number of cells is
depleted.
Cyclosporine, a medication designed to suppress the immune system,
has shown some positive feedback in the treatment of Graham Little
syndrome. There is documentation to support that the notion that
cyclosporin A could be effective at least in the initial phases
of this rare variant of lichen planopilaris – much in advance,
before the hair follicle develops signs of severe damage. Cyclosporine
is effective either by interfering with the acute inflammatory processes
or by limiting the progression of the disease into a chronic phase.
All these treatment options have their individual side effects.
It is therefore imperative that the side effects be weighed against
the benefits on case-to-case basis and close monitoring of the patient
is recommended throughout the course of therapy.
In conclusion, based on the overlapping clinical and pathologic
findings, the so-called Graham Little syndrome (or the Graham-Little-Piccardi-Lasseur
syndrome) has been variously classified by different authors as
a form of lichen planopilaris, a variant of Keratosis pilaris atrophicans,
or a distinct classifiable entity with its own distinctive features.
Graham Little Piccardi Lassueur syndrome references
- Vega Gutierrez J, Miranda-Romero A, Perez Milan
F, Martinez Garcia G. Related Articles, Links Graham Little-Piccardi-Lassueur
syndrome associated with androgen insensitivity syndrome (testicular
feminization). J Eur Acad Dermatol Venereol. 2004 Jul;18(4):463-6.
PMID: 15196163
- Viglizzo G, Verrini A, Rongioletti F.
Familial Lassueur-Graham-Little-Piccardi syndrome. Dermatology.
2004;208(2):142-4.
PMID: 15057005
- Ghislain PD, Van Eeckhout P, Ghislain E.
Lassueur-Graham Little-Piccardi syndrome: a 20-year follow-up.
Dermatology. 2003;206(4):391-2.
PMID: 12771495
- Bianchi L, Paro Vidolin A, Piemonte P, Carboni I, Chimenti S.
Graham Little-Piccardi-Lassueur syndrome:
effective treatment with cyclosporin A. Clin Exp Dermatol. 2001
Sep;26(6):518-20.
PMID: 11678880
- Bardazzi F, Landi C, Orlandi C, Neri I, Varotti C. Graham Little-Piccardi-Lasseur
syndrome following HBV vaccination. Acta Derm Venereol. 1999 Jan;79(1):93.
PMID: 10086877
- Keining E, Rathjens B. [Attempt
at delimitation of the Graham-Little syndrome.] Dermatol Wochenschr.
1955;132(38):1016-23.
PMID: 13261637
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