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Lupus erythematosus lichen planus overlap syndrome introduction
Cicatricial alopecia, also known as scarring alopecia, is hair
loss that occurs in areas where there is scarring or other tissue
damage that destroys the hair follicles. Scarring alopecia can be
divided into two categories, primary and secondary. In primary scarring
alopecia, the hair follicle is the main target of inflammation.
In the case of secondary alopecia, the hair follicle is regarded
as an “innocent bystander” as it is destroyed in a non-specific
manner by skin inflammation. For both the clinician and the pathologist,
identifying the particular forms of primary scarring alopecia and
making a diagnosis can be rather challenging.
With respect to the classification system for primary scarring
alopecia, several methods have been adopted based on different criteria,
to allow for a diagnostic framework to guide dermatologists and
clinicians in pin-pointing and treating the disease. Perhaps the
most accepted classification of these disorders is the classification
as defined by the North American Hair Research Society (NAHRS),
a categorization that is based on the principal inflammatory cell
type observed in scalp biopsies taken from active lesions.
Despite the classification system, very often it is difficult to
define the particular condition under study within this classification
framework. Not all cases of scarring alopecia fit predictably into
the categories defined by the North American Hair Research Society,
and very often there maybe cases where features of two defined forms
of alopecia may overlap. In such cases, it is the job of the pathologist
to distinguish one form of alopecia from another. Often, even histological
features can be non specific and suggestive of more than one clinical
entity. One such condition described and documented is the occurrence
of lupus erythematosus (LE) and lichen planus (LP) together as an
overlapping syndrome (LE/LP). The disease occurs so infrequently
as to limit clinical cases for study.
This overlap syndrome with assorted features of lichen planopilaris
and lupus erythematosus has been described with a report of associated
cicatricial alopecia. The study conducted on a heterogeneous group
of patients who had clinical, histological and immuno-pathological
characteristics of both diseases at the same time raises the question
whether this is an autonomous disorder, or a variant of discoid
lupus erythematosus with features that resemble lichen planopilaris,
or a case lichen planopilaris with concurrent lupus erythematosus.
Lupus erythematosus lichen planus overlap syndrome clinical features
To understand the clinical features of lupus erythematosus (skin
disease in which there are red scaly patches, especially over the
nose and cheeks) - lichen planus overlap syndrome, one must understand
the individualistic features of lichen planus (a primary disorder
of the skin resulting in violaceous, polygonal, flat skin lesions)
as well as lupus erythematosus.
Lichen planus is a disorder of the skin and mucous membranes resulting
in inflammation, itching, and distinctive skin lesions. The exact
cause is unknown, but the disorder is likely to be related to an
allergic or immune reaction.
Lupus erythematosus (LE) is a chronic skin condition of sores with
inflammation and scarring favoring the face, ears, and scalp and
at times on other body areas. These lesions develop as a red, inflamed
patch with a scaling and crusty appearance. The center of the lesions
may appear lighter in color with a darker rim. When lesions occur
in the scalp, permanent scarring and hair loss can occur.
The cicatricial alopecia as seen in the Lupus erythematosus - lichen
planus overlap syndrome is a poorly characterized feature that appears
as erythematous or reddened patches on the scalp succeeded by follicular
plugging. The condition affects peripheral parts such as limbs,
fingers, and ears. Warts or wart like projections or ringed red – violet
color plaques is typical of the condition, often affecting the palms
of the hands and soles of the feet.
Lupus erythematosus lichen planus overlap syndrome pathology
Scalp biopsy as a tool for diagnosis becomes crucial in evaluating
the concerned alopecia. Initial biopsy of the scalp showed scarring
alopecia only, with subsequent biopsies showing histological features
of lupus erythematosus (skin disease in which there are red scaly
patches, especially over the nose and cheeks). Direct immuno-fluorescence,
a technique that uses antibodies linked to a fluorescent dye in
order to study antigens (substances which are capable, under appropriate
conditions, of inducing a specific immune response) in a sample
of tissue, was negative for the typical lupus erythematosus band,
but showed features consistent with lichen planopilaris.
Lupus erythematosus lichen planus overlap syndrome treatment
No defined course of treatment has yet been defined for this overlap
syndrome. Awareness of the existence of this condition is important
for management and prognostic purposes. Adults with Lupus erythematosus
- lichen planus overlap syndrome can eventually develop systemic
lupus erythematosus (SLE) in due course. SLE is an autoimmune disease
in which the individual’s own immune system is directed against
the body's own tissues. The course of SLE is often severe, with
possible renal or neurological ramifications.
At the present moment, clinical trials are required on an urgent
basis to define effective therapy to arrest the progress of such
an overlap syndrome. Continued research into follicle cycling and
genetic abnormalities of hair production will help in revealing
specific etiological factors of this condition, and allow the development
of targeted therapy.
Review of the literature based on lupus erythematosus - lichen
planus overlap syndrome (LE/LP) shows that lupus erythematosus and
lichen planopilaris may occur together in the same individual. As
a direct consequence of a fundamental dearth of critical information
with respect to etiological, clinical and pathological features,
the condition is incompletely defined. Further research, study and
documentation are required to increase our understanding of the
condition as well as for development of a defined course of effective
treatment.
Lupus erythematosus lichen planus overlap syndrome references
- Kim H, Pomeranz MK. Related Articles, Links
Lupus erythematosus and lichen planus overlap syndrome. J Drugs
Dermatol. 2004 May-Jun;3(3):311-2. PMID: 15176168
- Muller FB, Groth W, Mahrle G. [Multiple autoimmune syndrome.
Reynolds-syndrome (acral scleroderma, primary biliary cirrhosis,
Sjogren syndrome) associated
with the lupus erythematosus/lichen planus overlap syndrome]
Hautarzt. 2004 May;55(5):465-70. PMID: 15138654
- Tursen U, Oz O, Ikizoglu G, Kaya TI, Dusmez
D. A case of lichen planus-lupus erythematosus
overlap syndrome with eyelid involvement. Eur J Ophthalmol.
2002
May-Jun;12(3):244-6. PMID: 12113574
- Inaloz HS, Chowdhury MM, Motley RJ.
Lupus erythematosus/lichen planus overlap syndrome with
scarring alopecia. J Eur Acad Dermatol Venereol. 2001 Mar;15(2):171-4.
PMID: 11495530
- Mahler
V,
Hornstein OP, Meyer S, Albrecht HP, Kiesewetter F. [Lupus
erythematosus/lichen ruber planus overlap syndrome. 5 cases in
a patient sample of the Erlangen University Dermatology
Clinic
(1894-1995)] Hautarzt. 1998 Apr;49(4):295-302. PMID: 9606630
- de Jong EM, van
der Vleuten
CJ, van Vlijmen-Willems IM. Differences
in extracellular matrix proteins, epidermal growth and
differentiation
in discoid lupus erythematosus, lichen planus and the
overlap syndrome. Acta Derm Venereol. 1997 Sep;77(5):356-60.
PMID: 9298127
- Stary A, Schwarz T, Duschet P, Gschnait F. [Lichen ruber planus--lupus
erythematosus/overlap syndrome] Z Hautkr. 1987 Mar 1;62(5):381-94.
PMID: 3296507
- Nieboer C.
Lupus erythematosus/lichen planus (LE/LP) overlap syndrome.
J Am Acad Dermatol. 1985 Aug;13(2 Pt 1):297. PMID: 4044953
- Camisa C, Neff
JC, Olsen RG. Use of indirect immunofluorescence
in the lupus erythematosus/lichen planus overlap syndrome:
an additional diagnostic clue. J Am Acad Dermatol. 1984 Dec;11(6):1050-9.
PMID: 6392365
- Ahmed AR,
Schreiber
P, Abramovits W, Ostreicher M, Lowe NJ. Coexistence of lichen
planus and systemic lupus erythematosus. J Am Acad Dermatol. 1982
Oct;7(4):478-83. PMID: 7142458
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