Patient |
R.D., 51-year-old office manager |
Duration |
6 months |
Distribution |
Trunk, extremities, face and scalp |
| History |
RD is a 51-year-old office manager who presented in April
2004 with a four month history of pruritic lesions on back.
This began a few months after starting hydrochlorthiazide.
There was a history of “lichen planus” 20 - 30
years back after the birth of a child. This time, she was
preparing for a daughter's wedding and was under some stress.
Since April, she has developed more wide-spread lesions mostly
on the extremities and scarring alopecia. Potent topical corticosteroids
were not of value, neither was tacrolimus ointment. Plaquenil
was started in mid May at a dose of 200 mg. per day, but shortly
after it was increased to 200 mg b.i.d. the patient developed
a pruritic eruption different from her other lesions. This
cleared after stopping the Plaquenil. Other than mild myalgias,
she has no constitutional symptoms. She has just restarted
the Plaquenil.
|
| Physical Examination |
Initially, the only cutaneous findings were hyperkeratotic,
flesh-colored to slightly hyperpigmented papules located on
the mid-upper back. By mid May she had developed similar lesions:
discrete and confluent hyperpigmented folicular papules cheeks
and forehead and some flat-topped papules on both legs. At
around this time, she started to develop an inflammatory alopecia.
The scalp showed mild erythema and some scarring.
This has progressed and at present she has impressive diffuse
alopecia. After Plaquenil was increased to 400 mg/day she
developed erythematous papules and small plaques on the hips
and upper chest. These disappeared two weeks after stopping
Plaquenil. The eruption on the back and face is slowly improving,
but the alopecia is progressing
|
|
Images |
|
| Laboratory Data |
CBC normal, Blood Chemistries normal, Urinalysis normal.
ESR 25
ANA +ve 1:640 speckled
RNP Antibody 157.6 U (nl <20 U)
Anti DS-DNA (-ve), Anti-Histone (-ve). All other ANAs negative
including Ro and La
C-4 borderline low. G6PD normal.
|
|
Histopathology |
Interface dermatitis with pigment incontinence, superficial
and mid perivascular and periappendageal lymphocytic infiltrate,
with marked dermal mucin deposition and extravasated erythrocytes
|
| Diagnosis |
Follicular Lupus Erythematosus
|
| Reasons Presented |
For diagnostic and therapeutic suggestions. Follicular L.E.
is unusual. Please comment on this entity. The patient has
not responded well to therapy. Your suggestions regarding
treatment will also be appreciated.
|
| Questions and Discussions |
|
| References |
Reference:
1. Morihara K, Kishimoto S, Shibagaki R, Takenaka H, Yasuno H.
Follicular lupus erythematosus: a new cutaneous manifestation
of systemic lupus erythematosus. Br J Dermatol. 2002 Jul;147(1):157-9.
We report the clinical, histopathological and immunological features
of follicular erythema and petechiae in a 30-year-old Japanese
woman with systemic lupus erythematosus (SLE). Histology showed
this eruption to constitute a cutaneous manifestation of SLE.
To our knowledge, this is the first reported case of follicular
erythema and petechiae in association with SLE. Accordingly, we
propose that this rare eruption be termed 'follicular lupus erythematosus'.
|
| Comments from Faculty and Members |
Robert I. Rudolph, M.D., FACP, Clinical Professor
of Dermatology, University of Pennsylvania, Philadelphia. PA,
USA on Oct 18, 2004
Photos and case presentation are excellent. Based on the photos
I'd vote for lichen planopilaris, the Graham-Little syndrome,
or perhaps an LE/LP overlap. Sure doesn't look like "a
pure LE" to me. Good luck with therapy.
Diane Thaler M.D., Madison, WI, USA on Oct
18, 2004
kudos to david for not referring to "race"....
Massone Cesare MD, Department of Dermatology, Medical
University of Graz, Graz, Austria on Oct 19. 2004
Lichen planopilaris would be also my first impression. It could
fit both clinically (follicular keratotic lesions and Brocq's
pseudopelade) and histologically, and it would explain also
the worsening with Plaquenil therapy and the clinical improvement
after stopping Plaquenil. Pruritus is common in lichen. ANA
have been described in LP in 40% of cases of lichen planus using
rat oesophagus has substrate (Carrizosa et al, Exp Dermatol.
1997: 54). thiazides may induce lichenoid drug reactions and
could have been the Koebner in this case. Has the patient also
mucosal lesion? Is she HCV+? Has she variable alopecia of the
axillae and groins? CsA could be a therapeutic option.
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