Clinical Case Detail
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CPC Case IV
Provided By
Dr. Joel Laudenbach - 2011-05-18
Clinical History
A 36 year old female presents with a "cyclic problem of severe peeling, bleeding, sloughing and crusting of the lips every 4 - 6 weeks." The lips decrease in swelling for 1.5 weeks, improve and then it starts all over again. This first started approximately 2.5 years ago, and she has since tried various topicals and had inconclusive biopsies. Dermatologic patch testing was positive for nickel, rubber and cobalt reactions. Observing a nickel-free lifestyle has not been helpful for this patient.
PMHx: Atopy, ocular rosacea, chronic blepharitis, urticaria/flushing with stress/anxiety, and genital HSV (for 10 yrs.). She used to take suppressive HSV therapy (Valtrex 500mg BID), but only takes this regimen when she feels vaginal lesions starting.
Meds: FML (fluorometholone) ophthalmic ointment, doxycycline 100mg QD, glucosamine/chondroitin sulfate and evening Primrose.
ALL: NKDA Allergic to nickel, rubber & cobalt (per patch testing)
PSurgHx: s/p tonsillectomy
SocHx: operating room nurse
ROS: She has had
Differential Diagnosis
Serology & Testing
ANC = 1174 [1500 - 7800]
Ig A, G, M - normal
Angioedema panel, hereditary, comprehensive (normal) - C1 INH, Complement/C4 - all normal
ANA, ACE - normal
Previous biopsies: hyperkeratosis
Differential Diagnosis:
1. Persistent erythema multiforme - HSV associated
2. R/O progesterone related EM
3. R/O CT disease
4. R/O vesiculobullous disease
5. T/C prick testing
Pain / Management
Predinsone 50mg QD X 2 weeks
Valtrex 500mg BID
Antihistamines
Topical corticosteroids
Refer to GYN/Dermatology
Biopsy if non-responsive
Diagnosis
Biopsies - 12-11-2009
Routine -H & E
Direct immunofluorescence
Histopathology - Dr. Faizan Alawi & Dr. Hope Wettan
Biopsy Reports - 12-2009
H & E: Lichenoid mucositis, see note
Edematous fibrous stroma, well vascularized, superifcial & deep, dense, perivascular infiltrate of lymphocytes & plasma cells; HSV Ag is negative
Note: Cumulative findings are suggestive of erythema multiforme. "...could also be associated with lichenoid drug reaction or a contact immune reaction."
Negative direct immunofluorescence
Follow-up
Clinical/working diagnosis: Persistent erythema multiforme, HSV associated
Janurary 2010:
Prednisone 50mg QD X 2 weeks
Valtrex 500mg BID
Clobetasol ointment 0.05% applied 2 - 5 X/week
Images from Sept. 2010
Clinical Features
Swollen, cracked, bleeding & crusted lips
Intraoral lesions on non-keratinized mucosa, most pronounced anteriorly
Eyes - lacrimation, photophobia; genital &/or nasal lesions
Treatment & Prognosis
Discussion: EM
- Rare, acute, inflammatory disorder, hypersensitivity
- Self-limiting, episodic, recurrent/persistent
- Young adults, 20 -40 yrs. (20% in children)
- Skin, mucous membranes or both
- EM minor: One mucosal site, possible symmetrical target skin lesions (<10% skin surface)
- EM Major: 2 or more mucous membranes, variable skin involvement
- Variants/other: Stevens-Johnson syndrome & toxic epidermal necrolysis (TEN)
- Reaction to antigens induced by exposure to drugs, microbes, viruses
- HSV: 15 - 70% of recurring cases
- Infection within 2 weeks of EM onset
- Multiple drugs: NSAIDs, antibiotics, cephalosporins, progesterone, protease inhibitors, anticonvulsants
Discussion