Pediatric Dermatology
Lichen Planus: An Update and Review
Lichen planus (LP) is a papulosquamous eruption of the skin, scalp, nails, and mucous membranes. Although LP is more common in adults, it has...
Case Report
A 25-year-old woman with a 5-month history of severe lichen planus (LP) on the arms, legs, and trunk presented to the emergency department with generalized blisters and erythema over the entire body, including the face and soles, of 2 days’ duration. She was evaluated for the LP 1 week prior in a referral dermatology clinic, and in addition to topical corticosteroids, she received 1 injection of 40 mg intramuscular triamcinolone acetonide. Hours following the injection she developed nausea, vomiting, and fever. The patient reported that her last menstrual period was 3 weeks prior to the current presentation.
Physical examination revealed numerous lichenified, flat-topped, pink-violaceous, hyperpigmented, scaly papules and plaques (Figure 1), as well as tense, yellow, fluid-filled vesicles and bullae of various sizes on the neck, arms (Figure 2), legs, trunk, and dorsal aspect of the feet. The vesicles occurred on both normal skin and the lichenified plaques with a negative Nikolsky sign. There also were urticarial erythematous papules and plaques on the arms, trunk, neck, and face, some of which had vesicles or a violaceous dusky central hue (Figure 3). Vesicles were noted within both nostrils (nasal mucosa), and there were extremely tender erythematous patches and thick sheets of scales on the soles.
Figure 1. Pink, flat-topped, lichenoid papules and plaques on the right hand and arm.
Figure 2. Tense vesicles and bullae occurred on both normal skin and lichenoid papules and plaques on the right arm.
Figure 3. Urticarial erythematous papules and plaques over the patient’s face, some of which had vesicles or a violaceous dusky central hue.
An elevated β human chorionic gonadotropin level and transvaginal ultrasonography confirmed an intrauterine pregnancy of 12 weeks’ gestation despite the patient’s report of the last menstrual period.
Histologic examination of a vesicle on the right arm revealed hyperkeratosis with hypergranulosis, vacuolar alteration of the basal layer with a paucicellular subepidermal vesicle, and melanophages in the superficial dermis consistent with vesicular LP (Figure 4). Histologic examination of an erythematous edematous plaque on the right upper leg revealed edema in the upper dermis with a perivascular and interstitial lymphocytic infiltrate with eosinophils. A third biopsy of a lichenoid flat-topped papule on the left arm revealed a mild bandlike infiltrate of lymphocytes and scattered eosinophils, eosinophilic colloid bodies and edema in the papillary dermis, and subepidermal vesicles and vacuolar alteration of the basal layer consistent with a vesicular lichenoid dermatitis (Figure 5). Direct immunofluorescence (DIF) of perilesional skin showed linear deposition of C3 and IgM along the basement membrane zone (BMZ) in addition to a shaggy pattern with cytoid bodies (Figure 6). There also was a faint linear deposit of IgA along the BMZ with cytoid bodies but negative for IgG. These results were interpreted as consistent with LP pemphigoides (LPP). Neither an enzyme-linked immunosorbent assay nor an immunoblot analysis was performed.
Figure 4. Histologic examination of a vesicle on the right arm revealed hyperkeratosis with hypergranulosis, vacuolar alteration of the basal layer with a paucicellular subepidermal vesicle, and melanophages in the superficial dermis consistent with vesicular lichen planus (H&E, original magnification ×20).
Figure 5. Histologic examination of a lichenoid flat-topped papule on the left arm revealed scattered eosinophils, hyaline colloid bodies, papillary dermal edema, and mild vacuolar alteration of the basal layer (H&E, original magnification ×40).
Figure 6. Direct immunofluorescence of perilesional skin showed linear deposition of IgM along the basement membrane zone and a shaggy pattern with cytoid bodies (original magnification ×20).
Because the patient was pregnant and had failed to respond to topical and intramuscular corticosteroids, she was started on intravenous methylprednisolone in the emergency department until new lesions stopped appearing. She was then discharged home on oral prednisone 50 mg (0.5 mg/kg/d), with close observation by her obstetrician. She also used clobetasol propionate ointment 0.05% for more severe lesions and triamcinolone acetonide cream 0.1% for less severe lesions until lesions resolved.
During treatment, the patient developed cellulitis on the leg that presented as pustules and erythema at a site of an eroded bulla, inframammary and axillary cutaneous candidiasis, and hyperglycemia at 19 weeks’ gestation. The cutaneous infections resolved with oral clindamycin 300 mg 3 times daily for 10 days. Topical mupirocin was used to treat the cellulitis and a mixture of zinc oxide, econazole cream, and desonide cream twice daily treated the candidiasis. Her obstetrician managed the hyperglycemia.
The bullous lesions and LP completely resolved after 2 months of treatment with oral prednisone 50 mg daily. The patient tolerated a corticosteroid taper (dose decreased by 5 mg every 2 weeks) until arriving at 10 mg, which was then decreased to 7.5 mg until delivery. A cesarean delivery was performed due to a large-for-gestational-age fetus, and an internist was consulted for the necessary precautions to increase the steroid dose during delivery due to the stress of the surgery and the risk for a hypothalamic crisis. There were no peripartum complications, and the baby was born without cutaneous lesions and remains healthy 1 year later. The patient remained disease free over 2 months postpartum, until new LP lesions developed without vesicles or bullae, which were then controlled with topical therapy. She was subsequently lost to follow-up.
