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Dermatology > PEDIATRIC DISEASES
Lichen Striatus
Article Last Updated: Sep 12, 2006
AUTHOR AND EDITOR INFORMATION
Section 1 of 10
Author: June Kim, MD, Staff Physician, Department of Dermatology, University of New Mexico
June Kim is a member of the following medical societies: American Medical Association and American Medical Women's Association
Coauthor(s):
Wingfield Rehmus, MD, MPH, Co-Director of Clinical Trials, Clinical Instructor, Department of Dermatology, Stanford University Medical Center;
Nelly Rubeiz, MD, Consulting Staff, Department of Dermatology, American University of Beirut Medical Center; Associate Professor, Department of Dermatology, American University of Beirut, Lebanon;
Amal Mehanna, MD, Staff Physician, Department of Dermatology, American University of Beirut, Lebanon
Editors: Daniel J Hogan, MD, Director of Bay Pines Dermatology Residency Program, Bay Pines Veterans Affairs Healthcare System; Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA; Paul Krusinski, MD, Director of Dermatology, Professor, Department of Internal Medicine, Fletcher Allen Health Care, University of Vermont; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Author and Editor Disclosure
Synonyms and related keywords:
LS, linear lichenoid dermatosis, linear neurodermatitis, blaschkitis, Blaschko linear acquired inflammatory skin eruption, BLAISE
Background
Lichen striatus is a rare, benign, self-limited linear dermatosis of unknown origin that predominantly affects children. Lichen striatus is clinically diagnosed on the basis of its appearance and characteristic developmental pattern following the lines of Blaschko.
Pathophysiology
The skin is the primary organ system affected. However, the nails may also be involved.
Race
No racial predilection is recognized.
Sex
No consensus exists on sex predilection. Some studies show a 2- to 3-fold increased incidence in girls compared with boys, whereas others show an equal sex distribution.
Age
Lichen striatus is primarily a disease of young children. More than 50% of all cases occur in children aged 5-15 years. Other reports dispute this age range and claim that the median age of onset is 3 years. Although lichen striatus is rare in both infants and adults, the disease can occur in persons of any age.
History
Lichen striatus often appears as a sudden eruption of small papules on an extremity. The papules are usually asymptomatic, reaching maximum involvement within several days to weeks. When patients are symptomatic, the most common complaint was pruritus. Lichen striatus is self-limited, but it may resolve with postinflammatory hyper or hypopigmentation.
Physical
- Lichen striatus appears as a continuous or interrupted, linear band consisting of small (1- to 3-mm) pink, tan, or skin-colored lichenoid papules.
- The papules may be smooth, scaly, or flat topped. Occasionally, a vesicular component is present.
- The band may range from a few millimeters to 1-2 cm wide and extends from a few centimeters to the full length an extremity. The lesions are usually unilateral and single on an extremity along the Blaschko line. In rare cases, they may be bilateral or occur in multiple parallel bands.
- The lesions are most commonly located on a proximal extremity and less commonly on the trunk, head, neck, or buttock.
- In darkly pigmented individuals, eruptions may appear as a bandlike area of hypopigmentation.
- Nail involvement is uncommon with fewer than 30 reported cases worldwide.
- Nail lesions may occur before, after, or concurrently with the skin lesions. They may also be the only area of involvement.
- Often, only the medial or lateral portions are involved, and involvement is almost always restricted to one single nail.
- Nail changes may include longitudinal ridging, splitting, onycholysis, nail loss, hyperkeratosis of the nail bed, thinning or thickening of the nail plate, nail pitting, onychodystrophy, punctuate and striate leukonychia, and overcurvature of the nail plate.
Causes
The etiology of lichen striatus is unknown.
- Many etiologic or predisposing factors are suggested.
- The most commonly accepted hypothesis is the combination of genetic predisposition with environmental stimuli.
- Atopy may be a predisposing factor. One group reported that 85% of patients with lichen striatus have a family history of atopic dermatitis, asthma, or allergic rhinitis. However, another report disputes this claim, stating that the incidence of atopy is no greater than that of the general population.
- An autoimmune response may also be involved. A case of lichen striatus has been reported during pregnancy, and it has been postulated that the pregnancy may have triggered an autoimmune response leading to the appearance of the eruption. Some reports simply suggest that lichen striatus is an inflammatory skin disease mediated by T cells.
- An environmental (infectious or trauma) etiology has also been suggested. Familial cases, outbreaks among unrelated children in a shared living environment, and a possible seasonal variation suggest an environmental agent, such as a virus. However, results of viral testing have not conclusively proven this association. In addition, familial episodes of lichen striatus are not always simultaneous, signifying a possible genetic predisposition as a second explanation. Lichen striatus has been reported to occur shortly following trauma in 2 cases—one following BCG vaccination and another following a prick from a pineapple leaf.
- Lesions of lichen striatus follow the lines of Blaschko.
- Blaschko lines are thought to be embryologic in origin. They are believed to be the result of the segmental growth of clones of cutaneous cells or the mutation-induced mosaicism of cutaneous cells.
- In lichen striatus, an acquired event (eg, viral infection) may allow an aberrant clone of cutaneous cells to express a new antigen, resulting in the phenotypic skin changes.
Lichen Nitidus
Lichen Planus
Lichen Simplex Chronicus
Porokeratosis
Psoriasis, Plaque
Warts, Nongenital
Other Problems to be Considered
Nevus unius lateris
Tinea corporis
Inflammatory linear verrucous epidermal nevus
Linear fixed drug eruption
Linear Darier disease
Linear graft versus host disease
Procedures
- Skin biopsy can be performed to confirm the diagnosis, but it is rarely necessary.
- In ambiguous cases, direct immunofluorescence with staining for Civatte bodies has been proposed to distinguish between lichen planus and lichen striatus.
- Stains for immunoglobulin M, immunoglobulin G, and complement C3 are positive in lichen planus and negative in lichen striatus.
Histologic Findings
The histopathologic results vary depending on the stage of evolution. Often, a polymorphic epidermal reaction pattern with variable spongiotic and lichenoid changes is seen. However, unlike lichen planus, lichen striatus may result in a dense, usually perivascular, lymphohistiocytic infiltrate that extends deep into the dermis and that surrounds the eccrine sweat glands and ducts. Granulomatous inflammation may also be present.
Medical Care
- Because lichen striatus is a self-limited disorder and because the lesions spontaneously regress within 3 -12 months, no treatment is needed. The patient and family should be reassured.
- Lichen striatus of the nail may indicate a protracted course. Nail involvement resolves spontaneously without deformity within 30 months.
The goals of pharmacotherapy are to reduce morbidity and prevent complications.
Drug Category: Corticosteroids
Corticosteroids have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.
Topical and intralesional steroids do not necessarily hasten resolution; however, emollients and topical steroids may be used to treat associated dryness and pruritus, if present.
| Drug Name | Triamcinolone (Aristocort, Kenalog) |
| Description | For treatment of inflammatory dermatoses responsive to steroids. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability. Affects production of lymphokines and has inhibitory effect on Langerhans cells. |
| Adult Dose | Apply thin film bid until response observed |
| Pediatric Dose | Administer as in adults |
| Contraindications | Documented hypersensitivity, paronychia, cellulitis, impetigo, angular cheilitis, erythrasma, erysipelas, rosacea, perioral dermatitis, acne, skin with decreased circulation |
| Interactions | None reported |
| Pregnancy | C - Safety for use during pregnancy has not been established.
|
| Precautions | Can cause atrophy of groin, face, and axillae; if infection develops and is not responsive to antibiotics, discontinue until infection controlled |
| Drug Name | Clobetasol (Temovate) |
| Description | Class I superpotent topical steroid. Suppresses mitosis and increases synthesis of proteins that decrease inflammation and cause vasoconstriction. |
| Adult Dose | Apply bid for up to 2 wk; not to exceed 50 g/wk |
| Pediatric Dose | <12 years: Not recommended >12 years: Administer as in adults |
| Contraindications | Documented hypersensitivity; viral or fungal skin infections |
| Interactions | None reported |
| Pregnancy | C - Safety for use during pregnancy has not been established.
|
| Precautions | Can cause significant skin atrophy; may suppress adrenal function in prolonged therapy |
Drug Category: Immunosuppressants
Tacrolimus has been successful in treating persistent lesions on the face and extremities.
| Drug Name | Tacrolimus, topical 0.03%, 0.1% (Protopic) |
| Description | Immunomodulator; suppresses cytokine release from T lymphocytes. |
| Adult Dose | Apply to affected skin bid |
| Pediatric Dose | <2 years: Not established 2-15 years: Apply 0.03% to affected skin bid |
| Contraindications | Documented hypersensitivity |
| Interactions | None reported |
| Pregnancy | C - Safety for use during pregnancy has not been established.
|
| Precautions | For external use only; do not use with occlusive dressings; patients may have burning/stinging during first few days of application; skin may become photosensitive; patients encouraged to minimize exposure to natural and artificial sunlight Long-term safety of topical calcineurin inhibitors not established (>1 y of noncontinuous use); although a causal relationship has not been established, rare cases of malignancy (eg, skin malignancy, lymphoma) have been reported in patients treated with topical calcineurin inhibitors, including tacrolimus; therefore, avoid continuous long-term use of topical calcineurin inhibitors, including tacrolimus, in all age groups |
Complications
- Postinflammatory hyperpigmentation and hypopigmentation may last for several months to years after the disease resolves.
Prognosis
- The prognosis of patients with lichen striatus is excellent. Recovery is complete.
- Lesions usually regress spontaneously within 1 year, with a range of 4 weeks to 3 years.
- Relapses may occur, but these are uncommon.
| Media file 1:
Extensive unilateral lichen striatus that affects both the upper and lower extremities. Grouped keratotic lichenoid papules form plaques over the leg. |
 | View Full Size Image | |
Media type: Photo
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Lichen Striatus excerpt Article Last Updated: Sep 12, 2006
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