Lichen planus exanthematicus L43.81

Authors: Prof. Dr. med. Peter Altmeyer, Bahareh Ebrahimi

All authors of this article

Last updated on: 24.06.2021

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Synonym(s)

acute lichen planus; Eruptive lichen planus; Eruptive Lichen planus; Exanthematic Lichen planus; Exanthematic Lichen ruber; Generalized lichen planus; Generalized Lichen planus; Generalized Lichen ruber; lichen planus generalisatus; lichen ruber generalisatus

Definition
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Acute, exanthematous form of lichen planus. In contrast to classic lichen planus, the grouped arrangement of papules is often absent in the eruptive exanthematous variant and gives way to a disorderly disseminated distribution pattern. Exanthematous lichen planus tends to form large plaques that resolve only marginally into individual papules. The development of erythroderma is possible (although extremely rare).

Etiopathogenesis
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Unsolved.

Relatively often drug-induced (e.g. gold salts, antimalarials, beta-blockers) or occurring after vaccinations. The term "lichen planus-like drug eruption" or "generalized lichenoid drug eruption" is also used for this form of manifestation. S.u. Drug exanthem lichenoides.

Lichen planus-like exanthema is also observed in chronic graft-versus-host disease (see figure). Furthermore, after percutaneous radiotherapy (Kluger N 2017) and after vaccinations of various types (Rosenblatt AE et al. 2015)

Manifestation
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Children as well as adults.

Localization
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Adults:

  • Trunk, extremities with emphasis on the flexor sides of the wrists and forearms, face remains free.
  • Hands and feet often involved
  • Mucous membrane infestation (30-40%): Penis, oral and genital mucosa
  • Capillitium

Children:

  • extremities (70%)
  • Fuselage
  • Mucous membranes remain free
  • Capillitium remains free
  • no palmo-plantar infestation

Clinical features
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Initially about 0.1-0.2 cm large, raised, plateau-like, smooth, lacquer-like shiny (as if polished), clearly itchy, red papules bordered by the natural skin furrows.

Aggregation of several papules with formation of plaques of varying size. In long-term plaques, the typical "polished smooth surface" can give way to a verrucous, then sometimes whitish surface structure. Histologically this clinical finding is accompanied by severe acanthosis and orthohyperkeratosis. No parakeratotic keratinization.

The diagnostically important surface reflection of the (classical) lichen-planus papules is best seen when light is incident from the side.

Linear arrangements of the efflorescences in scratch or rub marks (see Koebner phenomenon [= isomorphic stimulus effect] below) are characteristic of the exanthematic form.

Important: The itching is answered by the patient with rubbing, not by scratching the efflorescences.

Infestation of the oral mucosa(see also Lichen planus mucosae) is observed in > 50% of patients. Typical are symmetrical, reticular or nummular white plaques, also disseminated, 0.1-0.2 cm large, white papules of the buccal mucosa and/or tongue and/or gingiva (explanation: the whitish discoloration of the papules/plaques corresponds to the "wash skin effect" of the hands. The lichen planus efflorescence keratinizes, horn absorbs water through the mouth moisture, swells and then appears whitish, opaque due to the strong refraction of light. In this way, it separates itself from the non-cornified, healthy oral mucosa).

A special feature is the erosive lichen planus of the oral mucosa.

Genitalia, in particular the glans penis and the vulva, are affected in the form of annular or circulatory, whitish but also red or erosive (therapy-resistant) plaques (see below lichen planus mucosae/ lichen planus vulvae).

Capillitium (see below Lichen planus follicularis capillitii).

Nails: S. lichen planus classic type.

Histology
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Image of the classical Lichen planus.

Therapy
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According to the Lichen planus.

Analysis of the triggering cause in question.

  • Discontinuation of possible medication. Local glucocorticoid therapy in case of severe itching.

If necessary, reduce internal therapy with prednisone equivalent, initially 10-20 mg/day p.o., maintenance dose adapted to the disease.

Azathioprine, sulfasalazine, methotrexate, acitretin were successfully used. These therapy modalities should always be evaluated under the aspect of the self-limited course of the disease.

Experimental: Apremilast, an oral PDE4 inhibitor, which was successfully tested in a smaller monocentric study for the lichen planus exanthematicus.

Progression/forecast
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Rather cheaper than the classic Lichen planus. Frequently self-limated course within a 3-6 month period!

Literature
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  1. de Golian EW et al (2014) Lichenoid drug reaction following influenza vaccination in an HIV-positive patient: a case report and literature review. J Drugs Dermatol 13:873-875
  2. Fessa C et al (2012) Lichen planus-like drug eruptions due to β-blockers: a case report and literature review. At J Clin Dermatol 13:417-421
  3. Noruka EN (2007) Lichen planus in African children: a study of 13 patients. Pediatrist Dermatol 24:495-498
  4. Kluger N (2017) Radiation-associated lichen planus: a case report and literature review.
    Acta Dermatovenerol Alp Pannonica Adriat 26:105-108.
  5. Omidian M et al (2010) Efficacy of sulfasalazine in the treatment of generalized lichen planus: randomized double-blinded clinical trial on 52 patients. J Eur Acad Dermatol Venereol 24:1051-1054
  6. Pedraz J et al (2010) Familial eruptive generalized lichen planus in a pediatric patient. J Dermatol 37:910-912
  7. Rose AE et al (2011) Erythrodermic lichen planus. Dermatol Online J 17:26
  8. Rose petal AE et al. (2015) Cutaneous reactions to vaccinations. Clin Dermatol 33:327-332.
  9. Turan H et al (2009) Methotrexate for the treatment of generalized lichen planus. J Am Acad Dermatol 60:164-166
  10. Vazirnia A et al (2014) Acitretin for the management of generalized cutaneous lichen planus. Dermatol Online J PubMed PMID: 25244164.

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