Lupus erythematosus tumidus L93.2

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

LET; lupus erythematosus tumidus; Lupus vulgaris tumidus et hypertrophicus; TLE

History
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Hoffmann, 1909; Gougerot and Burnier, 1930

Definition
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Non-scarring variant of cutaneous lupus erythematosus characterized by indurated red plaques and nodules, without a significant epidermal component such as scaling or follicular hyperkeratosis. There are smooth transitions to lupus erythematosus profundus. Clinically prominent is a high light sensitivity.

Occurrence/Epidemiology
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Occurs in about 15% of cases of cutaneous lupus erythematosus.

Manifestation
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Mainly occurring in young adulthood. Age peak: 30th - 40th LJ. Less frequently occurring in children. No sex preference.

Localization
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Preferably on the face; also neck, décolleté, arms, shoulders are often affected.

Clinical features
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At light-exposed sites, localized or also disseminated, chronically stationary, 0.5-5.0 cm large, red, mostly non-hypersensitive, sharply defined succulent papules and plaques with smooth, shiny, non-scalying surface, without follicular hyperkeratoses, which can heal without scarring after months of progression. Deterioration after exposure to the sun.

Confluence may lead to the formation of annular or gyrated marginal plaques (differentiation from the annular type of subacute cutaneous lupus erythematosus).

Scarred healing is not observed (no follicular atrophy, no de- or hyperpigmentation).

Laboratory
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In about 10-20% of cases (Rodriguez -Caruncho et al. 2015) positive (mostly low-titre max. 1:320) ANA is found, in 5% anti-Ro/SSA and La/SSB. Anti-Cardiolipin AK in 10% pos. CRP in about 30% of patients increased, C3 in about 30% decreased.

Histology
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In the middle and deep dermis, and occasionally also in the aggressive subcutis, there are spot-shaped, sometimes nodular, perivascular or periadnexial, predominantly monomorphic, lymphoid-cell infiltrates, whereby small round lymphocytes with homogeneous chromatin-tight nuclei predominate. Few plasma cells. Germinal centres may occur, but are rather rare. No significant or only very slight epidermotropy (vacuolar degeneration of the basal cell layer is always absent!) or adnexotropy. Significant subepidermal edema and focal mucin deposits.

Direct Immunofluorescence
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At the dermo-epidermal junction zone and also around the follicles in most cases no specific fluorescence phenomena are detectable! Also in the UV-provoked lesions no specific fluorescence phenomena are visible.

Differential diagnosis
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Clinically as well as histopathologically a differentiation to polymorphic light dermatosis (PLD), lymphocytic infiltration, pseudolymphomas of the skin, granuloma anulare and REM-syndrome is necessary.

Therapy
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According to the lupus erythematodes chronicus discoides.

The treatment with topical glucocorticoids as well as the application of sun protection preparations with a high light protection factor leads in individual cases to a complete regression of the symptoms.

Alternative: The good response to a local therapy with tacrolimus has been proven in pilot studies.

General therapy
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Avoid direct exposure to the sun.

Internal therapy
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Therapy with chloroquine (3.5-4.0 mg/kg bw/day p.o.) or hydroxychloroquine (6.0-6.5 mg/kg bw/day p.o.) is very effective. Among them high rates of improvement (> 90%). Cave! Smoking significantly reduces the response rate of antimalarials.

Progression/forecast
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Cheap. Systemic involvement or transition to systemic lupus erythematosus (SLE) has not been observed.

Tables
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lupus erythematosus tumidus

REM syndrome

Lymphocytic infiltration of the skin

Lupus erythematosus, subacute cutaneous

Histology

Mucin deposits

usually not available; varying degrees of intensity

abundantly available

available

available

Vacuolar degeneration of the basal cell line

not available

mostly non-existent or very little pronounced

not available

available

Direct Immunofluorescence

negative

Mostly negative, rarely IgM, IgA, C3-precipitation at the dermatoepidermal junction zone.

negative

Negative in 40-50% of cases; in affected skin detection of IgG, IgM, IgA, C1 and C3 (= positive lupus band test), unaffected skin negative.

Manifestation

Mainly in young adulthood, age peak 30 - 40 LJ; less often in children.

Particularly in young adulthood, ratio of women to men 2:1.

Especially in male adults < 50 years.

Mainly middle-aged adults, more often among women.

Localization

Areas exposed to light: face preferred; also neck, décolleté, arms, shoulders.

Mainly middle of chest and/or back, more rarely also upper abdomen, elbows and face.

Areas exposed to light: Mainly face (forehead, cheeks), neck, throat.

Areas exposed to light: face, with the centre of the face rather recessed, upper thorax, arms.

Clinic

Sharply defined, disseminated, 5 mm to 3-5 cm large, red, usually not (!) hypersensitive papules and plaques with smooth, shiny, non-scalying surface, which heal without scarring. Pronounced photosensitivity (anamnestically in about 50% of cases, after photo provocation in > 70% of cases)

Reticular to planar, sharply defined, irregularly configured, light red, rarely brownish, possibly slightly urticarial erythema and papules. Occasional itching. About 30% of patients exacerbate after sun exposure.

Mostly several, often symmetrically localized, initially small papules, sharply defined, reddish or brownish-red, pad-like raised, coarse, occasionally circulatory infiltrates without epidermal involvement. No mutilations.

Sharply defined, coin-sized, scaly, infiltrated erythema. Centrally firmly adherent scales with pointed conical horny cones located in the follicle openings on the underside (wallpaper nail phenomenon, drawing nail phenomenon). Centrally beginning, atrophic regression with atrophy of the horn support and the follicle ostia, depigmentation or hyperpigmentation, telangiectasia, rarely also mutilation. Minor visceral infestation is possible, including polyarthritis, serositis, rarely renal or cerebrovascular diseases.


Test site

Shoulder and upper back

Test Fields

5 x 8 cm

Radiation sources

UV-A: Metal halide lamps (340-400 nm)

UV-B: Fluorescent lamp (Philips TL 12, 285-350 nm)

Radiation doses

UV-A: 3-4 x 60-100 J/cm2

UV-B: 3-4 x 1.5 times MED

Reading

24, 28, 72 hours and 1, 2, 3 weeks after radiation

Note(s)
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One of the main criteria for lupus erythematosus tumidus is the pronounced photosensitivity, which exists anamnestically in 50% of cases and is detectable in over 70% of patients with a photoprovocation test. Photoprovocation by UVA and/or UVB.

"tumidus" means puffy and denotes voluminous foci of the cutaneous LE.

Literature
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  1. Alexiades-Armenakas MR et al (2003) Tumid lupus erythematosus: criteria for classification with immunohistochemical analysis. Arthritis rheum. 49: 494-500
  2. Böckle BC et al (2015) Smoking is highly associated with discoid lupus erythematosus and lupus erythematosus tumidus: analysis of 405 patients. Lupus 24:669-674
  3. Dekle CL et al (1999) Lupus tumidus. J Am Acad Dermatol 41: 250-253
  4. Gougerot H, Burnier R (1930) Lupus érythémateux 'tumidus Bull Soc Fr Dermatol Syph 37: 1291-1292
  5. Hinz T et al (2013) Lupus tumidus following the lines of Blaschko. Int J Dermatol 52:1615-1617
  6. Hoffmann E (1909) Demonstrations: Isolated lupus erythematodes tumidus of the facial skin. The period 16: 159-160
  7. Kreuter A et al (2009) Lupus erythematosus tumidus. Arch Dermatol 145: 244-248
  8. Kuhn A et al (2003) Histopathologic findings in lupus erythematosus tumidus: review of 80 patients. J Am Acad Dermatol 48: 901-908
  9. Kuhn A et al (2002) Characterization of the inflammatory infiltrate and expression of endothelial cell adhesion molecules in lupus erythematosus tumidus. Arch Dermatol Res 294: 6-13
  10. Kuhn A et al (2002) Upregulation of epidermal surface molecule expression in primary and ultraviolet-induced lesions of lupus erythematosus tumidus. Br J Dermatol 146: 801-809
  11. Kuhn A et al (2000) Lupus erythematosus tumidus--a neglected subset of cutaneous lupus erythematosus: report of 40 cases. Arch Dermatol 136: 1033-1041
  12. Rémy-Leroux V et al (2008) Comparison of histopathologic-clinical characteristics of Jessner's lymphocytic infiltration of the skin and lupus erythematosus tumidus: Multicenter study of 46 cases. J Am Acad Dermatol 58: 217-223
  13. Rodriguez-Caruncho C et al (2015) Lupus erythematosus tumidus: a clinical and histological study of 25 cases. Lupus 24:751-755
  14. Sonntag M et al (2003) Lupus erythematosus tumidus in childhood. Report of 3 patients. Dermatology 207: 188-192
  15. Vieira V et al (2006) Lupus erythematosus tumidus: a series of 26 cases. Int J Dermatol 45: 512-517

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 29.10.2020