Lupus erythematosus tumidus L93.2

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 01.08.2023

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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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Characterized by indurated, surface-smooth, red papules and plaques, non-scarring (from lat tumidus: puffy) variant of cutaneous lupus erythematosus, without significant epidermal component such as scaling or follicular hyperkeratosis. There are smooth transitions to lupus erythematosus profundus. Clinically prominent is a high photosensitivity.

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

Manifestation
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Especially in young adulthood. Age peak: 30th-40th LJ. Less common in children. No sex preference.

Localization
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Preferably on the face; neck, décolleté, arms, shoulders may also be 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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Positive (mostly low titre max. 1:320) ANA are found in about 10-20% (Rodriguez -Caruncho et al. 2015) of cases, anti-Ro/SSA and anti-La/SSB anti-cardiolipin AK in 5%, positive in 10%. CRP elevated in about 30% of patients, C3 decreased in about 30%.

Histology
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In the middle and deep dermis, occasionally also in the adjacent subcutis, there are patchy, sometimes also nodular, perivascular or periadnexal, predominantly monomorphic, lymphoid cell infiltrates, with small round lymphocytes with homogeneous chromatin-dense nuclei predominating. Few plasma cells. Germinal centers may occur but are the rarity. No significant or only very slight epidermotropy (vacuolar degeneration of the basal cell layer is always absent!) or adnexotropy. Marked subepidermal edema as well as 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 from the following diseases is necessary:

polymorphous light dermatosis (PLD)

lymphocytic infiltration

Pseudolymphoma of the skin

granuloma anulare

REM syndrome

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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Favorable. 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)
Radiation sources	UV-B: Fluorescent lamp (Philips TL 12, 285-350 nm)
Radiation doses	UV-A: 3-4 x 60-100 J/cm2
Radiation doses	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 marked photosensitivity, which is present anamnestically in 50% of cases and detectable in more than 70% of patients with photoprovocation testing. Photoprovocation by UVA and/or UVB.

"Tumidus" means "bloated, distended" and refers to voluminous foci of cutaneous LE.

Literature
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Alexiades-Armenakas MR et al (2003) Tumid lupus erythematosus: criteria for classification with immunohistochemical analysis. Arthritis Rheum. 49: 494-500
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
Dekle CL et al (1999) Lupus tumidus. J Am Acad Dermatol 41: 250-253.
Gougerot H, Burnier R (1930) Lupus érythémateux 'tumidus'. Bull Soc Fr Dermatol Syph 37: 1291-1292.
Hinz T et al (2013) Lupus tumidus following the lines of Blaschko. Int J Dermatol 52:1615-1617
Hoffmann E (1909) Demonstrations: isolated lupus erythematosus tumidus of the facial skin. Derm Zeitschr 16: 159-160
Kreuter A et al (2009) Lupus erythematosus tumidus. Arch Dermatol 145: 244-248.
Kuhn A et al (2003) Histopathologic findings in lupus erythematosus tumidus: review of 80 patients. J Am Acad Dermatol 48: 901-908.
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
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.
Kuhn A et al (2000) Lupus erythematosus tumidus--a neglected subset of cutaneous lupus erythematosus: report of 40 cases. Arch Dermatol 136: 1033-1041
Pona A et al (2022) Lupus erythematosus tumidus Clinical Characteristics and Treatment: A Retrospective Review of 25 Patients. Cutis 109: 330-332.
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.
Rodriguez-Caruncho C et al (2015) Lupus erythematosus tumidus: a clinical and histological study of 25 cases. Lupus 24:751-755
Sonntag M et al (2003) Lupus erythematosus tumidus in childhood. Report of 3 patients. Dermatology 207: 188-192
Vieira V et al (2006) Lupus erythematosus tumidus: a series of 26 cases. Int J Dermatol 45: 512-517