Telangiectatic Palmoplantar Keratoderma in Systemic and Subacute Lupus Erythematosus

Telangiectatic Palmoplantar Keratoderma in Systemic and Subacute Lupus Erythematosus : to a Form of Lichen Planus-like Lupus Erythematosus ?

Palmoplantar keratoderma (PPK) associated to livid telangiectatic erythema during systemic lupus erythematosus (SLE) and subacute cutaneous lupus erythematosus (SCLE) is a rare phenomenon seldom reported in literature. The investigators hypothesize that clinic-immunologic assessment and detailed investigation of cutaneous biopsy specimen of PPK and erythema of patients suffering from SLE and SCLE could lead to determine more precisely nosological settings of this injury. Report the different therapeutics with efficacy assessment could be helpful to highlight useful treatment for these patients.

Study Overview

• Status: Unknown
• Conditions: Systemic Lupus Erythematosus; Lichen Planus

Detailed Description

Context: Palmoplantar keratoderma (PPK) associated to livid telangiectatic erythema during systemic lupus erythematosus (SLE) is a rare phenomenon seldom reported in literature. To our knowledge no case has been described in subacute cutaneous lupus erythematosus (SCLE) so far. Pathogeny and etiologic origin are not clear: it could be no-specific cutaneous manifestation of SLE, verrucous chilblain lupus, lichen planus, discoid lupus erythematosus lesions, overlap syndrome or coexistence of both diseases.

Objective:

1. Determine clinical immunological and histopathological features of patients with PPK and livid telangiectatic erythema-associated suffering from SLE and SCLE
2. Describe the different therapeutics used and efficacy assessment Methods: Multicentric retrospective descriptive study reporting 14 patients with SLE or SCLE suffering from an acral livid erythematosus keratoderma, with clinical data regarding lupus erythematosus and acral keratoderma, immunological blood samples findings and histopathological results of keratoderma and livid erythema biopsies with direct immunofluorescence if performed. The treatments set down in order to heal are noted followed by efficacy assessment: total failure, partial remission, complete remission.

• Study Type: Observational
• Enrollment (Actual): 14

Contacts and Locations

Study Locations

• France
  • Montpellier, France, 34295
    • UH Montpellier

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

patients suffering from SLE or SCLE with palmar and or plantar keratoderma with livid telangiectatic erythema

Description

Inclusion criteria:

• > 18 years old
• suffering from SLE or SCLE according to 2019 ACR/EULAR criteria
• With palmar and or plantar keratoderma with livid telangiectatic erythema

Exclusion criteria:

• < 18 years old
• suffering from lupus lesions without SLE or SCLE criteria
• without palmar and or plantar keratoderma with livid telangiectatic erythema

Study Plan

How is the study designed?

Design Details

• Observational Models: Case-Only
• Time Perspectives: Retrospective

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Determine clinic immunologic features	Determine clinic immunologic features of patients with PPK and livid telangiectatic erythema-associated suffering from SLE and SCLE.	1 day
Determine clinic histopathologic features	Epidemiologic clinic immunologic histopathologic data	1 day

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Different therapeutics used	Describe the different therapeutics used. Report of the drug therapy used to heal acral keratoderma for each patient	1 day
Different therapeutics efficacy assessment	Efficacy assessment of the treatments: total failure, partial remission, complete remission	1 day

Collaborators and Investigators

• Sponsor: University Hospital, Montpellier
• Investigators: Study Director: Didier BESSIS, PhD, University Hospitals of Montpellier

Study record dates

Study Major Dates

• Study Start (Actual): April 1, 2020
• Primary Completion (Anticipated): November 30, 2020
• Study Completion (Anticipated): December 30, 2020

Study Registration Dates

• First Submitted: April 16, 2020
• First Submitted That Met QC Criteria: April 19, 2020
• First Posted (Actual): April 21, 2020

Study Record Updates

• Last Update Posted (Actual): April 21, 2020
• Last Update Submitted That Met QC Criteria: April 19, 2020
• Last Verified: March 1, 2020

More Information

Terms related to this study

• Keywords: Palmoplantar keratoderma with livid telangiectatic erythema; Systemic lupus; Subacute lupus erythematosus
• Additional Relevant MeSH Terms: Skin Diseases; Immune System Diseases; Autoimmune Diseases; Genetic Diseases, Inborn; Connective Tissue Diseases; Skin Diseases, Genetic; Skin Diseases, Papulosquamous; Lichenoid Eruptions; Keratosis; Lupus Erythematosus, Systemic; Lichen Planus; Keratoderma, Palmoplantar
• Other Study ID Numbers: RECHMPL20_0206

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: Undecided
• IPD Plan Description: NC

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No