Rare Pulmonology News

Disease Profile

Lupus erythematosus tumidus

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

-

ICD-10

L93.2

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Intermittent cutaneous lupus; Tumid lupus erythematosus

Categories

Skin Diseases

Summary

Tumid erythematosus lupus is considered a rare type of chronic cutaneous lupus erythematosus. Cutaneous lupus erythematosus (CLE) can be divided into acute cutaneous lupus, subacute cutaneous lupus, and chronic cutaneous lupus.[1] Tumid erythematosus lupus is characterized by smooth, non-scarring, pinkto violet-colored pimples (papules) on the skin without any other apparent skin changes, such as scarring. Patients with tumid lupus erythematosus usually do not have other symptoms of systemic lupus erythematosus or other types of cutaneous lupus erythematosus.[2][1] The papules appear on sun-exposed areas of the face, upper back, V area of the neck, trunk, and arms, and more rarely on thighs and legs. They usually affect equally both sides of the body, but may affect only one side. Normally, the papules clear without leaving scars.[1] The treatment is very effective in most cases, and may include sun protection, anti-malarials drugslocal corticosteroids, topical tacrolimus and light therapy.[2][1][3]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Organizations Providing General Support

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      In-Depth Information

      • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.

        References

        1. Verma P, Sharma S, Yadav P, Namdeo C & Mahajan G. Tumid Lupus Erythematosus: An Intriguing Dermatopathological Connotation Treated Successfully with Topical Tacrolimus and Hydroxyxhloroquine Combination. Indian Journal of Dermatology. 2014; 59(2):210. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3969703/.
        2. Stead J, Headley C, Ioffreda M, Kovarik C & Werth V. Coexistence of Tumid Lupus Erythematosus with Systemic Lupus Erythematosus and Discoid Lupus Erythematosus: A Report of 2 cases. Journal of clinical rheumatology?: practical reports on rheumatic & musculoskeletal diseases. 2008; 14(6):338-341. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2829660/.
        3. Truchuelo MT, Boixeda P, Alcántara J, Moreno C, de las Heras E & Olasolo PJ. Pulsed dye laser as an excellent choice of treatment for lupus tumidus: a prospective study. J Eur Acad Dermatol Venereol. October, 2012; 26(10):1272-9. https://www.ncbi.nlm.nih.gov/pubmed/21957901.