Dowling–Degos disease

Dowling–Degos disease
Other names: Reticular pigmented anomaly of the flexures[1]
SpecialtyDermatology
SymptomsLace-like darkening in skin folds[1]
Usual onsetAfter puberty[1]
Diagnostic methodVisualisation, skin biopsy[2]
Differential diagnosisReticulate acropigmentation of Kitamura, Haber syndrome, neurofibromatosis type 1, acanthosis nigricans[2]
FrequencyRare, <50 cases worldwide (as of 2022)[2]
DeathsNone[2]

Dowling–Degos disease (DDD), is a type of reticulate pigmentary disorder of the skin.[1] It presents with small dark spots and bumps that form a lace-like pattern in skin folds such as underarms, neck, under breasts, and genital areas.[2] The condition may result in psychological stress.[2]

It runs in families and generally appears in adolescence, although may present in later adulthood.[1] It is due to mutations in structural/desmosomal proteins found within stratified squamous epithelium.[3] DDD is associated with KRT5.[4] Diagnosis is by visualisation and skin biopsy.[2] It may appear similar to reticulate acropigmentation of Kitamura, Haber syndrome, neurofibromatosis type 1, and acanthosis nigricans.[2]

Treatments tried include applying hydroquinone, tretinoin, adapalene, and corticosteroids, but are generally unsuccessful.[2] Applying corticosteroids and fusidic acid after Er:YAG laser has been reported to be successful in some reports.[2]

It is rare.[2] Since the first report in 1938, there have been less than 50 cases described.[2]

Signs and symptoms

  • Dowling-Degos disease
    Dowling-Degos disease
  • Dowling-Degos disease
    Dowling-Degos disease

Genetics

  • Pedigree of family with generalized Dowling-Degos disease (DDD). Father (P), first son (case 2), and second daughter (case 3) with DDD
    Pedigree of family with generalized Dowling-Degos disease (DDD). Father (P), first son (case 2), and second daughter (case 3) with DDD
  • 82-year-old father with Dowling-Degos disease
  • 58-year-old son with Dowling-Degos disease
  • 50-year-old daughter with Dowling-Degos disease
    50-year-old daughter with Dowling-Degos disease

Diagnosis

  • Skin biopsy
    Skin biopsy

See also

References

  1. 1 2 3 4 5 James, William D.; Elston, Dirk; Treat, James R.; Rosenbach, Misha A.; Neuhaus, Isaac (2020). "36. Disturbances of pigmentation: Dowling–Degos disease". Andrews' Diseases of the Skin: Clinical Dermatology (13th ed.). Edinburgh: Elsevier. p. 866. ISBN 978-0-323-54753-6. Archived from the original on 2023-06-30. Retrieved 2023-05-05.
  2. 1 2 3 4 5 6 7 8 9 10 11 12 Rice, Ashley S.; Cook, Christopher (2023). "Dowling Degos Disease". StatPearls. StatPearls Publishing. PMID 30285365. Archived from the original on 2021-04-03. Retrieved 2023-05-05.
  3. ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
  4. ↑ Adya, Keshavmurthy A; Inamadar, Arun C; Palit, Aparna (2014). "Reticulate Dermatoses". Indian Journal of Dermatology. 59 (1): 3–14. doi:10.4103/0019-5154.123480. ISSN 0019-5154. PMID 24470653. Archived from the original on 2022-08-17. Retrieved 2023-05-05.
Classification
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