Skip to main content
Article thumbnail
Location of Repository

Dowling-Degos Disease: Case Report and Review of the Literature

By Aleksandra Batycka-Baran, Wojciech Baran, Anita Hryncewicz-Gwozdz and Walter H.C. Burgdorf


Dowling-Degos disease (DDD) is an unusual pigmentary disorder usually caused by mutations in keratin 5. A 44-year-old woman in good general health presented due to the recent appearance of numerous pigmented macules on her axillary and anogenital skin. A biopsy showed lacy, finger-like epidermal extensions into the dermis which were heavily pigmented and associated with tiny cysts or dilated follicles. We view DDD as part of a spectrum of disorders which are morphologically related but vary in location and time of expression. In addition, both the clinical and histological differential diagnostic considerations are extensive. Copyright (C) 2010 S. Karger AG, Basel

Topics: Medizin, ddc:610
Publisher: Ludwig-Maximilians-Universität München
Year: 2010
DOI identifier: 10.1159/000278349
OAI identifier:
Provided by: Open Access LMU

Suggested articles


  1. (1932). Acanthosis nigricans unassociated with malignancy. Bull Soc Fr Dermatol Syphiligr
  2. (1971). Acquired axillary and inguinal pigmentation: an epidermal naevoid abnormality not to be c o n f u s e d w i t h a c a n t h o s i s n i g r i c a n s .
  3. Acquired axillary and inguinal pigmentations. Dermatologica
  4. (2001). AN: Lentiginous macules and patches of neurofibromatosis (an approach to better terminology).
  5. Axillary freckling as a diagnostic aid in neurofibromatosis.
  6. (2009). Changing a concept – controversy on the confusing spectrum of the reticulate pigmented disorders of the skin.
  7. (2006). Confl u e n t an d r eti cula t e p a p ill o ma t o s is ( Go u -gerot-Carteaud syndrome): a minocyclineresponsive dermatosis without evidence for yeast in pathogenesis. A study of 39 patients and a proposal of diagnostic criteria.
  8. (1984). Crovato F: The spectrum of Dowling-Degos disease.
  9. (1997). Elenitsas R: Oral pigmentation representing Laugier-Hunziker syndrome. Cutis
  10. (1973). Enfermedad de los puntos pardos: dark dot disease. Med Cutan Ibero Lat Am
  11. (1995). EW: Fibroblast growth factor receptor 3 (FGFR3) transmembrane mutation in Crouzon syndrome with acanthosis nigricans.
  12. (1938). Freudenthal W: Acanthosis nigricans.
  13. (1996). Fuchs E: The genetic basis of epidermolysis bullosa simplex with mottled pigmentation.
  14. (2001). Galli-Galli disease: an unrecognized entity or an acantholytic variant of Dowling-Degos disease?
  15. Grice K: Reticulate pigmented anomaly of the flexures: Dowling Degos disease, a new genodermatosis.
  16. (1967). Histologic iconography. Lentiginosis profusa perigenito-axillaris.
  17. (2007). Lin YC: Generalized DowlingDegos disease.
  18. (2006). Loss-of-function mutations in the keratin 5 gene lead to Dowling-Degos disease.
  19. Naegeli-Franceschetti-Jadassohn syndrome and dermatopathia pigmentosa reticularis: two allelic ectodermal dysplasias caused by dominant mutations in KRT14.
  20. (1954). Ossipowski B: Dermatose pigmentaire réticulée des plis. Ann Dermatol Syphiligr (Paris)
  21. (2007). p r e c h e r E , I n d e l m a n M , K h a m a y s i
  22. PTEN hamartoma tumor syndromes.
  23. (1982). T: Genodermatosis in a pair of brothers: DowlingDegos, Grover, Darier, Hailey-Hailey or Galli-Galli disease? Hautarzt
  24. (2007). The expanding spectrum of Galli-Galli disease.
  25. (2007). TM: Identification of a keratinassociated protein with a putative role in vesicle transport.
  26. (1999). WP: Dowling-Degos disease (reticulate pigmented anomaly of the flexures): a clinical and histopathologic study of 6 cases.
  27. Zhang X: Two novel mutations and evidence for haploinsufficiency of the ADAR gene in dyschromatosis symmetrica hereditaria.

To submit an update or takedown request for this paper, please submit an Update/Correction/Removal Request.