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Dermatopathia Pigmentosa Reticularis Differential Diagnoses

  • Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: Dirk M Elston, MD  more...
Updated: Jan 19, 2016

Diagnostic ConsiderationsPoints of differentiation

The diagnosis of dermatopathia pigmentosa reticularis (DPR) is based on the presence of the clinical triad and a typical histopathologic picture; however, the following hyperpigmentation disorders should be considered and excluded:

  • Acromelanosis progressiva
  • Hereditary symmetric dyschromatosis of Dohi
  • Reticular acropigmentation of Kitamura
  • Heterochromia extremitarium
  • Reticulate pigmented dermatosis of the flexures
  • Congenital diffuse mottling of the skin
  • Hereditary universal dyschromatosis
  • Dyskeratosis congenita syndrome
  • Franceschetti-Jadassohn or Naegeli syndrome

Each of the following demonstrate a distinctive distribution of pigmentation with diffuse frecklelike or reticulate hyperpigmentation on the dorsa of the hands and feet, the extremities, or the flexural areas (axillae, groin): acromelanosis progressiva, hereditary symmetric dyschromatosis of Dohi, reticular acropigmentation of Kitamura, heterochromia extremitarium, and reticulate pigmented dermatosis of the flexures.

Differentiation from congenital diffuse mottling of the skin can be made histopathologically by demonstrating clubbing of the rete ridges and hyperpigmentation of the basal layer without pigmentary incontinence.

Hereditary universal dyschromatosis is associated with hyperpigmented and hypopigmented macules of irregular shape and size, as well as small stature and high-tone deafness.

Dyskeratosis congenita syndrome is an X-linked condition and occurs most commonly in males, usually, in males older than 5 years. In addition to reticulate hyperpigmentation, nail dystrophy, and hyperkeratosis and atrophy of the palms and soles, patients with dyskeratosis congenita also have leukokeratosis of mucosal surfaces and blood dyscrasias.

Franceschetti-Jadassohn (Naegeli) syndrome is an autosomal dominant condition with gray-brown reticulate pigmentation of the trunk and neck that fades after adolescence. Dental anomalies and a bleeding tendency are frequently observed in these patients.

Franceschetti-Jadassohn (Naegeli) syndrome and dermatopathia pigmentosa reticularis share complete absence of dermatoglyphics; a reticulate pattern of hyperpigmentation; palmoplantar keratoderma; abnormal sweating; and anomalies of the teeth, hair, and skin.[20]

Differential Diagnoses

Contributor Information and Disclosures

Noah S Scheinfeld, JD, MD, FAAD Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

Noah S Scheinfeld, JD, MD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Abbvie<br/>Received income in an amount equal to or greater than $250 from: Optigenex<br/>Received salary from Optigenex for employment.


Hong-Duo Chen, MD Director, Immunodermatological Key Laboratory; Professor, Department of Dermatology, Number 1 Hospital, China Medical University, China

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Jacek C Szepietowski, MD, PhD Professor, Vice-Head, Department of Dermatology, Venereology and Allergology, Wroclaw Medical University; Director of the Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Poland

Disclosure: Received consulting fee from Orfagen for consulting; Received consulting fee from Maruho for consulting; Received consulting fee from Astellas for consulting; Received consulting fee from Abbott for consulting; Received consulting fee from Leo Pharma for consulting; Received consulting fee from Biogenoma for consulting; Received honoraria from Janssen for speaking and teaching; Received honoraria from Medac for speaking and teaching; Received consulting fee from Dignity Sciences for consulting; .

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