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Reticulate Pigmented Anomaly Clinical Presentation

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
 
Updated: Jun 24, 2016
 

History

A rare pigmentary disorder, Dowling-Degos disease (reticulate pigmented anomaly) is characterized by reticulate pigmentation of the flexures, prominent comedolike lesions and pitted scars.

Some patients report pruritus in the involved regions. Most patients in the authors' experience report increased pigmentation in the neck, the axillae, or the groin, with an onset varying from childhood to adult life. It has been described in one family as occurring together with dyschromatosis universalis hereditaria.[19]

A rare association has been described between hidradenitis suppurativa and Dowling-Degos disease (reticulate pigmented anomaly), in one case together with multiple epidermal cysts. This may reflect a single underlying defect of follicular proliferation.[20]

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Physical

The flexural pigmentation has its onset from childhood to adult life. It may be intense, with a brownish black color and sometimes steel blue or navy overtones. However, if the condition is less severe, it is stippled in shades of brown. Rarely, the macules may be hypopigmented.[21]

No verrucous or velvety papillomatosis is present, as might be seen in acanthosis nigricans. If the patches are palpable, it is because of lichenification that produces a glossy and at times somewhat wrinkled appearance. The margins may show tiny pigmented comedones.

In some cases of Dowling-Degos disease (reticulate pigmented anomaly), comedolike papules may be present on the back and/or neck. Dowling-Degos disease with follicular localization has been characterized as an uncommon variant of this evolving dermatosis.[22] Some patients with Dowling-Degos disease (reticulate pigmented anomaly) have pitted perioral scars.

In addition, speckled macules involving the dorsum of the hands, the proximal nail folds, or the scrotum[3] may be seen. It may begin as numerous pigmented macules on the axillary and anogenital skin.[23] There may be diffuse penile pigmentation.[24] Fingernail dystrophy may be present.[17] The finding of speckled macules on the scrotum is isolated and limited to the scrotal and penile skin.[3] This pigmented eruption on the male external genitalia is possibly a cutaneous marker of underlying testicular carcinoma. In female patients, speckled macules may be found on the vulva.[25, 26] Note the image below.

Speckled macules on the male external genitalia. Speckled macules on the male external genitalia.
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Causes

See Pathophysiology. Dowling-Degos disease (reticulate pigmented anomaly) may coexist with hidradenitis suppurativa. The follicular occlusion inherent in Dowling-Degos disease (reticulate pigmented anomaly) may predispose to the development of hidradenitis suppurativa.[27, 28] PUVA therapy neither aggravates nor reveals Dowling-Degos disease,[29] although it was described in one patient after this treatment.

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Contributor Information and Disclosures
Author

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.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, Rutgers New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

Mark A Crowe, MD Assistant Clinical Instructor, Department of Medicine, Division of Dermatology, University of Washington School of Medicine

Mark A Crowe, MD is a member of the following medical societies: American Academy of Dermatology and North American Clinical Dermatologic Society

Disclosure: Nothing to disclose.

Borys Savchyk, MD, PhD Director, Molecular Biology Institute, Medical University of Lviv, Ukraine

Disclosure: Nothing to disclose.

Alexander Zajchenko, MD Head, Professor, Department of Skin and Venereal Diseases, Medical University of Lviv, Ukraine

Disclosure: Nothing to disclose.

References
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Speckled macules on the male external genitalia.
Histologic view showing elongated thin rete ridges with melanin concentrated at the tips (hematoxylin and eosin).
 
 
 
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