Skip to main content
Open Access Publications from the University of California

Dermatology Online Journal

Dermatology Online Journal bannerUC Davis

A review of The Pigmentary System (2nd Edition)

Main Content

A review of The Pigmentary System (2nd Edition)
Noah Scheinfeld
Dermatology Online Journal 14 (8): 18

Assistant Clinical Professor of Dermatology, Department of Dermatology, Columbia University, New York, NY.

The Pigmentary System (2nd Edition) by James J. Nordlund, Raymond Boissy Vincent J. Hearing, Richard A. King, William Oetting (Editor), Jean-Paul Ortonne (Editor)

Hardcover: 1229 pages
Publisher: Blackwell Pub; (May 1, 2006)
ISBN: 1405120347
List Price: $359.95


Great accomplishment does not mean a complete, dispositive elaboration of the future questions suggested by a great work. Moses only saw the Land of Israel from a distance, never to enter it. Several of the greatest novels of twentieth century "The Brothers Karamazov" and "The Leopard" are prequels for unwritten works of greater scope and unity. Dostoyevsky wanted to write the story of Alyosha, the mystic uncloistered, cast out into the secular world; di Lampedusa wanted to chronicle the lives of Tancredi and Angelica to explore transfer of power from the patrician to the plebeian, from the privileged to the opportunistic. These future stories remain hinted at but unelaborated.

The Pigmentary System reminds me of these works by its breadth and achievement. Vast aspects of melanin biology and pathophyisiology are explained and reviewed from the standpoint of the pigmentary system; a powerful melanin and pigmentary apparatus centered paradigm is used to accomplish this. The immune and hematology systems, circulation, and genetic "systems" that interact with the pigmentary systems are mentioned in passing as other players, not different sides of the same player. If all the human systems are considered at once, I think the concept of distinct and isolated systems would be seen as an illusion.

Specific diseases involving pigmentation that highlight the continuous interplay of human systems in health and disease exist. Progressive systemic scleroderma (sclerosis) (PSS), classified typically as a type of collagen vascular disease is not discussed in The Pigmentary Systems. Idiopathic changes of hyperpigmentation and depigmentation in PSS occur including (1) vitiligo-like depigmentation with perifollicular pigment retention mimicking repigmenting vitiligo (pseudo vitiligo of PSS), (2) diffuse hyperpigmentation with accentuation in sun exposed areas simulating adrenal insufficiency (Addison Disease), and (3) hyperpigmentation and hypopigmentation in areas of sclerosis. While rare, these manifestations of PSS highlight the limitations of the melanin and pigmentary apparatus-centered paradigm that is inadequate for explaining pigmentary alteration due to systemic causes.

In addition, a few small conditions may have been overlooked. The section on café au lait macules (CALM) on the syndromes has no sections on the syndromes of ring chromosome 7, 11, 12, 15, or 17. Why would the twisting of chromosomes into rings result in pigmentary pathology in the form of CALMs? This question highlights the inaccuracy of considering the pigmentary systems in isolation.

A disease that trumps the existing melanin and pigmentary apparatus-centered paradigm in explaining the progression of skin melanoma is ocular melanoma. I recently heard a lecture by Daniel Coit of Sloan Kettering. He explained that he was thankful that a paper of his, that suggested the lack of progression of cutaneous melanoma in the absence of lymph node pathology, had been hung up in publication for 3 years. When he went back to look at the data, the melanomas had spread anyway. I noted that melanoma does not need to spread through the lymphatics. Ocular melanoma has no access to lymphatics, but once it reaches a certain size it spreads hematogenously. Amazingly, after enucleating large ocular melanomas, the melanoma seems to assume a stealth mode, undetectable by PCR, only to become visible and detectable in the liver some years later. Physicians and researchers do not hear the crosstalk between the ocular melanoma and the hematological and immunological systems that allow such evasion to occur. What we might say is that the interplay of complex systems still has the appearance of magic, which our scientific books, focused on single systems, remain unable to capture.

The Pigmentary System remains one of the more admirably crafted and well enmeshed, put-together, multi-author texts. It explains the production and distribution of melanin in a comprehensive and agreeable style. Common expressions of pigmentary alteration, such as those around the eyes and those that occur after surgery, receive attention. The Pigmentary System made me think, for the first time, of Vogt-Koyanagi-Harada syndrome as vitiligo of the internal organs possessing melanocytes, a systemic vitiligo if you will. The Pigmentary System extensively addresses defects in pigmentary apparatus (rather the formation of melanin) such as those found in Chediak-Higashi Syndrome, Hermansky-Pudlack Syndrome, Griscelli Syndrome, Elejalde Syndrome, and Cross-McKusick-Breen Syndrome.

The Pigmentary System's discussion of a single bodily "system" is important, but I think makes the work a prolegomena rather than a summa. It leaves for future authors to fit together all "systems" that are necessary for human functionality. Scientists – who do not apply the fundamental insight of Vico that all human knowledge can be collapsed around the common point humanity – miss the unity of state of being a healthy functional human. Such an understanding suggests that researchers at the NIH who focus on static genetics, genetics in isolation, will consistently over-promise and under-deliver. The systems of the body are dynamic. Perhaps in the decades that will unfold, we will find a book entitled, Pigmentary Systemics: A Unified Approach to Pigmentation on a book-seller's table to add to our libraries as the scion to the excellent, The Pigmentary System.

© 2008 Dermatology Online Journal