Patterned Past: Satirical Tales of Piebaldism Unveiled through the Ages

Prologue Approaches to skin and body differences in the 18thcentury were varied. The medical community attempted to categorise people as specimens – examples of how human beings had deviated from what was considered normal. For the public, curiosity about body difference became a form of entertaining spectacle. People went to fairs and shows to view giants, dwarves, fat people, spotted children and black albinos, all of whom were considered wonders of nature. How much of this has changed? Are we as curious about visible differences now as people were then? What is it like to live with similar conditions today?[1] - Temi Odumosu Role of Melanocytes in Human History and Evolution Evolutionary hypothesis has long tried to explain the stark differences in skin color amongst humans. Melanin protected hominids from damaging ultraviolet (UV) rays in the savanna mosaic of Africa. Evolution of light skin came about due to the need to produce more Vitamin D in the ice age of Eucrasia.[2] Dermatologists have long been taught that density of melanocytes is the same in all races and that diversity in color is due to differences in proportion of eumelanin and pheomelanin.[3] In spite of mere biochemical changes, there has always been a fundamental point of friction regarding skin color, to say the least. Although stigma is prevalent in many aspects of medical care, addressing negative connotations in dermatological disorders becomes paramount as the visibility of the skin contributes to heightened stigmatization and requires a considerable focus within the realm of medical care provided by dermatologists. Disorders such as leprosy, vitiligo, atopic dermatitis, and albinism are long known to affect, not just the quality of life but also psychosocial well-being. Addressing stigma in skin disorders becomes preeminent in the pediatric population as it can adversely affect long-term psychosocial development of the child and foster negative attitude toward others as early as preschool age.[4] Not White Enough, Not Black Enough The most radical denouncement faced by melanized populations is usually toward depigmentary disorders such as vitiligo, albinism, piebaldism, and hypopigmentary nevoid conditions. Although these disorders can be seen in people of any color, the stark contrast of leukoderma patches against natural melanized skin color makes them more vulnerable toward prejudice by their community as well.[5] Stereotypes and myths regarding albinism, especially in the African population, have been documented in literature. Individuals affected with albinism have encountered a range of superstitions and remarks. Unfounded claims of origin of these disorders exist. From being struck by lightning in utero enabling them to read people’s thoughts; to misguided belief like the notion that engaging in intimate relations with woman with albinism can cure a man with human immunodeficiency virus (HIV), all have put affected individuals in grave danger. In addition, they were subjected to alarming threat of being hunted for their body parts as they were believed by some to possess supernatural or magical powers International Federation of Red Cross and Red Crescent Societies’ report on albinism in 2009 portrays a grim statistic regarding albino children enduring horrific kidnappings, amputations, decapitations, and transactional black market that dealt with the sale of their body parts sold as amulets, all against the backdrop of misinformation, ignorance, and superstition.[6] The enduring impact persists even today through experiences of teasing, staring, along with well-intentioned yet uninformed questions regarding their appearances.[7] They are adept at the theory of othering that envelops stereotyping, prejudice, and stigmatization. This makes people affected by these disorders as dissimilar from what is considered “the normal.”[8] Piebaldism: Relics of a Bygone Age Piebaldism, once referred to as partial albinism, has been long known to be a distinct genodermatoses unrelated to albinism. Albinism is an autosomal recessive disorder related to deficiencies in melanin production and piebaldism is an autosomal dominant disorder of melanocyte migration characterized by stark depigmentary patches over mid-forehead, extremities, and white forelock. Etymology of piebaldism is dawned from the Latin word for “magpie,” used to describe animals that have black and white patches on their bodies. Affiliation to animals was perpetuated in the 1700s when people affected with piebaldism were referred to as “leopard” or “zebra people.” People with distinct disorders such as dwarfism, gigantism, albinism, and piebaldism, historically regarded as ‘wonders of nature,’ were bought and paraded in fairs and show by white colonialist showmen for money. In addition, they were also a source of medical amusement and hence were enshrined in artwork and medical literature simultaneously dehumanizing their very legacy.[5] Temi Odumosu, an author, art historian, and exhibition curator of “A Visible Difference: skin, Race and Identity 1720–1820,” has compelled readers and art enthusiasts to question the conscience related to such colonial artwork, especially pertaining to slavery.[1] Portraits of George Alexander Gratton [Figure 1], Mary Sabina [Figure 2], and John Bobey [Figures 3 and 4] are three such representations of people with piebaldism and a memoir of what little is known about them is mentioned in Table 1.[1]Figure 1: D8254; The Portrait of George Alexander, An Extraordinary Spotted Boy from the Caribbean Islands in the West Indies. Courtesy of National Portrait Gallery, London. Https://npgimages.com/search/?Searchquery=alexander+grattonFigure 2: RCSSC/P256; Portrait of Mary Sabina (b 1736), oil on canvas, artist unknown, c. 1744. Courtesy of the Royal College of Surgeons of England. Http://surgicat.rcseng.ac.uk/Details/collect/46017Figure 3: 172i; John Boby, a black man with white markings. Reproduction of an etching, 1803.https://wellcomecollection.org/search?query=john+boby#bkf9tnf6Figure 4: Primrose Bobey, John Richardson (b. 1772). Ne Plus Ultra. Arrived Here for a Short Time Only (Hull, ca. 1790). Courtesy of Potter and Potter Auctions. https://auctions.potterauctions.com/LotDetail.aspx?inventoryid=63,271Table 1: Lives of individuals affected with piebaldism through history as portrayed by art[ 1 ]Piebaldism: A Curable Genodermatosis Piebaldism and Waardenburg syndrome, both are disorders of melanocyte proliferation from neural crest cells, hence present with an anterior white forelock, among other distinct attributes. Migration, proliferation, and differentiation of melanocytes take place along the dorsolateral pathway reaching ventrally, which is its most distant site. Hence, stable congenital leukoderma patches prominent at the midline is a stark presentation of piebaldism.[9] This distinct absence of melanocytes on the affected areas has led to the inception of vitiligo surgeries being performed on piebaldism patients.[10] PubMed searches report various cellular and tissue grafts being tried for piebaldism. This has opened hopeful avenues for people affected by piebaldism, albeit skill dependent. Parallels with Respect to the Present Day Although portraits and treatment of affected individuals seem like a problem of the ancient past and of an unrelated race, the juxtaposition is not farfetched. Humans have always been bewitched by fairer skin. Colorism or act of discrimination against a person due to the differences in their skin color has been long used as a strategy and a stratification tool by imperialist nations.[11] From discrimination in job opportunities to the formation of racial gerrymandering, the postcolonial hierarchy still exists with respect to skin color.[12] The inherent postcolonial mindset of associating a light-skinned individual as ‘beautiful’ is still prevalent and blatant even in India. A similar present-day practice is noticed in our daily enactment where parents bring their children with a primary complaint of dark skin that requires a glowing or skin-lightening treatment.[11] Children with heavily melanized skin are self-conscious about their skin color very early in their lives, likely due to other family members mentioning it casually or offering home remedies to rectify the same. Depravity of the Skin-lightening Industry and Hypocrisy of the Skin Color: An Introspection From having milk with saffron to the application of turmeric and milk cream as a face mask, the one industry that has exploited people’s insecurity is the billion-dollar skin-lightening market. Fair and Lovely, now Glow and Lovely, has long paraded naturally light-skinned women in a country of melanized girls making them insecure in their own skin. Just in the past 14 years, PubMed search of ‘skin lightening’ has shown a drastic influx of research articles abridged to a point where adverse effects of various depigmenting agents have become the forefront of discussion. As dermatologists, we rarely ask or address a patient’s bias and insecurities when such treatments are sought. Despite this delusion of wanting a fairer skin tone, a disorder that reduces skin color such as vitiligo, has severe psychological effects on affected individuals severely affecting their quality of life.[13] This disproportionately affects women. From regarding the disease grotesque, being treated as social pariahs, to having suicidal ideations, the impact of a disease like vitiligo just based off of a difference in color on affected individuals is dire.[14] This dichotomous hypocrisy is fittingly expressed in the poem “The Mirror” by Dr. Feroze Kaliyadan, especially for the Indian context.[15] Epilogue Despite social advancements, presence of a colonial mindset remains apparent. People today are still just as curious about visible differences in skin color as they were back in the past. To combat this issue, modern society needs to implement extensive measures, ensuring that individuals affected do not face the hardships of being scrutinized for their skin. They are definitely cognizant of their dyspigmentation and do not need the rest of us to make it apparent. Demystifying misinformation, facilitating open communication channels, and garnering support from the families and community are crucial steps in combating this issue to ensure they lead normal lives. Till then, it looks like we will stay just as archaic as the paintings we gaze at. Declaration of consent The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s)/guardian(s) of the patient. In the form, the parent(s)/guardian(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child/children will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.