Acquisition and loss: A tribute to Jouni Uitto, MD, PhD

By Warren R. Heymann, MD, FAAD
Jan. 11, 2023
Vol. 5, No. 2

I was stunned and frozen in time and thought when Dr. Sylvia Hsu informed me that Dr. Jouni Uitto had passed away — a similar reaction as I experienced when hearing of President Kennedy’s or John Lennon’s assassination. How could this be? Jouni and I were on a Zoom Journal Club weeks before, as he was graciously reviewing an article on Kindler syndrome. I first met Jouni when he came to chair the Department of Dermatology at Jefferson in 1986, when I was getting my feet wet at Cooper Hospital. Jouni was recruited to Jefferson as an esteemed international authority on collagen disorders. Naturally, I was impressed by his achievements, but I was awed by his warmth and generosity of spirit, which blossomed over our decades working together through the Philadelphia Dermatological Society (PDS). The dermatology universe has lost a scholar; I have lost a mentor and friend. I was fortunate to express my thanks to him for all he has done for the dermatology community and me via email — his wife told me that he smiled when she read it to him. I will always cherish that. Please read Dr. Neda Nikbakht’s elegant obituary distributed to the PDS (following this commentary).

As I was perusing the literature, I came across what is likely to be one of Dr. Uitto’s final publications — a superlative review of acquired ichthyosis (AI), published in the January 2023 issue of the Journal of the European Academy of Dermatology and Venereology. (1) Ichthyosis vulgaris (IV) is an inherited form of non-syndromic ichthyosis, with a very early age of onset. IV is associated with loss of function FLG gene mutations, which leads to a defective skin barrier. (2) This commentary will focus on AI. In honor of Dr. Uitto, I will present the abstract in italics, adding some newer literature in brackets without italics.

Acquired ichthyosis (AI) is a relatively rare cutaneous entity characterized by transient, generalized scaling and pruritus in the absence of family history of ichthyosis or atopic disease. The hyperkeratosis in AI can range from the mild, white-to-brown scaling resembling that in ichthyosis vulgaris (IV) to the more prominent dark brown scaling phenotype, similar to that found in lamellar ichthyosis. [Kurim et al presented a case of AI in a 69-year-old woman with a history of relapsed acute lymphocytic leukemia. She displayed plate-like sheets of desquamation 15 days after the administration of ponatinib, a third-generation tyrosine kinase inhibitor. (3)] The disease can wax and wane in relation to endogenous and/or exogenous factors. Histopathology of AI is similar to that found in IV. [Absence of the granular layer with diminished filaggrin. (4)] AI is usually of cosmetic concern to patients but can, in some cases, reflect the presence of more serious conditions, including malignancies [especially lymphoproliferative disorders such as Hodgkin disease, cutaneous T-cell lymphoma, multiple myeloma, and others (2,5)], autoimmune diseases [such as sarcoidosis, hypothyroidism, others (6,7)] or metabolic disorders. [Infectious diseases such as HIV/AIDS, HTLV-1, Hansen disease, tuberculosis, and others may be associated with AI (1)] In some cases, AI can be an adverse effect of a medication [especially statins, niacin, allopurinol, EGFR inhibitors, BRAF inhibitors, and many others (1,2)] or the cutaneous symptom of a toxic exposure [paradichlorobenzene in mothballs, kava beverages (1)]. Other conditions, such as severe xerosis or eczema, can present with clinical findings similar to AI, making diagnosis a challenge. [Austin et al presented a striking case of a 79-year-old woman with dramatic AI that proved to be seborrheic dermatitis (8)]. Furthermore, cases of AI are sporadic throughout the literature and have been documented across a wide variety of medical settings distinct from dermatology, which often contribute to misdiagnosis of this disease. Definitive management requires prompt identification and treatment of the inciting factors combined with conservative therapies, which can include topical emollients, keratolytics, retinoids or corticosteroids, and in rare cases, oral retinoids.

From genetics to the bedside, Dr. Uitto epitomized excellence in dermatology. He was a colleague, friend, and most importantly, a true mensch.

Point to Remember: There are acquisitions and losses in life. In dermatology, acquired ichthyosis mandates a careful history, physical examination, and laboratory assessment to ascertain if there are associated disorders. In life, if we are fortunate, we acquire dear friends. Losing them is hard, but with time, we treasure the all too brief time we had together. Thank you, Dr. Uitto.

Our expert’s viewpoints

Sylvia Hsu, MD, FAAD
Professor and Chair
Department of Dermatology
Temple University Lewis Katz School of Medicine

Dermatology has lost a great legend. Every day, Dr. Uitto would walk through his laboratories and jokingly ask everyone: “Are you working hard or hardly working?” He was the brains behind every project, and his motto was “a paper a day.” I was in awe of how he easily wrote papers by looking at the data presented to him and dictating an entire manuscript, including all the references from the top of his head. He would be done with the manuscript in no time! Fast forward 30 years and the most recent paper we worked on together was also Dr. Uitto’s idea — acquired ichthyosis (AI). AI in isolation is a self-limiting cutaneous entity with several overlapping clinical mimics, such as ichthyosis vulgaris, asteatotic dermatosis, and severe xerosis. The acute appearance of dry, scaly skin in an otherwise healthy individual may not elicit concern from a patient’s perspective, but it should prompt further investigation or surveillance by clinicians. In most cases, AI is a cosmetic concern. However, it can also be the initial presenting sign of a systemic disorder. The most commonly reported associations of AI include medications and malignancies, particularly Hodgkin lymphoma and the CD30-positive lymphoproliferative disorders. This AI paper was published shortly after Dr. Uitto passed away. Rest in peace, Dr. Uitto. You will be sorely missed.

Jason B. Lee, MD, FAAD
Clinical Vice Chair
Director, Jefferson Dermatopathology Center
Director, Jefferson Pigmented Lesion Clinic
Jefferson University Hospitals

Dr. Uitto had a profound influence on the careers of many individuals, including mine. He shaped the trajectory of my career path by having the confidence in me to build a dermatopathology laboratory fresh out of my fellowship 25 years ago. Over the years, I have come to know a man of unwavering dedication to his research and the people around him. He was always approachable, cheerful, and encouraging, personal attributes we all should strive to emulate. He was a steadfast pillar in our department for nearly 36 years who will be dearly missed by all of us who were touched by him.

Dr. Uitto’s obituary, as shared with members of the Philadelphia Dermatological Society
Neda Nikbakht, MD, FAAD
Associate Professor of Dermatology
Director, Cutaneous Lymphoma Clinic
Department of Dermatology and Cutaneous Biology
Thomas Jefferson University

Jouni Uitto, MD, PhD, 1943-2022

Dear Members of the Philadelphia Dermatological Society,

It is with great sadness that I announce the passing of Dr. Jouni Uitto, Professor and Chair of the Department of Dermatology and Cutaneous Biology at Thomas Jefferson University where he served as the longest acting chair for 36 years. He passed away peacefully on December 17, 2022, with his family by his side. Dr. Uitto had a profound impact on the global scientific community and made tremendous contributions to the field of dermatology.

Born and raised in Helsinki, Finland, Dr. Uitto graduated from the University of Helsinki with an MD and PhD in biochemistry in 1970. He then completed a five-year post-doctoral fellowship in Philadelphia, followed by a dermatology residency at Washington University School of Medicine in St. Louis. He began his academic career with a faculty appointment at the University of California, Los Angeles.

Dr. Uitto moved from California to Philadelphia in 1986 to lead the Dermatology Department at Jefferson. Over the years, he made significant discoveries in understanding the genetic basis of skin disease particularly regarding epidermolysis bullosa and pseudoxanthoma elasticum. Dr. Uitto was a deeply committed and an exemplary mentor. He trained more than 140 research fellows from 28 countries, 19 of whom became chairs of departments in their respective fields.

Dr. Uitto was the recipient of numerous national and international accolades over the course of his career. Some of his most prestigious research awards include: the highest honor of international dermatology, the Marchionini Gold Medal (2019); the Knight of the White Rose of the Republic of Finland (2020); the Society for Investigative Dermatology’s most prestigious awards, the Montagna Lecture (2002) and Rothman award (2014); and the American Academy of Dermatology’s Eugene J. Van Scott Award for Innovative Therapy of the Skin-Philip Frost Leadership Lecture (2013). Dr. Uitto published close to 1000 peer-reviewed articles, received more than 70,000 citations, and currently holds an H-index of 133. 

Dr. Uitto touched so many lives both professionally and personally and will be greatly missed. The Uitto family has elected to hold arrangements privately. Cards may be directed to Department of Dermatology at Jefferson, c/o Carol Kelly at 450 BLSB, 233 S. 10th Street, Philadelphia, PA, 19107. An opportunity will be announced for contributions to a lectureship fund in Dr. Uitto’s memory. In addition, the Philadelphia Dermatological Society will dedicate an in-person event at the College of Physicians of Philadelphia on Wednesday, June 14, 2023, with lectures highlighting Dr. Uitto’s scientific legacy.

1. Park JS, Saeidian AH, Youssefian L, Hsu S, Vahidnezhad H, Uitto J. Acquired ichthyosis, asteatotic dermatitis or xerosis? An update on pathoetiology and drug-induced associations. J Eur Acad Dermatol Venereol. 2023 Jan;37(1):47-56. doi: 10.1111/jdv.18608. Epub 2022 Oct 7. PMID: 36165597.

2. Majmundar VD, Baxi K. Hereditary And Acquired Ichthyosis Vulgaris. 2022 Aug 8. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan–. PMID: 32965989.

3. Karim MS, Watson I, Boh EE. Diffuse plate-like sheets of desquamation. JAAD Case Rep. 2022 Jan 6;21:52-55. doi: 10.1016/j.jdcr.2021.11.032. PMID: 35146102; PMCID: PMC8818897.

4. Hagiwara R, Shinkuma S, Yokoyama R, Ansai O, Hayashi R, Katagiri T, Abe R. Acquired ichthyosis disclosing intravascular large B-cell lymphoma. J Dermatol. 2021 Oct;48(10):E500-E501. doi: 10.1111/1346-8138.16051. Epub 2021 Jul 6. PMID: 34227138.

5. Hagiwara R, Shinkuma S, Yokoyama R, Ansai O, Hayashi R, Katagiri T, Abe R. Acquired ichthyosis disclosing intravascular large B-cell lymphoma. J Dermatol. 2021 Oct;48(10):E500-E501. doi: 10.1111/1346-8138.16051. Epub 2021 Jul 6. PMID: 34227138.

6. Saardi KM, DeWitt CA, Cardis MA. Acquired Ichthyosis in a Middle-aged Woman. JAMA Dermatol. 2020 Jul 1;156(7):809-810. doi: 10.1001/jamadermatol.2020.1081. PMID: 32401266.

7. Delaleu J, Nguyen H, Jachiet M, Vignon-Pennamen MD, Bondéelle L, Vidal-Trécan T, Bouaziz JD, Bagot M, de Masson A. Hypothyroidism revealed by acquired ichthyosis in an adult patient. Ann Dermatol Venereol. 2021 Jun;148(2):130-132. doi: 10.1016/j.annder.2020.10.017. Epub 2021 Jan 16. PMID: 33461790.

8. Delaleu J, Nguyen H, Jachiet M, Vignon-Pennamen MD, Bondéelle L, Vidal-Trécan T, Bouaziz JD, Bagot M, de Masson A. Hypothyroidism revealed by acquired ichthyosis in an adult patient. Ann Dermatol Venereol. 2021 Jun;148(2):130-132. doi: 10.1016/j.annder.2020.10.017. Epub 2021 Jan 16. PMID: 33461790.

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