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Salim Haim and the syndrome that bears his name

  • Author(s): Aboud, Khalid Al
  • Aboud, Daifullah Al
  • et al.
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Letter: Salim Haim and the syndrome that bears his name
Khalid Al Aboud MD1, Daifullah Al Aboud MD2
Dermatology Online Journal 17 (8): 15

1. Dermatology Department, King Faisal Hospital, Makkah, Saudi Arabia
2. Dermatology Department, Taif University, Taif, Saudi Arabia


Abstract

Professor Salim Haim (1919-1983) was a well-known dermatologist. In 1965, Dr. Haim and Dr. Munk, a radiologist, reported a rare congenital type of genodermatosis, later known as Haim-Munk syndrome. This syndrome is characterized by palmoplantar keratosis, pes planus, onychogryphosis, periodontitis, arachnodactyly, and acroosteolysis. This report discusses Haim and Haim-Munk syndrome.



Figure 1Figure 2
Figure 1. Salim Haim (1919-1983). This picture was taken in 1970.

Figure 2. This picture was taken in 1981, approximately 2 years before he died at the age of 64.

Professor Salim Haim (1919-1983) (Figures 1 and 2) was a renowned dermatologist [1]. Among his great contributions to dermatology, he is credited with describing a syndrome [2], later known as Haim-Munk syndrome (HMS) [3-11] .

HMS is also known as keratosis palmoplantaris with periodontopathia and onychogryposis and as Cochin Jewish disorder [3].

HMS is a rare autosomal recessive condition that has affected few families worldwide [8, 10]. It is caused by a mutation in the cathepsin C gene (CTSC) [11].

The hallmarks of HMS include palmoplantar keratoderma and periodontitis, as well as arachnodactyly, acroosteolysis, onychogryphosis, and marked osteopenia on hand-wrist radiographs [11]. A patient with HMS, who had destructive arthritis of the wrists and shoulder joints, has also been reported [6].

HMS (OMIM #245010) is allelic to Papillon-Lefevre syndrome (PLS) (OMIM #245000) and juvenile periodontitis, also known as prepubertal periodontitis (PPP) (OMIM #170650) [3].

These disorders are caused by mutations in cathepsin C (CTSC), mapped to 11q14.2.

PPP is a rare and rapidly progressive disease in young children that results in the destruction of the periodontal support of the primary dentition. This condition may occur as part of a recognized syndrome or as an isolated finding. Both autosomal dominant and recessive forms of Mendelian transmission have been reported for PPP [5]. Of the many types of palmoplantar keratoderma (PPK), only PLS and HMS are associated with premature periodontal destruction [4, 9]. Because HMS and PLS are allelic variants of cathepsin C mutations, some groups have suggested that other factors (environmental or genetic) are important determinants of the clinical phenotype of HMS and PLS [7].

Whereas PLS has been detected worldwide, HMS has only been described among descendants of a religious isolate that originated in Cochin, India. Parental consanguinity is a characteristic of many cases of both conditions [4].

PLS affects calcification of the dura mater and is associated with increased susceptibility to infections. HMS is marked by arachnodactyly, acroosteolysis, and onychogryphosis [3].

Skin manifestations are more severe and develop later in HMS than in PLS. The periodontium is less severely affected in HMS than in PLS [3].

There is no specific treatment for HMS. However, one report noted 2 cases of HMS in the same family [11]. Both siblings were treated with cotrimoxazole, acetretin, and topical keratolytics and were followed up for over 1 year, demonstrating remarkable improvement in their palmoplantar keratoderma, and periodontitis.

Prof. Haim was born in Baghdad in 1919. He began his medical studies at the American University of Beirut and received his MD in 1944 at the Royal College in Baghdad, Iraq [1]. He immigrated to Haifa in 1951 and worked alongside Prof. Ziperkowsky in the Dept. of Dermatology and Venereology at Tel Hashomer Hospital [1]. In 1954, Prof. Haim served as an army physician at the #10 Hospital in Haifa. In 1957, he established a dermatology and venereology department at Rambam Hospital in Haifa [1], acting as department head until his death in 1983. In 1965, with Dr. Munk, he described a syndrome, later known as Haim-Munk syndrome, among members of a small community of Jews from Cochin, India [3] at Rambam Medical Center, Haifa.

In 1973, he received an associate professorship in Dermatology and Venereology at the Technion Faculty of Medicine in Haifa and became full professor in 1979 [1].

He published more than 100 papers in dermatology in Hebrew [12] and English. He wrote on diverse topics in dermatology, including mouth and nail disorders [12, 13], but Behcet disease was one of his favorite subjects.

His extensive research of Behcet disease made him famous worldwide [1]. He felt strongly that dermatological diseases are multisystemic and attempted to prove this theory in his many studies. He was one of the first teachers at the Technion Faculty of Medicine, established in 1973 in Haifa [1]. Professor Haim died in Haifa in 1983.

ACKNOWLEDGMENTS: The authors wish to thank Nissim Haim, MD, the son of Prof. Haim, who is the Director of the Chemotherapy Unit, Division of Oncology, Rambam Medical Center, and Dr. Arieh Ingber for providing assistance in preparing this manuscript.

References

1. Ingber A. History of dermatology and venereology medicine in Israel: the "founding fathers." IMAJ 2008; 10:406-409. [PubMed]

2. Haim S, Munk J. Keratosis palmo-plantaris congenita, with periodontosis, archnodactyly and peculiar deformity of the terminal phalanges. Brit J Derm 1965; 77: 42-54. [PubMed]

3. Haim-Munk syndrome; HMS. [A page on the Internet]. From OMIM, Online Mendelian Inheritance in Man. Copyright (c) 1966-2011 Johns Hopkins University [This page was last modified 2007 April 25; cited 2011 April 5]. Available at http://www.ncbi.nlm.nih.gov/omim/245010

4. Hart TC, Hart PS, Michalec MD, Zhang Y, Firatli E, Van Dyke TE, Stabholz A, Zlotogorski A, Shapira L, Soskolne WA. Haim-Munk syndrome and Papillon-Lefèvre syndrome are allelic mutations in cathepsin C. J Med Genet. 2000 ; 37(2):88-94. [PubMed]

5. Hart TC, Hart PS, Michalec MD, Zhang Y, Marazita ML, Cooper M, Yassin OM, Nusier M, Walker S. Localisation of a gene for prepubertal periodontitis to chromosome 11q14 and identification of a cathepsin C gene mutation. J Med Genet. 2000 ; 37(2):95-101. [PubMed]

6. Lidar M, Zlotogorski A, Langevitz P, Tweezer-Zaks N, Zandman-Goddard G. Destructive arthritis in a patient with Haim-munk syndrome. J Rheumatol. 2004 ; 31(4):814-7. [PubMed]

7. Cury VF, Gomez RS, Costa JE, Friedman E, Boson W, De Marco L. A homozygous cathepsin C mutation associated with Haim-Munk syndrome. Br J Dermatol. 2005 ; 152(2):353-6. [PubMed]

8. Janjua SA, Iftikhar N, Hussain I, Khachemoune A. Dermatologic, periodontal, and skeletal manifestations of Haim-Munk syndrome in two siblings. J Am Acad Dermatol. 2008 ; 58(2):339-44. [PubMed]

9. Rai R, Thiagarajan S, Mohandas S, Natarajan K, Shanmuga Sekar C, Ramalingam S. Haim Munk syndrome and Papillon Lefevre syndrome--allelic mutations in cathepsin C with variation in phenotype. Int J Dermatol. 2010 ; 49(5):541-3. [PubMed]

10. Erciyas K, Inaloz S, Erciyas AF. Periodontal manifestations in a patient with haim-munk syndrome. Eur J Dent. 2010 ; 4(3):338-40. [PubMed]

11. Mohan RS, Verma S. Haim Munk syndrome: Report of two siblings of Northern India treated with acitretin. Indian J Dermatol Venereol Leprol. 2011; 77(2):252. [PubMed]

12. Haim S. [Oral leukoplakia]. Harefuah. 1981; 101(1-2):35-7. [PubMed]

13. Almagor G, Haim S. Familial koilonychia.Dermatologica. 1981; 162(5):400-3. [PubMed]

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