A number sign (#) is used with this entry because of evidence that Haim-Munk syndrome (HMS) is caused by homozygous mutation in the gene encoding. Haim–Munk syndrome is a cutaneous condition caused, like Papillon-Lefevre Syndrome, by a mutation in the cathepsin C gene. It is named after Dr. Salim Haim. Abstract. Of the many palmoplantar keratoderma (PPK) conditions, only Papillon- Lefèvre syndrome (PLS) and Haim-Munk syndrome (HMS) are associated with.
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Haim-Munk syndrome is a rare genetic disorder characterized by the development of red, scaly thickened patches of skin on the palms of the hands and soles of the feet palmoplantar hyperkeratosisfrequent pus-producing pyogenic skin infections, overgrowth hypertrophy of the fingernails and toenails onychogryposisand degeneration of the structures that surround and support the teeth periodontosis.
Patients also demonstrate hypertrophy and curving of nails onychogryphosisflat footextreme length and slenderness of fingers and toes arachnodactylyand osteolysis involving the distal phalanges of fingers and toes acro-osteolysis.
The permanent teeth then erupted normally followed by severe gingival inflammation. The proband did have a history of repeated skin infections. For the first month, patient was kept on weekly recalls, with motivation of patient and reinforcement of oral hygiene. In lateral projection of feet [ Figure 8 ], the line of the first metatarsal made an angle instead of coinciding with the midtalar line revealing that the proband had flat feet.
CC ]. X-ray of hands revealed that the metacarpal index was 9. Resources Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder [e.
Related articles Haim Munk syndrome palmo plantar keratoderma acetretin acroosteolysis cathepsin C gene. Clinical Synopsis Toggle Dropdown.
Chromosomes are found in the nucleus of all body cells. Priyanka Pahwa,S.
How to cite this article: Views Read Edit View history. Pediatricians, surgeons, physicians who evaluate and treat skin problems dermatologistsdentists, specialists in treating disorders affecting the structures supporting and surrounding the teeth periodontistsspecialists in treating disorders affecting the feet podiatristsand other health care professionals may need to systematically and comprehensively plan an affected child’s treatment.
Comparisons may be useful for a differential diagnosis:. Meleda disease is inherited as an autosomal recessive trait. This case report describes a patient with the cardinal features of Haim-Munk syndrome. A genetic analysis of the Papillon-Lefevre syndrome in a Jewish family from Cochin.
Keratosis palmo-plantaris congenita, with periodontosis, arachnodactyly and a peculiar deformity of the terminal phalanges. Photographs of younger girl. The skin was dry and rough to touch. The content of the website and databases of the National Organization for Rare Disorders NORD is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD.
Genetic studies of syndromes with severe periodontitis and palmoplantar hyperkeratosis. In most cases, syyndrome with Haim-Munk syndrome exhibit overgrowth hypertrophy of the fingernails and toenails causing them to become abnormally thick to appear hooked and curved inward.
However, most affected individuals develop chronic severe inflammation and degeneration of the tissues that surround and support the teeth gingivitis and periodontosis.
There are several additional disorders that are characterized by skin abnormalities similar to those observed in Haim-Munk syndrome. Use of special footwear may help affected individuals who exhibit flat feet pes planus. Both the siblings were treated with cotrimoxazole, acetretin and topical keratolytics and followed up over a period of one year, showed remarkable improvement in palmo plantar keratoderma and periodontitis.
The pattern was entirely consistent with autosomal recessive inheritance. Puliyel and Sridharan Iyer found 20 persons with Haim-Munk syndrome in 9 sibships over 4 generations of an inbred kindred.
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Radiographs of the elder boy. Unfortunately, it is not free to produce. Puliyel and Sridharan Iyer noted that the patients reported by Smith and Rosenzweig were related to the patients of Haim and Munk Physicians may carefully monitor affected individuals to help prevent and ensure early identification of infection. Both deciduous and mjnk teeth are usually affected in the order of their eruption. In addition, Hart et al. The syndrome of palmar-plantar hyperkeratosis and premature periodontal destruction of the teeth.
On intraoral examination, marked drifting of teeth was noticed.