EHLERS-DANLOS SYNDROME WITH MULUSCOID PSEUDO-TUMOR, SPHEROID AND KERATINOUS CYST – REPORT A RARE CASE

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MOGHIMI MANSOOR; SOLTANI HAMID REZA; AMINI RAD OMID

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Paper Information

Journal: Journal of Isfahan Medical School Year:2011 | Volume:28 | Issue:113 Page(s): 787-793

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Title

EHLERS-DANLOS SYNDROME WITH MULUSCOID PSEUDO-TUMOR, SPHEROID AND KERATINOUS CYST – REPORT A RARE CASE

Author(s)

MOGHIMI MANSOOR | SOLTANI HAMID REZA | AMINI RAD OMID | Issue Writer Certificate

Keywords

EHLERS-DANLOSQ3

MULUSCOID PSEUDO-TUMORQ3

SPHEROIDQ3

KERATINOUS CYSTQ3

Abstract

Background: EHLERS-DANLOS syndrome is consisted of a group of heterogeneous and rare inherited disorders of connective tissue that is diagnosed by increased range of joint movements, friability, and increasing of skin elasticity. The diagnosis of this syndrome is often difficult and based on clinical criteria and family history. Many skin lesions are reported from the disease up to now and various and simultaneously skin lesions in this case is the main propose of this report.Case report: A 11-year-old male with cutaneous lesions on the right forearm, left elbow, and right knee was referred after a mild injury. Patient recalled history of similar lessions, delay in wound healing, easy bruising, and recurrent dislocation of wrist from childhood period. According to clinical manifestation, EHLERS-DANLOS syndrome was probable diagnosis for this patient. However due to the disavailability of specific diagnostic test in our country, there was no definite diagnosis. After sending biopsies of skin lesions to the pathology laboratory, SPHEROID nodule of forearm, organized hematoma in the elbow, and KERATINOUS CYST in the right knee were diagnosed.Conclusion: Fat necrosis, traumatic hematoma, hernia of fat into the dermis with calcification and atrophic scars have been reported as various skin lesions in EHLERS-DANLOS syndrome. Three synchronous skin lesions and perforated KERATINOUS CYST with marked inflammatory of foreign body distinguished this case from other similar previous cases. This case is the first case of EHLERS-DANLOS syndrome with mentioned cutaneous dermatologic manifestations in Iran.

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APA: Copy

MOGHIMI, MANSOOR, SOLTANI, HAMID REZA, & AMINI RAD, OMID. (2011). EHLERS-DANLOS SYNDROME WITH MULUSCOID PSEUDO-TUMOR, SPHEROID AND KERATINOUS CYST – REPORT A RARE CASE. JOURNAL OF ISFAHAN MEDICAL SCHOOL (I.U.M.S), 28(113), 787-793. SID. https://sid.ir/paper/50409/en

Vancouver: Copy

MOGHIMI MANSOOR, SOLTANI HAMID REZA, AMINI RAD OMID. EHLERS-DANLOS SYNDROME WITH MULUSCOID PSEUDO-TUMOR, SPHEROID AND KERATINOUS CYST – REPORT A RARE CASE. JOURNAL OF ISFAHAN MEDICAL SCHOOL (I.U.M.S)[Internet]. 2011;28(113):787-793. Available from: https://sid.ir/paper/50409/en

IEEE: Copy

MANSOOR MOGHIMI, HAMID REZA SOLTANI, and OMID AMINI RAD, “EHLERS-DANLOS SYNDROME WITH MULUSCOID PSEUDO-TUMOR, SPHEROID AND KERATINOUS CYST – REPORT A RARE CASE,” JOURNAL OF ISFAHAN MEDICAL SCHOOL (I.U.M.S), vol. 28, no. 113, pp. 787–793, 2011, [Online]. Available: https://sid.ir/paper/50409/en

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