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Gorlin-goltz syndrome: a rare case.

Ganguly S, Jaykar KC, Kumar R, Jha AK, Banerjee PK - Indian J Dermatol (2015 Mar-Apr)

Bottom Line: Gorlin-Goltz syndrome or nevoid basal cell carcinoma syndrome is characterized by multiple basocellular epitheliomas, keratocysts in the jaws, bifid ribs, palmar and/or plantar pits and ectopic calcifications of the falx cerebri.We describe a case of Gorlin-Goltz syndrome illustrating the importance of a thorough examination including the examination of palms and soles and detailed investigations in a patient having lesions suggestive of basal cell carcinoma and multiple naevi.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Venereology and Leprosy, Pondicherry Institute of Medical Sciences, Pondicherry, Puducherry, India.

ABSTRACT
Gorlin-Goltz syndrome or nevoid basal cell carcinoma syndrome is characterized by multiple basocellular epitheliomas, keratocysts in the jaws, bifid ribs, palmar and/or plantar pits and ectopic calcifications of the falx cerebri. We describe a case of Gorlin-Goltz syndrome illustrating the importance of a thorough examination including the examination of palms and soles and detailed investigations in a patient having lesions suggestive of basal cell carcinoma and multiple naevi.

No MeSH data available.


Related in: MedlinePlus

Groups of basaloid cells (H and E, ×40)
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Figure 6: Groups of basaloid cells (H and E, ×40)

Mentions: A 65-years-old male patient presented to the dermatology out-patient department with an ulcerated swelling on the forehead. According to the patient he had a dark colored elevated swelling on the forehead for the past 20 years, which has slowly increased in size over the time and became ulcerated for the past 3 months. On examination there was a single round ulcerated plaque on the forehead at the hair margin with elevated edges and pale granulation tissue on the floor [Figure 1]. A thorough examination revealed multiple hyperpigmented papules with smooth surfaces over face, post auricular area [Figure 2] and back. A similar larger hyperpigmented plaque was seen on the back and it showed superficial ulceration [Figure 3]. Both the palms showed multiple pits [Figure 4]. There was no history of similar complaints in his parents. Examination of the oral cavity revealed a single nodule on the right end of upper gingiva, which turned out to be an odontogenic cyst [Figure 5]. A skin biopsy was done from the edge of the ulcer and it showed groups of basaloid cells [Figure 6] and keratohyalin cysts [Figure 7]. Radiographs of chest (AP view), spine and skull were done and failed to reveal any abnormality. Based on the above findings a diagnosis of Gorlin-Goltz syndrome or basal cell naevus syndrome (BCNS) was made. The patient was counselled and referred to the surgery and dental department for further treatment.


Gorlin-goltz syndrome: a rare case.

Ganguly S, Jaykar KC, Kumar R, Jha AK, Banerjee PK - Indian J Dermatol (2015 Mar-Apr)

Groups of basaloid cells (H and E, ×40)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4372962&req=5

Figure 6: Groups of basaloid cells (H and E, ×40)
Mentions: A 65-years-old male patient presented to the dermatology out-patient department with an ulcerated swelling on the forehead. According to the patient he had a dark colored elevated swelling on the forehead for the past 20 years, which has slowly increased in size over the time and became ulcerated for the past 3 months. On examination there was a single round ulcerated plaque on the forehead at the hair margin with elevated edges and pale granulation tissue on the floor [Figure 1]. A thorough examination revealed multiple hyperpigmented papules with smooth surfaces over face, post auricular area [Figure 2] and back. A similar larger hyperpigmented plaque was seen on the back and it showed superficial ulceration [Figure 3]. Both the palms showed multiple pits [Figure 4]. There was no history of similar complaints in his parents. Examination of the oral cavity revealed a single nodule on the right end of upper gingiva, which turned out to be an odontogenic cyst [Figure 5]. A skin biopsy was done from the edge of the ulcer and it showed groups of basaloid cells [Figure 6] and keratohyalin cysts [Figure 7]. Radiographs of chest (AP view), spine and skull were done and failed to reveal any abnormality. Based on the above findings a diagnosis of Gorlin-Goltz syndrome or basal cell naevus syndrome (BCNS) was made. The patient was counselled and referred to the surgery and dental department for further treatment.

Bottom Line: Gorlin-Goltz syndrome or nevoid basal cell carcinoma syndrome is characterized by multiple basocellular epitheliomas, keratocysts in the jaws, bifid ribs, palmar and/or plantar pits and ectopic calcifications of the falx cerebri.We describe a case of Gorlin-Goltz syndrome illustrating the importance of a thorough examination including the examination of palms and soles and detailed investigations in a patient having lesions suggestive of basal cell carcinoma and multiple naevi.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Venereology and Leprosy, Pondicherry Institute of Medical Sciences, Pondicherry, Puducherry, India.

ABSTRACT
Gorlin-Goltz syndrome or nevoid basal cell carcinoma syndrome is characterized by multiple basocellular epitheliomas, keratocysts in the jaws, bifid ribs, palmar and/or plantar pits and ectopic calcifications of the falx cerebri. We describe a case of Gorlin-Goltz syndrome illustrating the importance of a thorough examination including the examination of palms and soles and detailed investigations in a patient having lesions suggestive of basal cell carcinoma and multiple naevi.

No MeSH data available.


Related in: MedlinePlus