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 © 2001-2009, DermAtlas | Image Name: | Sclerotylosis_3_071104 | File Type: | jpg | |
| Diagnosis: | HURIEZ SYNDROME / SCLEROTYLOSIS / NAIL DYSTROPHY / KERATODERMA, PALMOPLANTAR | Category: | genodermatosis/genetic disorder / paraneoplastic syndrome / nail disorders | ||
| Body Site: | nail, hand / finger hand / finger | Age: | 56 years | ||
| Contributor: | Eric Ehrsam, MD | ||||
| Description: | sclerotic, atrophic hands and fingers with nail hypoplasia and crusting and erythema on the tops of hands | ||||
| Comments: | A 56-year old man was noted to have acrosclerosis and nail atrophy of fingers. He developed two squamous cell carcinomas on his left index that lead to an amputation. His father and one brother had similar findings. Sclerotylosis or Huriez Syndrome is a genodermatosis characterized by sclerodactyly, nail anomalies, and palmar-plantar keratoderma. Other associated findings include telangiectasias of the lip, flexural contractures of the fifth finger, and poikiloderma of the nasal skin. Patients with Huriez syndrome have a high risk of developing cutaneous squamous cell carcinomas as well as gastrointestinal tumors. (Delaporte E et al: Keratoderma with scleroatrophy of the extremities or sclerotylosis (Huriez syndrome): a reappraisal. Brit. J. Derm. 1995; 133: 409-416) | ||||
| Related Images: | All related Images Sclerotylosis_1_071104 Sclerotylosis_2_071104 | ||||
 © 2001-2009, DermAtlas | Image Name: | Sclerotylosis_2_071104 | File Type: | jpg | |
| Diagnosis: | HURIEZ SYNDROME / SCLEROTYLOSIS / NAIL DYSTROPHY / KERATODERMA, PALMOPLANTAR | Category: | genodermatosis/genetic disorder / paraneoplastic syndrome / nail disorders | ||
| Body Site: | nail, hand / finger hand / finger | Age: | 56 years | ||
| Contributor: | Eric Ehrsam, MD | ||||
| Description: | shiny, atrophic, fibrotic fingers with nail hypoplasia and freckling | ||||
| Comments: | A 56-year old man was noted to have acrosclerosis and nail atrophy of fingers. He developed two squamous cell carcinomas on his left index that lead to an amputation. His father and one brother had similar findings. Sclerotylosis or Huriez Syndrome is a genodermatosis characterized by sclerodactyly, nail anomalies, and palmar-plantar keratoderma. Other associated findings include telangiectasias of the lip, flexural contractures of the fifth finger, and poikiloderma of the nasal skin. Patients with Huriez syndrome have a high risk of developing cutaneous squamous cell carcinomas as well as gastrointestinal tumors. (Delaporte E et al: Keratoderma with scleroatrophy of the extremities or sclerotylosis (Huriez syndrome): a reappraisal. Brit. J. Derm. 1995; 133: 409-416) | ||||
| Related Images: | All related Images Sclerotylosis_1_071104 Sclerotylosis_3_071104 | ||||
 © 2001-2009, DermAtlas | Image Name: | Sclerotylosis_1_071104 | File Type: | jpg | |
| Diagnosis: | HURIEZ SYNDROME / SCLEROTYLOSIS / NAIL DYSTROPHY / KERATODERMA, PALMOPLANTAR | Category: | genodermatosis/genetic disorder / paraneoplastic syndrome / nail disorders | ||
| Body Site: | nail, hand / finger hand / finger | Age: | 56 years | ||
| Contributor: | Eric Ehrsam, MD | ||||
| Description: | sclerotic, atrophic hands and fingers with nail hypoplasia and crusting and erythema on the tops of hands | ||||
| Comments: | A 56-year old man was noted to have acrosclerosis and nail atrophy of fingers. He developed two squamous cell carcinomas on his left index that lead to an amputation. His father and one brother had similar findings. Sclerotylosis or Huriez Syndrome is a genodermatosis characterized by sclerodactyly, nail anomalies, and palmar-plantar keratoderma. Other associated findings include telangiectasias of the lip, flexural contractures of the fifth finger, and poikiloderma of the nasal skin. Patients with Huriez syndrome have a high risk of developing cutaneous squamous cell carcinomas as well as gastrointestinal tumors. (Delaporte E et al: Keratoderma with scleroatrophy of the extremities or sclerotylosis (Huriez syndrome): a reappraisal. Brit. J. Derm. 1995; 133: 409-416) | ||||
| Related Images: | All related Images Sclerotylosis_2_071104 Sclerotylosis_3_071104 | ||||
 © 2001-2009, DermAtlas | Image Name: | scleromyxedema_1_050507 | File Type: | jpg | |
| Diagnosis: | SCLEROMYXEDEMA | Category: | paraneoplastic syndrome | ||
| Body Site: | total body / chest neck | Age: | 47 years | ||
| Contributor: | Bernard Cohen, MD | ||||
| Description: | diffuse waxy infiltrative 2-3 mm papules | ||||
| Comments: | This 47-year-old woman had a 15 year history of progressive infiltrative confluent papules on the chest, abdomen, back, hands, and forearms. Although she felt well, she had chronic hepatitis C, and serum protein electrophoresis revealed a IgG lambda type paraproteinemia. | ||||
| Related Images: | All related Images scleromyxedema_8_050507 scleromyxedema_7_050507 scleromyxedema_6_050507 scleromyxedema_5_050507 scleromyxedema_4_050507 scleromyxedema_3_050507 scleromyxedema_2_050507 | ||||
 © 2001-2009, DermAtlas | Image Name: | scleromyxedema_2_050507 | File Type: | jpg | |
| Diagnosis: | SCLEROMYXEDEMA | Category: | paraneoplastic syndrome | ||
| Body Site: | total body / chest neck | Age: | 47 years | ||
| Contributor: | Bernard Cohen, MD | ||||
| Description: | diffuse waxy infiltrative 2-3 mm papules | ||||
| Comments: | This 47-year-old woman had a 15 year history of progressive infiltrative confluent papules on the chest, abdomen, back, hands, and forearms. Although she felt well, she had chronic hepatitis C, and serum protein electrophoresis revealed a IgG lambda type paraproteinemia. | ||||
| Related Images: | All related Images scleromyxedema_8_050507 scleromyxedema_7_050507 scleromyxedema_6_050507 scleromyxedema_5_050507 scleromyxedema_4_050507 scleromyxedema_3_050507 scleromyxedema_1_050507 | ||||
 © 2001-2009, DermAtlas | Image Name: | scleromyxedema_3_050507 | File Type: | jpg | |
| Diagnosis: | SCLEROMYXEDEMA | Category: | paraneoplastic syndrome | ||
| Body Site: | total body / chest abdomen | Age: | 47 years | ||
| Contributor: | Bernard Cohen, MD | ||||
| Description: | diffuse waxy infiltrative 2-3 mm papules | ||||
| Comments: | This 47-year-old woman had a 15 year history of progressive infiltrative confluent papules on the chest, abdomen, back, hands, and forearms. Although she felt well, she had chronic hepatitis C, and serum protein electrophoresis revealed a IgG lambda type paraproteinemia. | ||||
| Related Images: | All related Images scleromyxedema_8_050507 scleromyxedema_7_050507 scleromyxedema_6_050507 scleromyxedema_5_050507 scleromyxedema_4_050507 scleromyxedema_2_050507 scleromyxedema_1_050507 | ||||
 © 2001-2009, DermAtlas | Image Name: | scleromyxedema_4_050507 | File Type: | jpg | |
| Diagnosis: | SCLEROMYXEDEMA | Category: | paraneoplastic syndrome | ||
| Body Site: | total body / hand wrist | Age: | 47 years | ||
| Contributor: | Bernard Cohen, MD | ||||
| Description: | diffuse waxy infiltrative 2-3 mm papules | ||||
| Comments: | This 47-year-old woman had a 15 year history of progressive infiltrative confluent papules on the chest, abdomen, back, hands, and forearms. Although she felt well, she had chronic hepatitis C, and serum protein electrophoresis revealed a IgG lambda type paraproteinemia. | ||||
| Related Images: | All related Images scleromyxedema_8_050507 scleromyxedema_7_050507 scleromyxedema_6_050507 scleromyxedema_5_050507 scleromyxedema_3_050507 scleromyxedema_2_050507 scleromyxedema_1_050507 | ||||
 © 2001-2009, DermAtlas | Image Name: | scleromyxedema_5_050507 | File Type: | jpg | |
| Diagnosis: | SCLEROMYXEDEMA | Category: | paraneoplastic syndrome | ||
| Body Site: | total body / wrist | Age: | 47 years | ||
| Contributor: | Bernard Cohen, MD | ||||
| Description: | diffuse waxy infiltrative 2-3 mm papules | ||||
| Comments: | This 47-year-old woman had a 15 year history of progressive infiltrative confluent papules on the chest, abdomen, back, hands, and forearms. Although she felt well, she had chronic hepatitis C, and serum protein electrophoresis revealed a IgG lambda type paraproteinemia. | ||||
| Related Images: | All related Images scleromyxedema_8_050507 scleromyxedema_7_050507 scleromyxedema_6_050507 scleromyxedema_4_050507 scleromyxedema_3_050507 scleromyxedema_2_050507 scleromyxedema_1_050507 | ||||
 © 2001-2009, DermAtlas | Image Name: | scleromyxedema_6_050507 | File Type: | jpg | |
| Diagnosis: | SCLEROMYXEDEMA | Category: | paraneoplastic syndrome | ||
| Body Site: | total body / forearm arm | Age: | 47 years | ||
| Contributor: | Bernard Cohen, MD | ||||
| Description: | diffuse waxy infiltrative 2-3 mm papules | ||||
| Comments: | This 47-year-old woman had a 15 year history of progressive infiltrative confluent papules on the chest, abdomen, back, hands, and forearms. Although she felt well, she had chronic hepatitis C, and serum protein electrophoresis revealed a IgG lambda type paraproteinemia. | ||||
| Related Images: | All related Images scleromyxedema_8_050507 scleromyxedema_7_050507 scleromyxedema_5_050507 scleromyxedema_4_050507 scleromyxedema_3_050507 scleromyxedema_2_050507 scleromyxedema_1_050507 | ||||
 © 2001-2009, DermAtlas | Image Name: | scleromyxedema_7_050507 | File Type: | jpg | |
| Diagnosis: | SCLEROMYXEDEMA | Category: | paraneoplastic syndrome | ||
| Body Site: | total body / forearm arm | Age: | 47 years | ||
| Contributor: | Bernard Cohen, MD | ||||
| Description: | diffuse waxy infiltrative 2-3 mm papules | ||||
| Comments: | This 47-year-old woman had a 15 year history of progressive infiltrative confluent papules on the chest, abdomen, back, hands, and forearms. Although she felt well, she had chronic hepatitis C, and serum protein electrophoresis revealed a IgG lambda type paraproteinemia. | ||||
| Related Images: | All related Images scleromyxedema_8_050507 scleromyxedema_6_050507 scleromyxedema_5_050507 scleromyxedema_4_050507 scleromyxedema_3_050507 scleromyxedema_2_050507 scleromyxedema_1_050507 | ||||
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