 | Your DermAtlas query returned at least 10 ImagesClick on the IMAGE to see an enlargement.The number of images has been limited to 10 per page. |
Next Result Set  |
| | Your DermAtlas query returned at least 10 ImagesClick on the IMAGE to see an enlargement.The number of images has been limited to 10 per page. |
| Next Result Set |
 © 2001-05, DermAtlas | Image Name: | cartilage_hair_hypoplasia_2_0108 | File Type: | jpg | |
| Diagnosis: | GRANULOMATOUS REACTION / CARTILAGE HAIR HYPOPLASIA / IMMUNODEFICIENCY, HEREDITARY | Category: | granulomatous disorder / genodermatosis/genetic disorder | ||
| Body Site: | elbow / arm | Age: | 14 years | ||
| Contributor: | Albert Yan, MD | ||||
| Description: | annular violaceous rubbery plaque with pink border | ||||
| Comments: | This 14-year-old boy with cartilage hair hypoplasia (CHH), Blackfan-Diamond anemia, and chronic immune deficiency developed disseminated flat warts and granulomatous violaceous rubbery plaques on both elbows. Cartilage hair hypoplasia (CHH) is a form of short-limbed dwarfism characterized by T-cell immunodeficiency and fine, sparse hair. The genetic deficiency has been mapped to chromosome 9p13 and some patients have mutations in the RNA component of ribonuclease mitochondrial RNA processing (RNAse MRP). Along with hematologic abnormalities and immune deficiency, clinical manifestations may include short hands, brachydactyly, fine blond hair, notched incisors, and microdontia. Although severe viral infections and opportunistic infections (Candida, Pneumocystis, and CMV) have been associated with CHH, epidermodysplasia verruciformis (EDV) has not been previously reported among these patients. EDV is an autosomal recessive (and sometimes X-linked) inherited disease associated with a specific immune defect in the recognition of certain human papilloma viruses (HPV). The warty growths typically begin in the first 5 years of life on the hands, trunk and face but can be delayed until puberty in approximately 20% of cases. Typically a persistent and chronic infection, residence of tumorigenic HPV strains may predispose to malignant transformation to squamous cell carcinoma especially in the 3rd to 5th decade. Cancer risk is markedly increased in the HPV strains 5, 8 and 47 (although reported I many other strains) due to there ability to express E6 and E7 despite immune attack. Cancers develop mostly in sun-exposed areas indicating that both specific HPV strains and damaging UV radiation is needed to create tumors. Therapy for EDV involves avoidance of UV and X-ray radiation. Systemic and topical retinoids as well as interferon-alpha have been reported with variable success. Granulomatous plaques (often of a sarcoidal nature) have been reported in immunodeficiency diseases such as common variable immunodeficiency (up to 10% of patients) and ataxia-telangiectasia. It is hypothesized that poor T-cell immune response and not true sarcoidosis may be responsible for the granuloma formation. By contrast, patients with X-linked agammaglobulinemia, a disorder associated more with B-cell dysfunction rather than T-cells, do not develop these granulomas. It is our suspicion that the granulomatous plaques of cartilage-hair hypoplasia may likewise result from the T-cell dysfunction described above. References: 1. Majewski S, Jablonska S: Epidermodysplasia verruciformis as a model of human papillomavirus-induced genetic cancer of the skin. Arch Dermatol. 1995;131(11):1312-8. 2. Mechanic LJ. Dikman S. Cunningham-Rundles C. Granulomatous disease in common variable immunodeficiency. Ann Int Med. 1997;127(8 Pt 1):613-7. 3. Murakawa GJ, McCalmot T, Frieden IJ. Chronic plaques in a patient with ataxia telangiectasia. Cutaneous granulomatous lesions in a patient with AT. Archives of Dermatology. 1998;134(9):1145,1148. 4. Ridanpaa M, Suslisalo T, de la Chapelle A: Genetic and physical mapping of the cartilage-hair hypoplasia locus on 9p13. Am J Hum Genet. 1995;57:A201. | ||||
| Related Images: | All related Images cartilage_hair_hypoplasia_3_0108 cartilage_hair_hypoplasia_1_0108 | ||||
 © 2001-05, DermAtlas | Image Name: | leishmaniasis_1_031130 | File Type: | jpg | |
| Diagnosis: | LEISHMANIASIS / GRANULOMATOUS REACTION / ULCER | Category: | infections and infestations / granulomatous disorder / ulcer | ||
| Body Site: | hand / wrist | Age: | 38 years | ||
| Contributor: | Bernard Cohen, MD | ||||
| Description: | indurated ulcerated plaque with several more proximal nodules | ||||
| Comments: | Several months after returning from a trip to Costa Rica, this healthy 38-year-old man deveoped a painless violaceous plaque on his right hand. The first lesion ulcerated and several new nodules appeared on the right wrist and forearm in a sporotrichoid pattern. His 8-year-old daughter developed a similar lesion on her arm. Polymerase chain reaction using species-specific primers confirmed the diagnosis of infection with Leishmania mexicana. | ||||
 © 2001-05, DermAtlas | Image Name: | sarcoidosis_1_031001 | File Type: | jpg | |
| Diagnosis: | SARCOIDOSIS / GRANULOMATOUS REACTION | Category: | lumps & bumps (plaques, nodules, tumors) / granulomatous disorder | ||
| Body Site: | arm | Age: | 45 years | ||
| Contributor: | Bernard Cohen, MD | ||||
| Description: | diffuse symmetric fibrotic skin colored papules | ||||
| Comments: | This 45-year-old woman developed multiple firm 3-6 mm papules on her arms, legs, and trunk. The lesions were not symptomatic, and she had no evidence of systemic disease. A skin biopsy showed non-caseating granulomas and fibrosis. | ||||
| Related Images: | All related Images sarcoidosis_4_031001 sarcoidosis_3_031001 sarcoidosis_2_031001 | ||||
 © 2001-05, DermAtlas | Image Name: | sarcoidosis_2_031001 | File Type: | jpg | |
| Diagnosis: | SARCOIDOSIS / GRANULOMATOUS REACTION | Category: | lumps & bumps (plaques, nodules, tumors) / granulomatous disorder | ||
| Body Site: | hand | Age: | 45 years | ||
| Contributor: | Bernard Cohen, MD | ||||
| Description: | diffuse symmetric fibrotic skin colored papules | ||||
| Comments: | This 45-year-old woman developed multiple firm 3-6 mm papules on her arms, legs, and trunk. The lesions were not symptomatic, and she had no evidence of systemic disease. A skin biopsy showed non-caseating granulomas and fibrosis. | ||||
| Related Images: | All related Images sarcoidosis_4_031001 sarcoidosis_3_031001 sarcoidosis_1_031001 | ||||
 © 2001-05, DermAtlas | Image Name: | sarcoidosis_3_031001 | File Type: | jpg | |
| Diagnosis: | SARCOIDOSIS / GRANULOMATOUS REACTION | Category: | lumps & bumps (plaques, nodules, tumors) / granulomatous disorder | ||
| Body Site: | arm | Age: | 45 years | ||
| Contributor: | Bernard Cohen, MD | ||||
| Description: | diffuse symmetric fibrotic skin colored papules | ||||
| Comments: | This 45-year-old woman developed multiple firm 3-6 mm papules on her arms, legs, and trunk. The lesions were not symptomatic, and she had no evidence of systemic disease. A skin biopsy showed non-caseating granulomas and fibrosis. | ||||
| Related Images: | All related Images sarcoidosis_4_031001 sarcoidosis_2_031001 sarcoidosis_1_031001 | ||||
 © 2001-05, DermAtlas | Image Name: | sarcoidosis_4_031001 | File Type: | jpg | |
| Diagnosis: | SARCOIDOSIS / GRANULOMATOUS REACTION | Category: | lumps & bumps (plaques, nodules, tumors) / granulomatous disorder | ||
| Body Site: | arm | Age: | 45 years | ||
| Contributor: | Bernard Cohen, MD | ||||
| Description: | diffuse symmetric fibrotic skin colored papules | ||||
| Comments: | This 45-year-old woman developed multiple firm 3-6 mm papules on her arms, legs, and trunk. The lesions were not symptomatic, and she had no evidence of systemic disease. A skin biopsy showed non-caseating granulomas and fibrosis. | ||||
| Related Images: | All related Images sarcoidosis_3_031001 sarcoidosis_2_031001 sarcoidosis_1_031001 | ||||
 © 2001-05, DermAtlas | Image Name: | granulomatous_reaction_6_031008 | File Type: | jpg | |
| Diagnosis: | ATAXIA-TELANGIECTASIA / GRANULOMATOUS REACTION / IMMUNODEFICIENCY, HEREDITARY | Category: | granulomatous disorder | ||
| Body Site: | ankle / foot | Age: | 12 years | ||
| Contributor: | Hisham Abdel Hafez, MD | ||||
| Description: | annular and round violaceous plaques with central scale, fibrosis and atrophy | ||||
| Comments: | Two years ago this 12-year-old boy with ataxia-telangiectasia developed painful violaceous plaques on hig right hand and wrist. The lesions slowly progressed and he developed new plaques on his left ankle a year ago. Multiple biopsies have shown granulomatous changes, but special stains and cultures for bacteria, fungi, and mycobacteria have been negative. Despite treatment with antibiotics and topical steroids, the eruption has continued to progress. The contributor is seeking suggestions for further evaluation and treatment. | ||||
| Related Images: | All related Images granulomatous_reaction_1_031008 granulomatous_reaction_2_031008 granulomatous_reaction_3_031008 granulomatous_reaction_4_031008 granulomatous_reaction_5_031008 | ||||
 © 2001-05, DermAtlas | Image Name: | granulomatous_reaction_5_031008 | File Type: | jpg | |
| Diagnosis: | ATAXIA-TELANGIECTASIA / GRANULOMATOUS REACTION / IMMUNODEFICIENCY, HEREDITARY | Category: | granulomatous disorder | ||
| Body Site: | ankle / foot | Age: | 12 years | ||
| Contributor: | Hisham Abdel Hafez, MD | ||||
| Description: | annular and round violaceous plaques with central scale, fibrosis and atrophy | ||||
| Comments: | Two years ago this 12-year-old boy with ataxia-telangiectasia developed painful violaceous plaques on hig right hand and wrist. The lesions slowly progressed and he developed new plaques on his left ankle a year ago. Multiple biopsies have shown granulomatous changes, but special stains and cultures for bacteria, fungi, and mycobacteria have been negative. Despite treatment with antibiotics and topical steroids, the eruption has continued to progress. The contributor is seeking suggestions for further evaluation and treatment. | ||||
| Related Images: | All related Images granulomatous_reaction_1_031008 granulomatous_reaction_2_031008 granulomatous_reaction_3_031008 granulomatous_reaction_4_031008 granulomatous_reaction_5_031008 granulomatous_reaction_6_031008 | ||||
 © 2001-05, DermAtlas | Image Name: | granulomatous_reaction_4_031008 | File Type: | jpg | |
| Diagnosis: | ATAXIA-TELANGIECTASIA / GRANULOMATOUS REACTION / IMMUNODEFICIENCY, HEREDITARY | Category: | granulomatous disorder | ||
| Body Site: | hand / wrist arm | Age: | 12 years | ||
| Contributor: | Hisham Abdel Hafez, MD | ||||
| Description: | annular and round violaceous plaques with central scale, fibrosis and atrophy | ||||
| Comments: | Two years ago this 12-year-old boy with ataxia-telangiectasia developed painful violaceous plaques on hig right hand and wrist. The lesions slowly progressed and he developed new plaques on his left ankle a year ago. Multiple biopsies have shown granulomatous changes, but special stains and cultures for bacteria, fungi, and mycobacteria have been negative. Despite treatment with antibiotics and topical steroids, the eruption has continued to progress. The contributor is seeking suggestions for further evaluation and treatment. | ||||
| Related Images: | All related Images granulomatous_reaction_1_031008 granulomatous_reaction_2_031008 granulomatous_reaction_3_031008 granulomatous_reaction_5_031008 granulomatous_reaction_6_031008 | ||||
 © 2001-05, DermAtlas | Image Name: | granulomatous_reaction_3_031008 | File Type: | jpg | |
| Diagnosis: | ATAXIA-TELANGIECTASIA / GRANULOMATOUS REACTION / IMMUNODEFICIENCY, HEREDITARY | Category: | granulomatous disorder | ||
| Body Site: | ankle / foot | Age: | 12 years | ||
| Contributor: | Hisham Abdel Hafez, MD | ||||
| Description: | annular and round violaceous plaques with central scale, fibrosis and atrophy | ||||
| Comments: | Two years ago this 12-year-old boy with ataxia-telangiectasia developed painful violaceous plaques on hig right hand and wrist. The lesions slowly progressed and he developed new plaques on his left ankle a year ago. Multiple biopsies have shown granulomatous changes, but special stains and cultures for bacteria, fungi, and mycobacteria have been negative. Despite treatment with antibiotics and topical steroids, the eruption has continued to progress. The contributor is seeking suggestions for further evaluation and treatment. | ||||
| Related Images: | All related Images granulomatous_reaction_1_031008 granulomatous_reaction_2_031008 granulomatous_reaction_4_031008 granulomatous_reaction_5_031008 granulomatous_reaction_6_031008 | ||||
| Next Result Set |
© DermAtlas, Johns Hopkins University; 2000-2005
Bernard A. Cohen, MD, Christoph U. Lehmann, MD
Return to the DermAtlas Home Page
Link directly to this page: http://DermAtlas.med.jhmi.edu/derm/result.cfm?Diagnosis=1284877337