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DermAtlas: HAND - perforating disorder of renal disease and/or diabetes
© 2001-2009, DermAtlas
Image Name: perforating_disorder_renal_1_090401   File Type: jpg
Diagnosis: PERFORATING DISORDER OF RENAL DISEASE AND/OR DIABETES   Category: perforating disorders /
Metabolic disorders
Body Site: hand / arm   Age: 45 years
Contributors: Patrick Keehan, D.O.
Greg Hosler, MD, PhD
   
Description: Multiple hyperkeratotic brown papules and nodules are present on the extensor surfaces of the hands. Some have the appearance of having a central plug. These are non-tender.
Comments: This 45 year old man with end stage renal disease and diabetes mellitus developed scattered hyperkeratotic papules and nodules on the extensor surfaces of his upper extremities. A leson was biopsied to rule out various perforating disorders, such as Kyrle disease or perforating granuloma annulare. Acquired perforating disorders are commonly seen with chronic renal disease, liver disease and diabetes. The nomenclature has evolved, to include Kyrle disease, prforating folliculitis, perforating collagenosis, perforating disorder of renal disease and/or diabetes, etc, and likely represent the same process. This particular biopsied lesion fits with this disease spectrum, as it is clearly a perforating process, but further characterization is limited.
Related Images: perforating_disorder_renal_2_090401 

DermAtlas: Histology - perforating folliculitis
© 2001-2009, DermAtlas
Image Name: perforating_folliculitis_4_080801   File Type: jpg
Diagnosis: PERFORATING FOLLICULITIS /
PERFORATING DISORDER, ACQUIRED
  Category: perforating disorders /
Metabolic disorders
Body Site: arm / leg
hand
  Age: 57 years
Contributors: Patrick Keehan, D.O.
Greg Hosler, MD, PhD
   
Description: At the base of the transepidermal channel, there is a distorted follicle with a broken hair shaft.
Comments: This 57 year old African-American female presented with a 3-month history of eruptive hyperkeratotic papules and nodules on her proximal lower extremities, upper extremities, and dorsal hands. She has a history of renal failure with 7 years of hemodialysis. The patient was placed on topical tretinoin cream 0.05% and has had near complete clearance of these lesions within 6 weeks. The acquired perforating disorders overlap clinically and histologically. The terminology has evolved too, using Kyrle's disease, perforating disease of chronic renal/liver failure and/or diabetes, perforating folliculitis, etc. These may represent the same disease along a spectrum. Perforating collagenosis is separate, with different demographics and often a history of trauma. This case is best classified as perforating folliculitis as the biopsy was classic for this entity. It shows a distorted follicle with an overlying funnel-shaped transepidermal channel.
Related Images: All related Images  perforating_folliculitis_1_080801  perforating_folliculitis_2_080801  perforating_folliculitis_3_080801 

DermAtlas: Histology - perforating folliculitis
© 2001-2009, DermAtlas
Image Name: perforating_folliculitis_3_080801   File Type: jpg
Diagnosis: PERFORATING FOLLICULITIS /
PERFORATING DISORDER, ACQUIRED
  Category: perforating disorders /
Metabolic disorders
Body Site: arm / leg
hand
  Age: 57 years
Contributors: Patrick Keehan, D.O.
Greg Hosler, MD, PhD
   
Description: The central portion of the biopsy has a cystic structure plugged with keratin and basophilic granular material. It is vaguely wedge- or funnel-shaped. A significant inflammatory infiltrate is not seen.
Comments: This 57 year old African-American female presented with a 3-month history of eruptive hyperkeratotic papules and nodules on her proximal lower extremities, upper extremities, and dorsal hands. She has a history of renal failure with 7 years of hemodialysis. The patient was placed on topical tretinoin cream 0.05% and has had near complete clearance of these lesions within 6 weeks. The acquired perforating disorders overlap clinically and histologically. The terminology has evolved too, using Kyrle's disease, perforating disease of chronic renal/liver failure and/or diabetes, perforating folliculitis, etc. These may represent the same disease along a spectrum. Perforating collagenosis is separate, with different demographics and often a history of trauma. This case is best classified as perforating folliculitis as the biopsy was classic for this entity. It shows a distorted follicle with an overlying funnel-shaped transepidermal channel.
Related Images: All related Images  perforating_folliculitis_1_080801  perforating_folliculitis_2_080801  perforating_folliculitis_4_080801 

DermAtlas: ARM - perforating folliculitis
© 2001-2009, DermAtlas
Image Name: perforating_folliculitis_2_080801   File Type: jpg
Diagnosis: PERFORATING FOLLICULITIS /
PERFORATING DISORDER, ACQUIRED
  Category: perforating disorders /
Metabolic disorders
Body Site: arm / leg
hand
  Age: 57 years
Contributors: Patrick Keehan, D.O.
Greg Hosler, MD, PhD
   
Description: A close-up view demonstrates this central keratotic plug with a rim of hypopigmentation.
Comments: This 57 year old African-American female presented with a 3-month history of eruptive hyperkeratotic papules and nodules on her proximal lower extremities, upper extremities, and dorsal hands. She has a history of renal failure with 7 years of hemodialysis. The patient was placed on topical tretinoin cream 0.05% and has had near complete clearance of these lesions within 6 weeks. The acquired perforating disorders overlap clinically and histologically. The terminology has evolved too, using Kyrle's disease, perforating disease of chronic renal/liver failure and/or diabetes, perforating folliculitis, etc. These may represent the same disease along a spectrum. Perforating collagenosis is separate, with different demographics and often a history of trauma. This case is best classified as perforating folliculitis as the biopsy was classic for this entity. It shows a distorted follicle with an overlying funnel-shaped transepidermal channel.
Related Images: All related Images  perforating_folliculitis_1_080801  perforating_folliculitis_3_080801  perforating_folliculitis_4_080801 

DermAtlas: ARM - perforating folliculitis
© 2001-2009, DermAtlas
Image Name: perforating_folliculitis_1_080801   File Type: jpg
Diagnosis: PERFORATING FOLLICULITIS /
PERFORATING DISORDER, ACQUIRED
  Category: perforating disorders /
Metabolic disorders
Body Site: arm / leg
hand
  Age: 57 years
Contributors: Patrick Keehan, D.O.
Greg Hosler, MD, PhD
   
Description: The dorsa of the hands have several discrete hyperkeratotic papules and nodules. These have a central keratotic plug with a rim of hypopigmentation.
Comments: This 57 year old African-American female presented with a 3-month history of eruptive hyperkeratotic papules and nodules on her proximal lower extremities, upper extremities, and dorsal hands. She has a history of renal failure with 7 years of hemodialysis. The patient was placed on topical tretinoin cream 0.05% and has had near complete clearance of these lesions within 6 weeks. The acquired perforating disorders overlap clinically and histologically. The terminology has evolved too, using Kyrle's disease, perforating disease of chronic renal/liver failure and/or diabetes, perforating folliculitis, etc. These may represent the same disease along a spectrum. Perforating collagenosis is separate, with different demographics and often a history of trauma. This case is best classified as perforating folliculitis as the biopsy was classic for this entity. It shows a distorted follicle with an overlying funnel-shaped transepidermal channel.
Related Images: All related Images  perforating_folliculitis_2_080801  perforating_folliculitis_3_080801  perforating_folliculitis_4_080801 

DermAtlas: Histology - granuloma annulare, perforating
© 2001-2009, DermAtlas
Image Name: perforating_granuloma_annulare_1_070417   File Type: jpg
Diagnosis: GRANULOMA ANNULARE, PERFORATING   Category: granulomatous disorder /
perforating disorders
Body Site: elbow   Age: 80 years
Contributors: Alde Carlo Gavino, MD
Greg Hosler, MD, PhD
   
Description: Histologic section of skin shows a prominent perforation through the epidermis, adjacent to which is a focal, palisaded granulomatous dermatitis surrounding a central area of connective tissue degeneration.
Comments: This 80-year old woman presented with a solitary white nodule on her elbow. The perforating variant of granuloma annulare (GA) is characterized by the transepidermal elimination of degenerate (necrobiotic) collagen fibers. It typically presents in the extremities as a localized group of papules with umbilicated crusts. The pathogenesis of GA is currently unknown although immune-mediated or infectious mechanisms are suspect. GA must be differentiated from necrobiosis lipoidica (NLD) and rheumatoid nodule. NLD most commonly occurs on the shins of diabetic patients and is characterized by a deeper and more diffuse degeneration of collagen fibers that commonly lacks mucin deposition. Rheumatoid nodule is also a deep process and is usually markedly fibrotic.
Related Images: All related Images  perforating_granuloma_annulare_3_070417  perforating_granuloma_annulare_2_070417 

DermAtlas: Histology - granuloma annulare, perforating
© 2001-2009, DermAtlas
Image Name: perforating_granuloma_annulare_2_070417   File Type: jpg
Diagnosis: GRANULOMA ANNULARE, PERFORATING   Category: granulomatous disorder /
perforating disorders
Body Site: elbow   Age: 80 years
Contributors: Alde Carlo Gavino, MD
Greg Hosler, MD, PhD
   
Description: Higher power view of the granulomatous process reveals histiocytes, lymphocytes, and fibroblasts radially arranged around a focus of degenerated collagen fibers with central mucin deposition.
Comments: This 80-year old woman presented with a solitary white nodule on her elbow. The perforating variant of granuloma annulare (GA) is characterized by the transepidermal elimination of degenerate (necrobiotic) collagen fibers. It typically presents in the extremities as a localized group of papules with umbilicated crusts. The pathogenesis of GA is currently unknown although immune-mediated or infectious mechanisms are suspect. GA must be differentiated from necrobiosis lipoidica (NLD) and rheumatoid nodule. NLD most commonly occurs on the shins of diabetic patients and is characterized by a deeper and more diffuse degeneration of collagen fibers that commonly lacks mucin deposition. Rheumatoid nodule is also a deep process and is usually markedly fibrotic.
Related Images: All related Images  perforating_granuloma_annulare_3_070417  perforating_granuloma_annulare_1_070417 

DermAtlas: Histology - granuloma annulare, perforating
© 2001-2009, DermAtlas
Image Name: perforating_granuloma_annulare_3_070417   File Type: jpg
Diagnosis: GRANULOMA ANNULARE, PERFORATING   Category: granulomatous disorder /
perforating disorders
Body Site: elbow   Age: 80 years
Contributors: Alde Carlo Gavino, MD
Greg Hosler, MD, PhD
   
Description: Alcian blue stains mucin positively at pH 2.5.
Comments: This 80-year old woman presented with a solitary white nodule on her elbow. The perforating variant of granuloma annulare (GA) is characterized by the transepidermal elimination of degenerate (necrobiotic) collagen fibers. It typically presents in the extremities as a localized group of papules with umbilicated crusts. The pathogenesis of GA is currently unknown although immune-mediated or infectious mechanisms are suspect. GA must be differentiated from necrobiosis lipoidica (NLD) and rheumatoid nodule. NLD most commonly occurs on the shins of diabetic patients and is characterized by a deeper and more diffuse degeneration of collagen fibers that commonly lacks mucin deposition. Rheumatoid nodule is also a deep process and is usually markedly fibrotic.
Related Images: All related Images  perforating_granuloma_annulare_2_070417  perforating_granuloma_annulare_1_070417 

DermAtlas: Histology - chondrodermatitis nodularis helicis
© 2001-2009, DermAtlas
Image Name: chondrodermatitis_1_060227   File Type: jpg
Diagnosis: CHONDRODERMATITIS NODULARIS HELICIS   Category: ulcer /
perforating disorders /
environmental injury
Body Site: ear   Age: 72 years
Contributor: Greg Hosler, MD, PhD    
Description: Histologic sections of skin show central flask-shaped epidermal ulceration with flanking acanthosis. Dysplastic changes are not seen. Helical cartilage is present in the dermis, ascending into the ulcer bed, with central fibrinoid necrosis. A fibrin rich ulcer bed is present, with associated vascular proliferation suggesting granulation tissue. A moderate inflammatory response is also present.
Comments: This 72-year old man developed an ulcer over his left helix. The biopsy was submitted to rule out squamous cell carcinoma. This is a typical presentation and clinical impression for chondrodermatitis nodularis helicis (CNH), as it shares many features with squamous cell carcinoma. The etiology of CNH is unknown, although some authors related it to trauma, perhaps from sleep or cell phone injury. The histology in this case is also classic, showing a benign ulcer with fibrinoid necrosis in the dermis. There is variable inflammation and granulation tissue. An "urban myth" circulates that this entity shows up on dermatology boards with the cartilage conspicuously absent, but this is not confirmed. The presence of cartilage is obviously helpful for the diagnosis, and transelimination of cartilage is a rare treat, but the diagnosis is often made from the characteristic epidermal and dermal alterations.

DermAtlas: Histology - reactive perforating collagenosis
© 2001-2009, DermAtlas
Image Name: reactive_perforating_collagenosis_3_060220   File Type: jpg
Diagnosis: REACTIVE PERFORATING COLLAGENOSIS   Category: perforating disorders /
cutaneous sign of systemic disease /
renal disease
Body Site: leg / trunk   Age: 57 years
Contributor: Greg Hosler, MD, PhD    
Description: A Masson trichrome stain helps confirm the presence of collagen fibers within the elimination channel. Collagen stains an intense blue with this special stain.
Comments: This 57-year-old man had chronic diabetes and renal failure. After being on dialysis for three years, he developed pruritic papules over his legs and trunk. He unfortunately died soon after diagnosis due to his multiple medical conditions. Reactive perforating collagenosis is one of the acquired perforating disorders. Some patients get this following trauma, usually during childhood. The other population with this disorder has a presentation similar to this patient, i.e. chronic renal failure and diabetes. This presentation overlaps with the other acquired perforating disorders such as Kyrle disease. Although distinction is largely academic, reactive perforating collagenosis is characterized by transelimination of collagen through the epidermis, detected by H&E or special stains, as in our case.
Related Images: All related Images  reactive_perforating_collagenosis_1_060220  reactive_perforating_collagenosis_2_060220 

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Bernard A. Cohen, MD, Christoph U. Lehmann, MD

DermAtlas was last updated: Oct-28-2009
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