DermAtlas: Online Dermatology Image Library dermatology image,pityriasis rubra pilaris,erythroderma

© 2001-2010, DermAtlas	*Image Name:*	pityriasis_rubra_pilaris_1_050112	*File Type:*	jpg
	*Diagnosis:*	PITYRIASIS RUBRA PILARIS	*Category:*	papulosquamous eruptions / genodermatosis/genetic disorder
	*Body Site:*	chest / abdomen arm / shoulder	*Age:*	12 years
	*Contributor:*	Albert Yan, MD
	*Description:*	widespread symmetric salmon-colored plaques with islands of sparing
	*Comments:*	This 12-year-old boy had a 7 month history of an asymptomatic, erythematous, scaly eruption that began on the trunk and spread to the face, neck, and extremities. He did not improve with topical steroids and was begun on topical tacrolimus ointment. Family history was negative for skin disease. A skin biopsy from his left arm showed acanthosis with overlying alternating foci of ortho- and parakeratosis. Minimal lymphocytic infiltrate was also noted in the upper dermis. Pityriasis Rubra Pilaris (PRP) is a papulosquamous disorder of unknown etiology characterized by abnormal epidermal hyperproliferation. It was first described by Tarral in 1828 and has since classified into 6 categories on the basis of presentation and history: classic adult type, atypical adult type, classic juvenile type, circumscribed juvenile type, atypical juvenile type, and HIV-associated type. Although the majority of cases appear to be acquired, a rare early-onset autosomal dominant inheritance pattern has been reported with onset during early childhood. Two peaks of onset are noted for the acquired form – during the 1st and 5th decades of life. The eruption usually begins on the head and neck with eventual involvement of the entire body. Follicular hyperkeratotic papules are prominent, progress in a cephalocaudal pattern, and coalesce into orange-red or salmon-colored scaling plaques with well defined borders and “islands of sparing.” Palmoplantar involvement characterized by thickened yellow keratoderma is frequently seen. In addition, scalp, nail and oral mucosal involvement may also be noted. Approximately 80 percent of patients clear spontaneously within several years, but the course can be quite variable. Childhood PRP is very difficult to treat and has been reported to be poorly responsive to topical steroids and phototherapy. The atypical juvenile variant type occurs during the first few years of life and exhibits a more chronic course. Oral retinoid therapy has proven to be successful in a number of cases. However, given the typically limited disease course, such therapy is generally reserved for persistent cases. References: 1. Albert MR, Mackool BT. Pityriasis rubra pilaris. Int J Dermatol. 1999, 38:1-11. 2. Allison DS, el-Azhary RA, Calobrisi SD. Pityriasis rubra pilaris in children. J Am Acad Dermatol. 2001;47:3386-9. 3. Gelmetti C, Schiuma AA, Cerri D, Gianotti F. Pityriasis rubra pilaris in childhood: a long-term study of 29 cases. Pediatr Dermatol. 1986; 3:446-51. 4. Seghal VN, Jain S, Kumar S, et al. Familial pityriasis rubra pilaris (adult classic-I): a report of three cases in a single family. Skin Med. 2002 1:161-4.
	*Related Images:*	pityriasis_rubra_pilaris_2_050112

© 2001-2010, DermAtlas	*Image Name:*	pityriasis_rubra_pilaris_2_050112	*File Type:*	jpg
	*Diagnosis:*	PITYRIASIS RUBRA PILARIS	*Category:*	papulosquamous eruptions / genodermatosis/genetic disorder
	*Body Site:*	knee / leg	*Age:*	12 years
	*Contributor:*	Albert Yan, MD
	*Description:*	widespread symmetric salmon-colored plaques with islands of sparing
	*Comments:*	This 12-year-old boy had a 7 month history of an asymptomatic, erythematous, scaly eruption that began on the trunk and spread to the face, neck, and extremities. He did not improve with topical steroids and was begun on topical tacrolimus ointment. Family history was negative for skin disease. A skin biopsy from his left arm showed acanthosis with overlying alternating foci of ortho- and parakeratosis. Minimal lymphocytic infiltrate was also noted in the upper dermis. Pityriasis Rubra Pilaris (PRP) is a papulosquamous disorder of unknown etiology characterized by abnormal epidermal hyperproliferation. It was first described by Tarral in 1828 and has since classified into 6 categories on the basis of presentation and history: classic adult type, atypical adult type, classic juvenile type, circumscribed juvenile type, atypical juvenile type, and HIV-associated type. Although the majority of cases appear to be acquired, a rare early-onset autosomal dominant inheritance pattern has been reported with onset during early childhood. Two peaks of onset are noted for the acquired form – during the 1st and 5th decades of life. The eruption usually begins on the head and neck with eventual involvement of the entire body. Follicular hyperkeratotic papules are prominent, progress in a cephalocaudal pattern, and coalesce into orange-red or salmon-colored scaling plaques with well defined borders and “islands of sparing.” Palmoplantar involvement characterized by thickened yellow keratoderma is frequently seen. In addition, scalp, nail and oral mucosal involvement may also be noted. Approximately 80 percent of patients clear spontaneously within several years, but the course can be quite variable. Childhood PRP is very difficult to treat and has been reported to be poorly responsive to topical steroids and phototherapy. The atypical juvenile variant type occurs during the first few years of life and exhibits a more chronic course. Oral retinoid therapy has proven to be successful in a number of cases. However, given the typically limited disease course, such therapy is generally reserved for persistent cases. References: 1. Albert MR, Mackool BT. Pityriasis rubra pilaris. Int J Dermatol. 1999, 38:1-11. 2. Allison DS, el-Azhary RA, Calobrisi SD. Pityriasis rubra pilaris in children. J Am Acad Dermatol. 2001;47:3386-9. 3. Gelmetti C, Schiuma AA, Cerri D, Gianotti F. Pityriasis rubra pilaris in childhood: a long-term study of 29 cases. Pediatr Dermatol. 1986; 3:446-51. 4. Seghal VN, Jain S, Kumar S, et al. Familial pityriasis rubra pilaris (adult classic-I): a report of three cases in a single family. Skin Med. 2002 1:161-4.
	*Related Images:*	pityriasis_rubra_pilaris_1_050112

© 2001-2010, DermAtlas	*Image Name:*	pityriasis_rubra_pilaris_4_040726	*File Type:*	jpg
	*Diagnosis:*	PITYRIASIS RUBRA PILARIS	*Category:*	papulosquamous eruptions
	*Body Site:*	leg	*Age:*	65 years
	*Contributor:*	Shahbaz A. Janjua, MD
	*Description:*	symmetric confluent erythematous scaly plaques
	*Comments:*	This 65-year-old man was evaluated for a 2 month history of a mildly pruritic eruption comprised of symmetric confluent reddish-orange scaly plaques with distinct areas of sparing on the trunk and extremities and a palmoplantar keratoderma.
	*Related Images:*	All related Images pityriasis_rubra_pilaris_1_040726 pityriasis_rubra_pilaris_2_040726 pityriasis_rubra_pilaris_3_040726

© 2001-2010, DermAtlas	*Image Name:*	pityriasis_rubra_pilaris_3_040726	*File Type:*	jpg
	*Diagnosis:*	PITYRIASIS RUBRA PILARIS	*Category:*	papulosquamous eruptions
	*Body Site:*	foot	*Age:*	65 years
	*Contributor:*	Shahbaz A. Janjua, MD
	*Description:*	symmetric confluent erythematous scaly plaques
	*Comments:*	This 65-year-old man was evaluated for a 2 month history of a mildly pruritic eruption comprised of symmetric confluent reddish-orange scaly plaques with distinct areas of sparing on the trunk and extremities and a palmoplantar keratoderma.
	*Related Images:*	All related Images pityriasis_rubra_pilaris_1_040726 pityriasis_rubra_pilaris_2_040726 pityriasis_rubra_pilaris_4_040726

© 2001-2010, DermAtlas	*Image Name:*	pityriasis_rubra_pilaris_2_040726	*File Type:*	jpg
	*Diagnosis:*	PITYRIASIS RUBRA PILARIS	*Category:*	papulosquamous eruptions
	*Body Site:*	thigh	*Age:*	65 years
	*Contributor:*	Shahbaz A. Janjua, MD
	*Description:*	symmetric confluent erythematous scaly plaques
	*Comments:*	This 65-year-old man was evaluated for a 2 month history of a mildly pruritic eruption comprised of symmetric confluent reddish-orange scaly plaques with distinct areas of sparing on the trunk and extremities and a palmoplantar keratoderma.
	*Related Images:*	All related Images pityriasis_rubra_pilaris_1_040726 pityriasis_rubra_pilaris_3_040726 pityriasis_rubra_pilaris_4_040726

© 2001-2010, DermAtlas	*Image Name:*	pityriasis_rubra_pilaris_1_040726	*File Type:*	jpg
	*Diagnosis:*	PITYRIASIS RUBRA PILARIS	*Category:*	papulosquamous eruptions
	*Body Site:*	trunk / arm	*Age:*	65 years
	*Contributor:*	Shahbaz A. Janjua, MD
	*Description:*	symmetric confluent erythematous scaly plaques
	*Comments:*	This 65-year-old man was evaluated for a 2 month history of a mildly pruritic eruption comprised of symmetric confluent reddish-orange scaly plaques with distinct areas of sparing on the trunk and extremities and a palmoplantar keratoderma.
	*Related Images:*	All related Images pityriasis_rubra_pilaris_2_040726 pityriasis_rubra_pilaris_3_040726 pityriasis_rubra_pilaris_4_040726

© 2001-2010, DermAtlas	*Image Name:*	Pityriasis_rubra_pilaris_1_031117	*File Type:*	jpg
	*Diagnosis:*	PITYRIASIS RUBRA PILARIS / ERYTHRODERMA	*Category:*	papulosquamous eruptions
	*Body Site:*	hand / palm	*Age:*	45 years
	*Contributor:*	Shahbaz A. Janjua, MD
	*Description:*	confluent erythematous scaly plaques
	*Comments:*	Over 6 months this 45-year-old man developed diffuse symmetric reddish-orange scaly plaques with distinct areas of sparing on the trunk and extremities and a palmoplantar keratoderma. He improved with oral acitretin therapy.
	*Related Images:*	Pityriasis_rubra_pilaris_1_031116

© 2001-2010, DermAtlas	*Image Name:*	Pityriasis_rubra_pilaris_1_031116	*File Type:*	jpg
	*Diagnosis:*	PITYRIASIS RUBRA PILARIS / ERYTHRODERMA	*Category:*	papulosquamous eruptions
	*Body Site:*	foot	*Age:*	45 years
	*Contributor:*	Shahbaz A. Janjua, MD
	*Description:*	confluent erythematous scaly plaques
	*Comments:*	Over 6 months this 45-year-old man developed diffuse symmetric reddish-orange scaly plaques with distinct areas of sparing on the trunk and extremities and a palmoplantar keratoderma. He improved with oral acitretin therapy.
	*Related Images:*	Pityriasis_rubra_pilaris_1_031117

© 2001-2010, DermAtlas	*Image Name:*	Pityriasis_rubra_pilaris_8_030926	*File Type:*	jpg
	*Diagnosis:*	PITYRIASIS RUBRA PILARIS	*Category:*	papulosquamous eruptions
	*Body Site:*	leg	*Age:*	45 years
	*Contributor:*	Shahbaz A. Janjua, MD
	*Description:*	diffuse symmetric reddish orange scaly plaques with distinct areas of sparing, palmoplantar keratoderma and keratotic follicular papules.
	*Comments:*	This 45-year-old man developed a progressive red papulosquamous eruption over two months characterized by reddish orange scaly plaques, palmoplantar keratoderma and keratotic, follicular papules more prominent on the extremities. The eruption became confluent and disseminated over the trunk, face, and extremities with distinct areas of sparing. He responded poorly to topical emolients and steroids. Oral acitretin therapy was being considered to improve his condition.
	*Related Images:*	All related Images Pityriasis_rubra_pilaris_1_030926 Pityriasis_rubra_pilaris_2_030926 Pityriasis_rubra_pilaris_3_030926 Pityriasis_rubra_pilaris_4_030926 Pityriasis_rubra_pilaris_5_030926 Pityriasis_rubra_pilaris_6_030926 Pityriasis_rubra_pilaris_7_030926

© 2001-2010, DermAtlas	*Image Name:*	Pityriasis_rubra_pilaris_7_030926	*File Type:*	jpg
	*Diagnosis:*	PITYRIASIS RUBRA PILARIS	*Category:*	papulosquamous eruptions
	*Body Site:*	knee	*Age:*	45 years
	*Contributor:*	Shahbaz A. Janjua, MD
	*Description:*	diffuse symmetric reddish orange scaly plaques with distinct areas of sparing, palmoplantar keratoderma and keratotic follicular papules.
	*Comments:*	This 45-year-old man developed a progressive red papulosquamous eruption over two months characterized by reddish orange scaly plaques, palmoplantar keratoderma and keratotic, follicular papules more prominent on the extremities. The eruption became confluent and disseminated over the trunk, face, and extremities with distinct areas of sparing. He responded poorly to topical emolients and steroids. Oral acitretin therapy was being considered to improve his condition.
	*Related Images:*	All related Images Pityriasis_rubra_pilaris_1_030926 Pityriasis_rubra_pilaris_2_030926 Pityriasis_rubra_pilaris_3_030926 Pityriasis_rubra_pilaris_4_030926 Pityriasis_rubra_pilaris_5_030926 Pityriasis_rubra_pilaris_6_030926 Pityriasis_rubra_pilaris_8_030926