Difference between revisions of "Pityriasis rubra pilaris"
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| Caption = | | Caption = | ||
| Synonyms = | | Synonyms = | ||
| Micro = alterating [[orthokeratosis]] and [[parakeratosis]] (known as "checkerboard pattern"), +/-eosinophils (common), follicular plugging, [[acantholysis]] (common) | | Micro = alterating [[orthokeratosis]] and [[parakeratosis]] (known as "checkerboard pattern"), marked uniform [[acanthosis]] with broad dermal papillae and broad epidermal ridges, , +/-eosinophils (common), follicular plugging, scattered foci of [[acantholysis]] (common), occasional mild spongiosis | ||
| Subtypes = | | Subtypes = | ||
| LMDDx = [[Dermatomycosis|fungal infection]] | | LMDDx = [[Dermatomycosis|fungal infection]], [[psoriasis]], [[Darier disease]], [[seborrhoeic dermatitis]] | ||
| Stains = PASD -ve (to r/o fungal infections) | | Stains = PASD -ve (to r/o fungal infections) | ||
| IHC = | | IHC = | ||
| EM = | | EM = | ||
| Molecular = | | Molecular = | ||
| IF = | | IF = negative (changes in ''Desmoglein 1'' & ''Desmoglein 3'' in [[pemphigus vulgaris]]) | ||
| Gross = salmon-coloured plaques, well-demarcated | | Gross = salmon-coloured plaques, well-demarcated | ||
| Grossing = | | Grossing = | ||
| Site = [[skin]] - see ''[[inflammatory skin disorders]]'' | | Site = [[skin]] - see ''[[inflammatory skin disorders]]'' | ||
| Assdx = | | Assdx = HIV infection - recalcitrant PRP | ||
| Syndromes = | | Syndromes = | ||
| Clinicalhx = | | Clinicalhx = Classic adult - self-limited exfoliative, erythematous dermatitis that starts over the head and neck and extends downwards forming orange plaques with spared islands of normal skin, follicular hyperkeratosis, palmar involvement with orange hyperkeratosis | ||
| Signs = | | Signs = | ||
| Symptoms = | | Symptoms = | ||
| Line 46: | Line 46: | ||
Features:<ref name=pmid24783259>{{Cite journal | last1 = Marrouche | first1 = N. | last2 = Kurban | first2 = M. | last3 = Kibbi | first3 = AG. | last4 = Abbas | first4 = O. | title = Pityriasis rubra pilaris: clinicopathological study of 32 cases from Lebanon. | journal = Int J Dermatol | volume = 53 | issue = 4 | pages = 434-9 | month = Apr | year = 2014 | doi = | PMID = 24783259 }}</ref> | Features:<ref name=pmid24783259>{{Cite journal | last1 = Marrouche | first1 = N. | last2 = Kurban | first2 = M. | last3 = Kibbi | first3 = AG. | last4 = Abbas | first4 = O. | title = Pityriasis rubra pilaris: clinicopathological study of 32 cases from Lebanon. | journal = Int J Dermatol | volume = 53 | issue = 4 | pages = 434-9 | month = Apr | year = 2014 | doi = | PMID = 24783259 }}</ref> | ||
*Alterating [[orthokeratosis]] and [[parakeratosis]] - known as "checkerboard pattern". | *Alterating [[orthokeratosis]] and [[parakeratosis]] - known as "checkerboard pattern". | ||
*Marked uniform [[acanthosis]] with broad dermal papillae and broad epidermal ridges. | |||
*Follicular plugging. | |||
*+/-Eosinophils (common). | *+/-Eosinophils (common). | ||
* | *Scattered foci of [[acantholysis]].<ref name=pmid23286804/> | ||
*[[ | *Occasional mild spongiosis. | ||
Notes: | |||
*PRP is easy to miss - clinical history (r/o PRP) usually needed to prompt correct diagnosis. | |||
===Microscopic DDx=== | |||
*[[Psoriasis vulgaris]]<ref name=pmid18418089>{{Cite journal | last1 = Brinster | first1 = NK. | title = Dermatopathology for the surgical pathologist: a pattern based approach to the diagnosis of inflammatory skin disorders (part I). | journal = Adv Anat Pathol | volume = 15 | issue = 2 | pages = 76-96 | month = Mar | year = 2008 | doi = 10.1097/PAP.0b013e3181664e8d | PMID = 18418089 }}</ref> - thinned suprapapillary plates, hypogranulosis, neutrophils in the stratum corneum, dilated papillary dermal blood vessels, acantholysis absent. | |||
*[[Lichen simplex chronicus]] - usually a localized process, not a widespread process. | |||
*Darier disease - acantholysis is prominent with corps ronds and grains, follicular plugging absent. | |||
*[[Pemphigus vulgaris]] - PRP with extensive acantholysis can mimic; [[immunofluorescence|IF]] will be negative in PRP. | |||
*[[Hailey-Hailey disease]] - falling brick wall pattern of acantholysis. | |||
*Seborrhoeic dermatitis - more spongiotic, often with neutrophils. | |||
*[[Dermatophytosis|Fungal infection]]. | |||
==Associated Diagnoses== | |||
*[[ | *[[HIV infection]] - recalcitrant PRP. | ||
===Images=== | ===Images=== | ||
| Line 59: | Line 73: | ||
==Stains== | ==Stains== | ||
*[[PASD stain|PASD]] -ve for fungal organisms.<ref name=pmid23286804/> | *[[PASD stain|PASD]] -ve for fungal organisms.<ref name=pmid23286804/> | ||
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==IHC== | ==IHC== | ||
==Molecular== | ==Molecular== | ||
==Sign out== | ==Sign out== --> | ||
==See also== | ==See also== | ||
*[[Inflammatory skin disorders]]. | *[[Inflammatory skin disorders]]. | ||
| Line 73: | Line 86: | ||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Dermatopathology]] | |||
Latest revision as of 05:02, 30 September 2014
| Pityriasis rubra pilaris | |
|---|---|
| Diagnosis in short | |
|
| |
| LM | alterating orthokeratosis and parakeratosis (known as "checkerboard pattern"), marked uniform acanthosis with broad dermal papillae and broad epidermal ridges, , +/-eosinophils (common), follicular plugging, scattered foci of acantholysis (common), occasional mild spongiosis |
| LM DDx | fungal infection, psoriasis, Darier disease, seborrhoeic dermatitis |
| Stains | PASD -ve (to r/o fungal infections) |
| IF | negative (changes in Desmoglein 1 & Desmoglein 3 in pemphigus vulgaris) |
| Gross | salmon-coloured plaques, well-demarcated |
| Site | skin - see inflammatory skin disorders |
|
| |
| Associated Dx | HIV infection - recalcitrant PRP |
| Clinical history | Classic adult - self-limited exfoliative, erythematous dermatitis that starts over the head and neck and extends downwards forming orange plaques with spared islands of normal skin, follicular hyperkeratosis, palmar involvement with orange hyperkeratosis |
| Prevalence | uncommon |
| Treatment | retinoids, methotrexate and others |
Pityriasis rubra pilaris, abbreviated PRP, is an uncommon inflammatory skin disorder.
General
- Uncommon.[1]
- Typically sporadic, may be familial.[2]
- Bimodal age distribution:[3]
- Children: <10 years.
- Adults: 40s and 50s.
Treatment:
- Retinoids, methotrexate and others.[3]
Gross
Microscopic
Features:[1]
- Alterating orthokeratosis and parakeratosis - known as "checkerboard pattern".
- Marked uniform acanthosis with broad dermal papillae and broad epidermal ridges.
- Follicular plugging.
- +/-Eosinophils (common).
- Scattered foci of acantholysis.[4]
- Occasional mild spongiosis.
Notes:
- PRP is easy to miss - clinical history (r/o PRP) usually needed to prompt correct diagnosis.
Microscopic DDx
- Psoriasis vulgaris[5] - thinned suprapapillary plates, hypogranulosis, neutrophils in the stratum corneum, dilated papillary dermal blood vessels, acantholysis absent.
- Lichen simplex chronicus - usually a localized process, not a widespread process.
- Darier disease - acantholysis is prominent with corps ronds and grains, follicular plugging absent.
- Pemphigus vulgaris - PRP with extensive acantholysis can mimic; IF will be negative in PRP.
- Hailey-Hailey disease - falling brick wall pattern of acantholysis.
- Seborrhoeic dermatitis - more spongiotic, often with neutrophils.
- Fungal infection.
Associated Diagnoses
- HIV infection - recalcitrant PRP.
Images
Stains
See also
References
- ↑ 1.0 1.1 Marrouche, N.; Kurban, M.; Kibbi, AG.; Abbas, O. (Apr 2014). "Pityriasis rubra pilaris: clinicopathological study of 32 cases from Lebanon.". Int J Dermatol 53 (4): 434-9. PMID 24783259.
- ↑ Mercer, JM.; Pushpanthan, C.; Anandakrishnan, C.; Landells, ID.. "Familial pityriasis rubra pilaris: case report and review.". J Cutan Med Surg 17 (4): 226-32. PMID 23815954.
- ↑ 3.0 3.1 3.2 Klein, A.; Landthaler, M.; Karrer, S. (2010). "Pityriasis rubra pilaris: a review of diagnosis and treatment.". Am J Clin Dermatol 11 (3): 157-70. doi:10.2165/11530070-000000000-00000. PMID 20184391.
- ↑ 4.0 4.1 4.2 Leger, M.; Newlove, T.; Robinson, M.; Patel, R.; Meehan, S.; Ramachandran, S. (Dec 2012). "Pityriasis rubra pilaris.". Dermatol Online J 18 (12): 14. PMID 23286804.
- ↑ Brinster, NK. (Mar 2008). "Dermatopathology for the surgical pathologist: a pattern based approach to the diagnosis of inflammatory skin disorders (part I).". Adv Anat Pathol 15 (2): 76-96. doi:10.1097/PAP.0b013e3181664e8d. PMID 18418089.