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Difference between revisions of "Pityriasis rubra pilaris"
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Pityriasis rubra pilaris (view source)
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| Caption = | | Caption = | ||
| Synonyms = | | Synonyms = | ||
| Micro = | | 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 = | | LMDDx = [[Dermatomycosis|fungal infection]], [[psoriasis]], [[Darier disease]], [[seborrhoeic dermatitis]] | ||
| Stains = | | 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 = | | 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 = | ||
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| Other = | | Other = | ||
| ClinDDx = | | ClinDDx = | ||
| Tx = | | Tx = retinoids, methotrexate and others | ||
}} | }} | ||
'''Pityriasis rubra pilaris''', abbreviated '''PRP''', is an uncommon [[inflammatory skin disorder]]. | '''Pityriasis rubra pilaris''', abbreviated '''PRP''', is an uncommon [[inflammatory skin disorder]]. | ||
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*Uncommon.<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> | *Uncommon.<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> | ||
*Typically sporadic, may be familial.<ref name=pmid23815954>{{Cite journal | last1 = Mercer | first1 = JM. | last2 = Pushpanthan | first2 = C. | last3 = Anandakrishnan | first3 = C. | last4 = Landells | first4 = ID. | title = Familial pityriasis rubra pilaris: case report and review. | journal = J Cutan Med Surg | volume = 17 | issue = 4 | pages = 226-32 | month = | year = | doi = | PMID = 23815954 }}</ref> | *Typically sporadic, may be familial.<ref name=pmid23815954>{{Cite journal | last1 = Mercer | first1 = JM. | last2 = Pushpanthan | first2 = C. | last3 = Anandakrishnan | first3 = C. | last4 = Landells | first4 = ID. | title = Familial pityriasis rubra pilaris: case report and review. | journal = J Cutan Med Surg | volume = 17 | issue = 4 | pages = 226-32 | month = | year = | doi = | PMID = 23815954 }}</ref> | ||
*Bimodal age distribution:<ref name=pmid20184391/> | |||
**Children: <10 years. | |||
**Adults: 40s and 50s. | |||
Treatment: | |||
*Retinoids, methotrexate and others.<ref name=pmid20184391/> | |||
==Gross== | ==Gross== | ||
*Salmon-coloured (red) [[plaque]]s interrupted by well-demarcated normal appearing [[skin]].<ref name=pmid20184391>{{Cite journal | last1 = Klein | first1 = A. | last2 = Landthaler | first2 = M. | last3 = Karrer | first3 = S. | title = Pityriasis rubra pilaris: a review of diagnosis and treatment. | journal = Am J Clin Dermatol | volume = 11 | issue = 3 | pages = 157-70 | month = | year = 2010 | doi = 10.2165/11530070-000000000-00000 | PMID = 20184391 }}</ref> | |||
==Microscopic== | ==Microscopic== | ||
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=== | ||
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==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]]. | ||
==References== | ==References== | ||
{{Reflist| | {{Reflist|2}} | ||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Dermatopathology]] | |||