History

Fact Explanation
Introduction Parapsoriasis is a group of inflammatory disorders with an unknown etiology [1] and that is resistant to conventional treatment. It is relatively uncommon. It resembles psoriasis, but considered as different group of disorders which has the combination of features of disorders psoriasis, lichen planus and seborrheic dermatitis. [7] Parapsoriasis is divided into two groups, pityriasis lichenoides and parapsoriasis en plaques [2,6] (small- and large-plaque parapsoriasis). Large plaque parapsoriasis is further subdivided into poikilodermatous and retiform parapsoriasis. [3] Some consider this as a variety of lymphoproliferative disorder, ranging from chronic dermatitis to T-cell cutaneous lymphoma (TCCL). [1]
Skin lesions [2] Skin lesions are consist of maculopapular, idiopathic, chronic scaly [3] psoriatic and lichenoid eruptions. Eruptions are consists of sharply defined plaques, which are sometimes scaly and brownish in colour. Lesions are round or oval in colour. [7]
Onset and duratiion [3] Parapsoriasis is an idiopathic, chronic scaly dermatosis. [3,5] Chronic nature is particularly important in the diagnosis. [5]
Age, gender and race [3] Usually seen among the middle age, peaks in the fifthe decade of life. All races are affected with a slight male preponderance . [3] Hypopigmented patches are commonly seen in the Indian subcontinent. [5]
Associated features It may be mildly pruritic [3,4] or non pruritic. Generalized malaise may be present. [4]
Past history of similar episodes [1] They can have recurrances of the disease. [1,5]
References
  1. DUARTE IA, KORKES KL, AMORIM VA, KOBATA C, BUENSE R, LAZZARINI R. An evaluation of the treatment of parapsoriasis with phototherapy An Bras Dermatol [online] 2013, 88(2):306-308 [viewed 13 September 2014] Available from: doi:10.1590/S0365-05962013000200028
  2. WHITTLE CH. ? Parapsoriasis Proc R Soc Med [online] 1943 Apr, 36(6):296 [viewed 13 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1998501
  3. NAG F, GHOSH A, BISWAS P, CHATTERJEE G, BISWAS S. Ichthyosiform Large Plaque Parapsoriasis: Report of a Rare Entity Indian J Dermatol [online] 2013, 58(5):385-387 [viewed 13 September 2014] Available from: doi:10.4103/0019-5154.117314
  4. EISMAN S., O'TOOLE E. A., JONES A., WHITTAKER S. J.. Granulomatous mycosis fungoides presenting as an acquired ichthyosis. Clin Exp Dermatol [online] 2003 March, 28(2):174-176 [viewed 13 September 2014] Available from: doi:10.1046/j.1365-2230.2003.01224.x
  5. SARVESWARI KN, YESUDIAN P. The conundrum of parapsoriasis versus patch stage of mycosis fungoides. Indian J Dermatol Venereol Leprol [online] 2009 May-Jun, 75(3):229-35 [viewed 13 September 2014] Available from: doi:10.4103/0378-6323.51239
  6. STEVENSON CJ. Parapsoriasis Lichenoides Proc R Soc Med [online] 1964 Apr, 57(4):316-317 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1897896
  7. AULD JW. A Case of Parapsoriasis Can Med Assoc J [online] 1935 Aug, 33(2):183-185 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1561302

Examination

Fact Explanation
Skin lesions-appearance Large plaque parapsoriasis appear as large, more than 5 cm, variably erythematous, round or irregularly shaped ichthyosiform patches or very thin plaques. [1] There may be intervening normal skin. Small plaque lesions are usually on the upper trunk, 2 to 6 cm in diameter, occasionally with a digitate appearance and no atrophy or poikiloderma. [3]
Site of lesions May involve mainly the trunk and extrimities. [1]
Scaling Dry adherent pigmented scales are present with varying shapes and sizes. [1]
Itching Lesions may be mildly itchy. [1]
Telangiectasia [1] Mild telangiectasia [1] will be present over some lesions.
Sensation There is usually no sensory loss . [1]
Features of mycosis fungoides: plaques, patches Parapsoriasis is a precursor of mycosis fungoides. [1] Mycosis fungoidesis a common primary cutaneous T-cell lymphoma, characterized by classical non-infiltrated patches, plaques, tumors, and erythrodermic stages. [2] Usually these are large patches of more than 5 cm in diameter that may expand slowly to form well-demarcated lesions . [3]
Lymphadenopathy Occasionally patients wth mycosis fungoides may present with regional lymphadenopathy due to dermatopathic changes in the node. [4]
References
  1. NAG F, GHOSH A, BISWAS P, CHATTERJEE G, BISWAS S. Ichthyosiform Large Plaque Parapsoriasis: Report of a Rare Entity Indian J Dermatol [online] 2013, 58(5):385-387 [viewed 13 September 2014] Available from: doi:10.4103/0019-5154.117314
  2. FURLAN FC, SANCHES JA. Hypopigmented mycosis fungoides: a review of its clinical features and pathophysiology An Bras Dermatol [online] 2013, 88(6):954-960 [viewed 13 September 2014] Available from: doi:10.1590/abd1806-4841.20132336
  3. SARVESWARI KN, YESUDIAN P. The conundrum of parapsoriasis versus patch stage of mycosis fungoides. Indian J Dermatol Venereol Leprol [online] 2009 May-Jun, 75(3):229-35 [viewed 13 September 2014] Available from: doi:10.4103/0378-6323.51239
  4. CAREY RW, PINCUS JD. An Invariant for Certain Operator Algebras Proc Natl Acad Sci U S A [online] 1974 May, 71(5):1952-1956 [viewed 14 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC388361

Differential Diagnoses

Fact Explanation
Psoriasis [15] Psoriasis is a chronic disease, with periods of exacerbation and remission. [8] characterized by that are clearly demarcated from adjacent, Cutaneous psoriatic lesions [7] are red silver scaly plaques involving tha trunk, back, scalp and extrimities. Redness is due to dilation of blood vessels and scales are due to rapid keratinocyte proliferation. [8] Adjusent skin may be normal. Psoriatic arthritis typically affects the distal interphalangeal joints. [6] Co-morbiditiessuch as cardiovascular disease, diabetes mellitus (mainly type 2), metabolic syndrome, obesity, impaired quality of life and depression are common in patients with psoriasis. [8]
Lichen planus [15] Lichen planus is a chronic inflammatory disease [5] of skin and the mucus membrane. [4]Usually presents n the fourth decade of life and is common in women more than men. [4] Skin lesions are violaceous flat-topped papules in ankles, wrist, and genitalia, with sparing of the facial skin. [4] Oral lesions involve the buccal mucosa. Nikolsky's sign (peeling of the epithelium from the epithelium–connective tissue junction on slight lateral pressure in nonaffected area) will be present on some occasions. [5]
Mycosis fungoides [1] Mycosis fungoides is the most common primary cutaneous T-cell lymphoma. Different types of disaese include granulomatous, pustular, purpuric, hyperkeratotic and verrucous, bullous, invisible, and hypopigmented variants. [2] Lesions may be hypopigmented-to-achromic lesions, mainly distributed on the trunk and proximal portions of the extremities,buttocks [3] , pelvic girdle and the lower limbs. [2] Itchiness, atrophy and telangiectasia are present. Granulomatous slack skin sare an erythematous atrophic, flaccid, pendular and redundant cutaneous lesions in folding areas. [3] Non-sun-exposed areas such as trunk below the waist line flanks, breasts, inner thighs, inner arms and periaxillary areas are affected. [9] Sun exposure will aggravate the condition. [2] Conventional type is common in the fifth to sixth decades of life, and hypopigmented type is common in the pediatric population. [2] Investigations should include peripheral blood examination, quantification of Sézary cells and T lymphocytes using flow cytometry.
Dermatitis Dermatitis is the inflammation of the skin. [10] It may be atopic or contact depending on the underlying pathogenesis. Contact dermatitis may be triggered by various irritants such as nicke, cromium, leather, cement dust, rubber latex etc. Atopic dermatitis is a chronic or relapsing disease [11] icharacterised by itching. [10] Genetic factors are involving in the atopy and they may have associated asthma/ hay fever. [11] Seborrheic dermatitis may resemble parapsoriasis in appearance. [15]
Cutaneous T cell lymphoma [15] Cutaneous T cell lymphoma is characterized by malignant proliferation of T lymphocytes. It involves the skin causing plaques. It differs from the parapsoriasis as this later involves lymph nodes and visceral organs. [12] There will be aberrant T-helper cells (CD3−CD4+ TCR+) in the peripheral blood and lesional skin. Lymphocyte count will be elevated in the blood. [12]
Pityriasis alba Lesions of the pityriasis alba may be asymptomatic superficial hypopigmented macules with slight overlying scaling. [15] They usually involves the face, neck and shoulders. Itching and personal history of atopy are present in some patients. [14] Aetiology may be related to the infection, nutritional factors, atopy and dryness of the skin.
Pityriasis rosea Pityriasis rosea is a papulosquamous disorder. Clinical features would be large erythematous herald patch and subsequent eruptions of multiple similar, smaller oval patches in the classic “Christmas tree” distribution. [13] Lesions may be papular, papulosquamous, vesicular, purpuric and lichenoid. [13]
References
  1. NAG F, GHOSH A, BISWAS P, CHATTERJEE G, BISWAS S. Ichthyosiform Large Plaque Parapsoriasis: Report of a Rare Entity Indian J Dermatol [online] 2013, 58(5):385-387 [viewed 13 September 2014] Available from: doi:10.4103/0019-5154.117314
  2. FURLAN FC, SANCHES JA. Hypopigmented mycosis fungoides: a review of its clinical features and pathophysiology An Bras Dermatol [online] 2013, 88(6):954-960 [viewed 13 September 2014] Available from: doi:10.1590/abd1806-4841.20132336
  3. YAMASHITA T, ABBADE LP, MARQUES ME, MARQUES SA. Mycosis fungoides and S?zary syndrome: clinical, histopathological and immunohistochemical review and update An Bras Dermatol [online] 2012, 87(6):817-830 [viewed 13 September 2014] Available from: doi:10.1590/S0365-05962012000600001
  4. LAVANYA N, JAYANTHI P, RAO UK, RANGANATHAN K. Oral lichen planus: An update on pathogenesis and treatment J Oral Maxillofac Pathol [online] 2011, 15(2):127-132 [viewed 13 September 2014] Available from: doi:10.4103/0973-029X.84474
  5. MITTAL N, SHANKARI GM, PALASKAR S. Role of angiogenesis in the pathogenesis of oral lichen planus J Oral Maxillofac Pathol [online] 2012, 16(1):45-48 [viewed 13 September 2014] Available from: doi:10.4103/0973-029X.92972
  6. VEALE D, RITCHLIN C, FITZGERALD O. Immunopathology of psoriasis and psoriatic arthritis Ann Rheum Dis [online] 2005 Mar, 64(Suppl 2):ii26-ii29 [viewed 13 September 2014] Available from: doi:10.1136/ard.2004.031740
  7. WINTERFIELD L, MENTER A, GORDON K, GOTTLIEB A. Psoriasis treatment: current and emerging directed therapies Ann Rheum Dis [online] 2005 Mar, 64(Suppl 2):ii87-ii90 [viewed 13 September 2014] Available from: doi:10.1136/ard.2004.032276
  8. JOHNSON-HUANG LM, LOWES MA, KRUEGER JG. Putting together the psoriasis puzzle: an update on developing targeted therapies Dis Model Mech [online] 2012 Jul, 5(4):423-433 [viewed 13 September 2014] Available from: doi:10.1242/dmm.009092
  9. SARVESWARI KN, YESUDIAN P. The conundrum of parapsoriasis versus patch stage of mycosis fungoides. Indian J Dermatol Venereol Leprol [online] 2009 May-Jun, 75(3):229-35 [viewed 13 September 2014] Available from: doi:10.4103/0378-6323.51239
  10. WILLIAMS HC, CHALMERS JR, SIMPSON EL. Prevention of atopic dermatitis F1000 Med Rep [online] :24 [viewed 15 September 2014] Available from: doi:10.3410/M4-24
  11. BOGUNIEWICZ M, LEUNG DY. Atopic Dermatitis: A Disease of Altered Skin Barrier and Immune Dysregulation Immunol Rev [online] 2011 Jul, 242(1):233-246 [viewed 15 September 2014] Available from: doi:10.1111/j.1600-065X.2011.01027.x
  12. SANO S, MATSUI Y, ITAMI S, YOSHIKAWA K. Immunological study on CD3 defective cutaneous T cell lymphoma cells from a patient with S?zary syndrome Clin Exp Immunol [online] 1998 Aug, 113(2):190-197 [viewed 15 September 2014] Available from: doi:10.1046/j.1365-2249.1998.00649.x
  13. SACCHIDANAND S, SHWETHA S, MALVANKAR DD, MALLIKARJUNA M. Polymorphic pityriasis rosea precipitating psoriasis Indian Dermatol Online J [online] 2013, 4(1):63-64 [viewed 15 September 2014] Available from: doi:10.4103/2229-5178.105494
  14. SORI T, NATH AK, THAPPA DM, JAISANKAR TJ. HYPOPIGMENTARY DISORDERS IN CHILDREN IN SOUTH INDIA Indian J Dermatol [online] 2011, 56(5):546-549 [viewed 15 September 2014] Available from: doi:10.4103/0019-5154.87152
  15. AULD JW. A Case of Parapsoriasis Can Med Assoc J [online] 1935 Aug, 33(2):183-185 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1561302

Investigations - for Diagnosis

Fact Explanation
Skin biopsy [1] Histopathology will reveal mild hyperkeratosis with localized parakeratosis, irregular acanthosis and mild spongiosis. [2] Dense lymphocytic infiltrate will be present in upper dermis. No nuclear atypia,or granuloma formation. Non-specific changes (focal spongiosis and psoriasiform or lichenoid dermatitis with exocytosis of small lymphocytes) will be seen in small plaque disease. [3]
Polymerase chain reaction (PCR) These are considered as disorders of monoclonal T cell disorders. [3] Polymerase chain reaction (PCR) can be used to demonstrate this associaetion.
Full blood count [4] Lmphocyte count will be high in the presence of mycosis fungoides/cutaneous T-cell lymphoma (MF/CTCL). [2] Sézary syndrome is the leukaemic variant of cutaneous T cell lymphoma. [4]
Blood film There can be atypical T lymphocytes, namely Sézary cells, in peripheral blood as well as skin infiltrate in cutaneous T cell lymphoma. [4]
References
  1. DUARTE IA, KORKES KL, AMORIM VA, KOBATA C, BUENSE R, LAZZARINI R. An evaluation of the treatment of parapsoriasis with phototherapy An Bras Dermatol [online] 2013, 88(2):306-308 [viewed 13 September 2014] Available from: doi:10.1590/S0365-05962013000200028
  2. NAG F, GHOSH A, BISWAS P, CHATTERJEE G, BISWAS S. Ichthyosiform Large Plaque Parapsoriasis: Report of a Rare Entity Indian J Dermatol [online] 2013, 58(5):385-387 [viewed 13 September 2014] Available from: doi:10.4103/0019-5154.117314
  3. SARVESWARI KN, YESUDIAN P. The conundrum of parapsoriasis versus patch stage of mycosis fungoides. Indian J Dermatol Venereol Leprol [online] 2009 May-Jun, 75(3):229-35 [viewed 13 September 2014] Available from: doi:10.4103/0378-6323.51239
  4. SANO S, MATSUI Y, ITAMI S, YOSHIKAWA K. Immunological study on CD3 defective cutaneous T cell lymphoma cells from a patient with S?zary syndrome Clin Exp Immunol [online] 1998 Aug, 113(2):190-197 [viewed 15 September 2014] Available from: doi:10.1046/j.1365-2249.1998.00649.x

Investigations - Fitness for Management

Fact Explanation
Haemoglobin level Cutaneous T-Cell Lymphoma is a skin malignancy which is a advanced spectrum of the parapsoriasis. [1] Malinancy may be associated with anaemia.
References
  1. LATKOWSKI JA, HEALD P. Strategies for Treating Cutaneous T-Cell Lymphoma: Part 1: Remission J Clin Aesthet Dermatol [online] 2009 Jun, 2(6):22-27 [viewed 14 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2923960

Investigations - Followup

Fact Explanation
Skin biopsy-Histopathology Cure of the disaese is shown when the histopathology reveals the normal skin after the treatment. [1] Folow up will be nearly 6 monthly for the large plaques and less frequently for the small plaque disaese.
Complete blood count Systemic therapy such as bis-chloroethyl-nitrourea (BCNU) may cause bone marrow suppression, most commonly leukopenia and thrombocytopenia. [2] Therefore monitoring with complete blood counts every two weeks during therapy and one month after discontinuing therapy due to the elayed hematopoietic toxicity is nesessary. [2]
References
  1. DUARTE IA, KORKES KL, AMORIM VA, KOBATA C, BUENSE R, LAZZARINI R. An evaluation of the treatment of parapsoriasis with phototherapy An Bras Dermatol [online] 2013, 88(2):306-308 [viewed 13 September 2014] Available from: doi:10.1590/S0365-05962013000200028
  2. LATKOWSKI JA, HEALD P. Strategies for Treating Cutaneous T-Cell Lymphoma: Part 1: Remission J Clin Aesthet Dermatol [online] 2009 Jun, 2(6):22-27 [viewed 14 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2923960

Investigations - Screening/Staging

Fact Explanation
Immunophenotyping analysis Immunohistochemical study of skin infiltrate will reveal CD3, CD2, CD5 and CD4. [1] It will also show the early features of mycosis fungoidosis such as wide spread epidermal expression of HLA-DR, predominance of CD+ T cell subsets and frequent CD 7 antigen deficiency. [1]
Gene rearrangement studies T cell receptor gene rearrangement studies are done, but can not exactly confirm the pattern of the disease. [1]
References
  1. NAG F, GHOSH A, BISWAS P, CHATTERJEE G, BISWAS S. Ichthyosiform Large Plaque Parapsoriasis: Report of a Rare Entity Indian J Dermatol [online] 2013, 58(5):385-387 [viewed 13 September 2014] Available from: doi:10.4103/0019-5154.117314

Management - General Measures

Fact Explanation
Patient education Patients should be educated on the nature of the disease particularly about the benign self-limiting nature of the disease. [1] Reassurance is specially important in small plaque parapsoriasis where it is entirely a benign condition. Exacerbating factors should be avoided including stress, infection, trauma, and use of medications such as angiotensin-converting enzyme inhibitors, beta-adrenergic blockers, lithium and the antimalarial agent hydroxychloroquine.
Emollients Lesions are usually scaly eruptions and emolients may be helpful to alleviate the scaliness. [1]
Treatment with Vitamin D2 [3] Parapsoriasis can be treated with Vitamin D2. [2] Low vitamin D status, may be associated due to the inadequate oral intake and sun avoidanceTherefore it is recommended that serum 25-hydroxyvitamin D [2] concentrations less than 30 ng/mL be considered in these patients.
Follow-up Number of lesions, size of lesions, and induration or epidermal atrophy should be checked on follow up. If there are adverse signs such as increasing number and size of the lesions and epodermal atrophy [4] , repeat skin biopsy may be needed to exclude the possible complcations such as mycosis fungoides.
References
  1. NAG F, GHOSH A, BISWAS P, CHATTERJEE G, BISWAS S. Ichthyosiform Large Plaque Parapsoriasis: Report of a Rare Entity Indian J Dermatol [online] 2013, 58(5):385-387 [viewed 13 September 2014] Available from: doi:10.4103/0019-5154.117314
  2. YORKE A. Parapsoriasis en Plaques--Treated with Vitamin D2 Proc R Soc Med [online] 1948 Nov, 41(11):755-756 [viewed 13 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2184674
  3. BARBER HW, ERSKINE D. Parapsoriasis en Plaques Treated with Calciferol Proc R Soc Med [online] 1948 Nov, 41(11):755 [viewed 13 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2184689
  4. CAREY RW, PINCUS JD. An Invariant for Certain Operator Algebras Proc Natl Acad Sci U S A [online] 1974 May, 71(5):1952-1956 [viewed 14 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC388361

Management - Specific Treatments

Fact Explanation
Topical steroids [2] Mid to high potency topical steroids are used to treat the parapsoriasis. [2] Steroids are vailable in ointment, cream, lotion and solution forms. Anti-inflammatory and antiproliferative effects of corticosteroids are used for the treatment of parapsoriasis.
Other medications : Bexarotene gel, topical nitrogen mustard, topical carmustine [3] Bexarotene gel is used in patients with the lesions less than 15-percent body surface area. [3] It is available as 1% solution and is used to treat the cutaneous lymphomatous lesions. [3] Nitrogen mustard is available as an ointment formulation, takes atleast 16 weeks to respond. Cutaneous hypersensitivity is the most common side effect of the treatment. [3] Carmustine is an oinment, side effects include contact hypersensitivity, erythema, skin burning, telangiectasia, and hyperpigmentation. [3]
Phototherapy [1] Phototherapy is used because of its anti-inflammatory and immunosuppressive effects. [1] Broad- or narrow-band UV-B or PUVA can be used to treat the parapsoriasis. UVA has a greater penetration than UVB. UVB-B only affects the epidermis and superficial dermis, UVB-B is indicated in situations where psoralen is contraindicated or when patient is on photosensitizing drugs.[1] It is indicated for all stages of the parapsoriasis. [1] Phototherapy requires an assessment of the response usually at monthly intervals.
Management of complications Development of T-cell cutaneous lymphoma is a recognized complication of the disease. [1] Proper follow up is nesessary to evaluate the emergance of these complications.
Bone marrow transplantation [3] Cutaneous T cell lymphoma may be tretaed with allogeneic bone marrow transplantation. [3]
References
  1. DUARTE IA, KORKES KL, AMORIM VA, KOBATA C, BUENSE R, LAZZARINI R. An evaluation of the treatment of parapsoriasis with phototherapy An Bras Dermatol [online] 2013, 88(2):306-308 [viewed 13 September 2014] Available from: doi:10.1590/S0365-05962013000200028
  2. NAG F, GHOSH A, BISWAS P, CHATTERJEE G, BISWAS S. Ichthyosiform Large Plaque Parapsoriasis: Report of a Rare Entity Indian J Dermatol [online] 2013, 58(5):385-387 [viewed 14 September 2014] Available from: doi:10.4103/0019-5154.117314
  3. LATKOWSKI JA, HEALD P. Strategies for Treating Cutaneous T-Cell Lymphoma: Part 1: Remission J Clin Aesthet Dermatol [online] 2009 Jun, 2(6):22-27 [viewed 14 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2923960