dermatology
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An indolent T-cell lymphoproliferative disorder Clinical features •2M:1F •Crops of rapidly growing, self-healing, pink papules up to 1.0 cm diameter •Heal with atrophic scars •Occasionally large nodules •Trunk & limbs are sites of predilection •Regional variant •Exceptional mucosal involvement •Follicular variant •Pustular variant •Self-limiting or protracted course •Up to 20% associated with mycosis fungoides, primary cutaneous anaplastic large cell lymphoma or Hodgkin lymphoma Histological features •Subdivided into 5 major & several rarer variants •Type A: 75-80%, wedge shaped infiltrate with base uppermost, large, anaplastic cells…
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Definition •Leukemic variant of CTCL with a very poor prognosis •Erythroderma affecting 80% or more of the integument; often scaly, intensely pruritic; sometimes marked edema •Lymphadenopathy •Circulating Sézary cell count of >1000/sq mm. •CD4:CD8 of 10 or more • CD4+/CD7- of 40% or more •CD4+ve/CD26-ve of 30% or more •Variable loss of T-cell antigens •T-cell monoclonal population…
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Clinical features •Very poor prognosis •Exceptionally rare & is only seen in stage IV disease (I have only seen three cases in 52 years!) •Bimodal age distribution for Hodgkin lymphoma- teenagers & over 50’s •Nodular sclerosis>mixed cellularity>lymphocyte depleted •Nodal disease extending to the skin, retrograde lymphatic spread andmetastatic disease •Single or multiple pink to red papules, nodules & plaques •Chest, head & neck, inguinal region, axilla but can present anywhere Histological…
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Clinical features •Very rare aggressive acute leukemia •Very commonly affects the skin in addition to the blood and bone marrow; also, lymph nodes & spleen •B symptoms •Mostly affects the elderly •3M:1F •Head, trunk & extremities •Bruise-like or erythematous maculopapular lesions, nodules or plaques •Associations with myelodysplastic syndrome, myelomonocytic leukemia or AML Histological features . No epidermal involvement . Dermal +/- subcutaneous fat infiltrate . No angioinvasion or angiodestruction . Monotonous blast…
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Clinical features •Aggressive with high mortality •Extranodal NK/T cell lymphoma, nasal type & aggressive NK-cell leukemia •Most commonly affects the nasal cavity & upper aerodigestive tract •Predominantly seen in Chinese, Korean & Japanese populations; rare in Europeans •Cutaneous involvement may co-exist with nasal lesions or represent the primary site •Skin lesions include often ulcerated nodules & plaques, vasculitic lesions, panniculitis & cellulitis-like lesions •Predilection for trunk & limbs •B symptoms…
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Clinical features •Rare cutaneous lymphoma with a generally good prognosis except for patients who develop a hemophagocytosis syndrome •2F:1M •Wide age range, can affect children & adolescents •Asymptomatic erythematous/violaceus nodules or deep plaques •Limbs & trunk but may be widespread •Variable B-symptoms in up to 50% of patients •Anemia, cytopenia, high ESR & abnormal liver function •Hemophagocytosis syndrome (20% of cases) •Coexistent autoimmune disease…
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Clinical features •May present with & without follicular mucinosis •May be accompanied (and often is) by syringotropic MF •Very rare & presents most commonly in middle-aged or elderly males •Face, scalp (often associated with alopecia) & neck, trunk or limbs •Eyebrow involvement is said to be typical •Intensely itchy, patches, plaques or grouped papules •Sometimes seen in tumor stage disease •Occasionally…
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This is such a large topic. The blog focuses on the clinical appearances and histology. Clinical features •Commonest form of cutaneous T-cell lymphoma although it is very rare with around 1000 new cases diagnosed annually in the USA •2M:1 F •Most common in 4th-6th decades, childhood disease is discussed in a separate blog •In most patients, the…
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Clinical featues •Primary cutaneous disease is very rare (3% of patients with Rosai Dorfman disease) •Benign, self limiting •Skin is commonly involved in patients with nodal disease (10%) •Primary cutaneous disease is more common in middle aged patients & shows a female predominance •Mostly White & Asian populations •Lesions are generally multiple, polymorphic and include papules, nodules or plaques variably flesh colored, pink,…
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Whether this lesions is a distinct entity sui generis is uncertain. Some authors regard it as a variant of actinic lentigo. I do not have rigid views on the matter although in my experience large cell acanthoma does not show the lentiginous epidermal hyperplsia seen in actinic lentigo. Clinical features •Presents as a circumscribed, often…
McKee Derm 