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Medications,
infections,
insect
bites
Knowns as Speigler-Fendt sarcoid Caused by Borrelia, infections, herpes zosters scars, tatoo, drugs Appears as discrete firm of doughy cutaneous papules or nodules Most lesions are asymptomatic, treatment not required If caused by medication, medication should be removed
Pseudolymphoma has to be distinguished from cutaneous lymphomas by the combination of clinicopathological correlation, histochemical studies, and, in selected cases, gene rearrangement studies T cell lymhoma can be usually distinguished from T cell pseudolymphoma by the presence of prominent epidermotropism, large and atypical lymphocytes, and T cell gene rearrangements up to 90%
The lack of acanthosis, "bottom-heavy" infiltrates, light-chain expression of monotypical B-cells, and immunoglobulin gene rearrangements (75%) provide strong evidence for the diagnosis of B-cell lymphoma A careful monitoring of these patients for the development of lymphoma is necessary
Coalescing erythematous follicular papules and raspberry-like nodules on the right shoulder.
Histological examination of the skin The microscopic examinations revealed dense and diffuse infiltrates of cytologically benign-appearing lymphocytes and scattered histiocytes in the upper and mid dermis
are not MF
Fungoides Pagetoid Reticulosis Sezary Syndrome Granulomatous Slack Skin Lymphomatoid papulosis
Mycosis Fungoides
Malignancy of T-lymphocytes, almost always MEMORY T-CELL Black>white M:F = 2:1 Race: MF is more common in black than in white patients (incidence ratio 1.6). Sex: MF occurs more frequently in men than in women (male-to-female ratio of 2:1). Age: The most common age at presentation is 50 years; however, MF also can be diagnosed in children and adolescents with apparently similar outcomes.
Mycosis Fungoides
Patch Stage premycotic, severe pruritis. Plaque Stage infiltrated plaque Tumore stage when de novo, called d emblee form Erythroderma Rare
MF Staging
TNMB system on skin (T) node (N), viscera (M), and blood (B)
MF Staging
N0 normal nodes N1 palpable but not pathologically MF N2 not palpable but pathologically MF N3 clinically and pathologically involved
MF Staging
Stage IA T1, N0, M0 8-9% progress Stage IB T2, N0, M0 11-16 years surv Stage IIA T1-2, N1, M0 7.7 years Stage IIB T3, N0-1, M0 3-8 years surv Stage IIIA T4, N0, M0 1.8-3.7 years Stage IIIB T4, N1, M0 1.8-3.7 years Stage IVA T1-4, N2-3, M0 Stage IVB T1-4, N0-3, M1
Lymph nodes in MF
Extracutaneous involvement is more clinically evident as the stage and extent of MF increases Peripheral lymphadenopathy is the most frequent site of extracutaneous involvement in MF Evaluate palpable lymphadenopathy by obtaining a biopsy because the result influences the patients stage, prognosis, and treatment.
MF Workup
CBC to review the buffy coat smear for Lymph nodes CMP Liver Function to include LDH (aggressive) and transaminase (liver involvement) values CXR If lymph nodes are palpable
CT
Histologic Findings
The criteria for diagnosis include the following: A bandlike upper dermal infiltrate of lymphocytes and other inflammatory cells, with no grenz zone, is present. Epidermotropism of mononuclear cells occurs. When a clear halo surrounds an intraepidermal mononuclear cell singly or in clumps, this is called a Pautrier microabscess. Its presence is suggestive of MF, but it is not necessary for diagnosis. Little spongiosis of the epidermis is found. Lymphocytes have nuclei that are hyperchromatic and convoluted or cerebriform.
Pagetoid Reticulosis
Localized epidermotropic reticulosis Woringer-Kolopp disease Acral mycoses fungoides Mycosis fungoides palmaris et plantaris
Pagetoid Reticulosis
0.6% of all MF cases Woringer-Kolopp variant: solitary lesion Ketron-Goodman variant: multiple lesions Long durantion without progression to frank lymphoma is the clincal hallmark of WoringerKolopp Local excision and radiation therapy maybe curative.
Medium power view. Keratinocyte enlargement is seen, and this can occur whenever there is an abnormal cell population in the lower epidermis. Clusters of dark mononuclear cells are in all levels of the epidermis.
High power view. It would be difficult to distinguish between abnormal T cells and small, dark, lymphocytoid melanocytes in this field.
Band-like infiltration of lymphoid cells in lower epidermis and upper dermis. Intraepidermal infiltrate were medium- to large-sized atypical cells. Lymphoid cells infiltrating upper dermis revealed no overt atypicality.
Sezary Syndrome
Leukemic phase of mycosis fungoides Generalized erythroderma, superficial lymphadenopathy, atypical cells in circulating blood Erythroderma from onset with leonine facies, eyelid edema, ectropion, alopecia, palm and sole hyperkeratosis Pruritis, burning, chill and profuse sweating
Prognosis
Difficult to treat Median survival is 3 years Low dose methotrexate has reasonable response rate of 50% Photophoresis Retinoid, interferon alfa, lowdose chlorabucil, prednisone, systemic chemo
Lymphomatoid Papulosis
LyP has a chronic indolent course in most patients; estimates indicate that as many as 10-20% of LyP patients have a history of associated malignant lymphoma (ALCL, HD, or mycosis fungoides [MF]) prior to, concurrent with, or subsequent to the diagnosis of LyP. Race: Black persons may be less affected than other racial groups. Sex: No consistent sex predominance is found in studies. Age: LyP may develop at any age, usually in the third to fourth decade
Presentation
Primary lesion: Each erythematous papule evolves into a red-brown, often hemorrhagic, papulovesicular or papulopustular lesion over days to weeks. Some lesions develop a necrotic eschar before healing spontaneously. Occasionally, noduloulcerative lesions may be present Each papule heals within 2-8 weeks, leaving a hypopigmented or hyperpigmented depressed oval varioliform scar. Large nodules and plaques may take months to resolve. Distribution: Characteristically, lesions appear on the trunk and extremities, although the palms and/or soles, face, scalp, and anogenital area also may be involved.
Lymphomatoid Papulosis
Type A: Characterized by large (25-40 mm) CD30+ atypical cells with polymorphic convoluted nuclei and a minimum of 1 prominent nucleolus. These large cells resemble Reed-Sternberg cells when binucleate. Type A LyP is the most common histologic variant. Type B: Characterized by smaller (8-15 mm) atypical cells with hyperchromatic cerebriform nuclei resembling the atypical lymphocytes in MF. CD30+ large cells are rare, but epidermotropism is more common in this variant.
Lymphomatoid Papulosis
Type C (diffuse large cell type): Characterized by sheets of CD30+ anaplastic large cells
Treatment of LyP
mid-to-high potency topical steroids to hasten resolution. Low-dose weekly methotrexate is a safe and effective treatment for suppressing LyP; however, the disease recurs within 1-2 weeks after ending medication. Oral psoralen plus UV-A phototherapy (PUVA) also effectively treats and suppresses the disease. carmustine, topical nitrogen mustard, intralesional interferon, low-dose cyclophosphamide, chlorambucil, and dapsone help disease suppression.
CD30-positive cutaneous T-Cell Lymphoma Secondary Cutaneous CD30 positive large-cell lymphoma Non-MF CD30-negative cutaneous large T-cell lymphoma Non-MF CD30-negative cutaneous pleomorphic small or medium sized cell lymphoma Subcutaneous (Panniculitis-Like) T-Cell Lymphoma Nasal/Nasal Type T/NK Cell Lymphoma
A, Marked edema of right calf at time of presentation. B, Erythematous nodules with associated vascular ectasia on abdominal wall.
AKA B-Cell lymphoma of follicular center cell origin AKA Reticulohistiocytoma of the dorsum Multiple papules and nodules in one anatomic region. 2/3 of case on the trunk, 1/5 on the head and neck 15% on the leg
Dense mononuclear infiltrate within entire dermis. This represent a primary cutaneous plasmacytoma
Cutaneous
alopecia Raynauds Pyoderma
vasculitis
gangrenosum
Hodgkins Disease
Vast majority of cutaneous Hodgkins disease report are type A lymphomatoid papulosis with Reed-Sternberg Difficult to prove cutaneous disease
Malignant Histiocytosis
Rare, fatal occur in men in second to fourth decade of life Solitary lesion or wide spread papule occur in 10% of cases Onset of acute fever, hepatosplenomegly, and painful lymphadenopathy
Leukemia Cutis
30% of Leukemia patient will have leukemia cutis Vast majority of derm manifestation are from AML Morphology: 60% multiple papules and nodules, 26% of infiltrated plaques Subtypes and variants:
Granulocytic
Cutaneous Myelofibrosis
Overproduction and premature death of atypical megakaryocytes in bone marrow Inrease in platelet-derived growth factor Extramedullary hematopoesis is the hallmark Blast cells escape marrow and enter circulation and form tumor Cutaneous EMH reported in 20 cases
Hypereosinophilic Syndrome
Icrease Eos with more than 1500 eos per cubit millimeter for 6 month or more. Cardiac disease most frequent complication 90% patient are men between 20 to 50 Angioedema and urticaria lesions most common. Sometimes papules.
Polycythemia Vera
Increase hermatocrite to 55% to 80% Associated with aquagenic pruritis in 50% of the patients. Elevation of blood and skin histamine Tx control of pruritis by antihistamin or PUVA. Referral to HemOnc