Fascin expression in CD30-positive cutaneous lymphoproliferative disorders

BACKGROUND: CD30-positive cutaneous lymphoproliferative disorders (LPD) represent a spectrum of diseases ranging from low-grade (lymphomatoid papulosis; LyP) to high-grade (pleomorphic and anaplastic large-cell lymphoma; PTL, ALCL) with overlapping morphologic and immunophenotypic features. The common phenotypic hallmark is the expression of CD30-antigen by the tumor cells which morphologically resemble Reed-Sternberg cells. Although LyP is a non-fatal recurring disorder, it is associated with systemic lymphomas including Hodgkin's lymphoma (HL), mycosis fungoides (MF) and ALCL in 5-20% of the cases. Currently there is no marker to predict the development of systemic lymphomas in patients with LyP. Fascin, an actin bundling protein, has recently been shown to be a unique marker found in almost 100% of classical HL. METHODS: Because of the association of LyP with HL, fascin expression was analyzed by immunohistochemistry in LyP (n = 45), cutaneous CD30+ ALCL (n = 17) and pleomorphic T-cell lymphoma (n = 9) (PTL) and LyP associated with systemic lymphomas (7 HL, 2 ALCL, 1 MF), with the intent to determine if fascin expression can predict disease progression. RESULTS: Fascin was expressed by tumor cells in 11/45 (24%) cases of LyP, 11/17 (64%) cases of ALCL, 7/9 (77%) cases of PTL and 6/10 (60%) cases of LyP associated with systemic lymphomas. Fascin expression in LyP was significantly less frequent than in ALCL (p < 0.001) and also than in LyP associated with lymphomas (p < 0.05). There was no significant difference of fascin expression within the histological subtypes of LyP. We found no evidence of ALK expression nor of Epstein-Barr virus expression in any case either by in situ hybridization or immunohistochemistry in the LyP cases associated with HL. CONCLUSIONS: This is the first study to demonstrate that fascin is expressed in cutaneous CD30+ LPD and that it is a candidate marker of disease progression in LyP.     

Is there a special relationship between CD30‐positive lymphoproliferative disorders and epidermal proliferation?

The relationship between CD30+ lymphoma and epithelial proliferations is not well defined. CD30+ lymphoma and lymphomatoid papulos (LyP) share immunohistochemical epitopes and some other features. A single case of LyP associated with multiple keratoacanthomas (KAs) was recently reported. We report two cases of atypical lymphocytic proliferation with features of CD30+ lymphoma and LyP intimately associated to KA and squamous cell carcinoma (SCC), KA type. This similar combination of an epidermal tumor and apparent involvement with atypical lymphocytic infiltrates raises the possibility of an association between the two entities. We speculate that the association may be more than expected to occur by chance and suggest several mechanisms by which the association may evolve.     

Cutaneous CD30 positive lymphoproliferative disorders with coexistent epithelial neoplasms: Report of two cases

Primary cutaneous large cell anaplastic lymphoma (C‐ALCL) and lymphomatoid papulosis (LyP) are cutaneous CD30+ lymphoproliferative disorders (CD30+ LPD). An association with CD30+ LPD and pseudoepitheliomatous hyperplasia has been recognized. Additionally, rare reports of epithelial neoplasms such as keratoacanthomas and squamous cell carcinomas (SCC) occurring in association with both C‐ALCL and LyP have been reported. We report two cases of CD30+ LPD with associated epithelial neoplasms; one patient with a primary cutaneous CD30+ LPD and SCC identified within the same lesion, and the other with a keratoacanthoma arising in a lesion of LyP. The pathogenesis of this association is poorly understood although various hypotheses exist. Awareness of the coexistence of these entities will avoid misdiagnosis and incorrect treatment.     

Lymphomatoid papulosis in association with mycosis fungoides: A clinical and histopathologic review of five Taiwanese cases

Background/ObjectivesLymphomatoid papulosis (LyP) is a cutaneous CD30+ lymphoproliferative disorder characterized by recurrent, self-healing lesions with a chronic clinical course. Approximately 10–20% of the patients have lymphomas, including mycosis fungoides (MF). LyP in association with MF is not well documented in Taiwan. We aimed to describe the clinicopathologic characteristics of LyP with MF in a Taiwanese case series of LyP.MethodsA retrospective clinicopathologic study was performed on cases of LyP with MF diagnosed in our Department during the period 1990–2012. The diagnosis of LyP and MF were based on their characteristic clinical and pathologic features as well as correlation with the clinical course of the specific skin lesions.ResultsA total of 24 cases of LyP (10 males and 14 females, age 18–63 years, mean 40.4 years) were included. Multiple biopsies were often done in individual patients during the clinical course to establish the diagnosis of LyP and MF. LyP was further classified pathologically as type A (n = 16), B (n = 3), C (n = 3), and mixed type with A&B (n = 1) and A&C (n = 1). Five cases (21%) also had MF; two had juvenile-onset LyP and three had juvenile-onset MF (one with hypopigmented MF, one with hyperpigmented MF, two with CD8+ LyP, and two with CD8+ MF). In the case of juvenile-onset hypopigmented CD8+ MF, the patient developed CD8+ LyP 25 years after the onset of MF and died of aggressive epidermotropic CD8+ lymphoma involving the skin and lung.ConclusionMF occurred in five of the 24 cases (21%) in the present series of LyP. These five cases had several unusual clinical and pathologic features, including subtle or uncommon skin manifestation of MF and more frequent juvenile-onset and CD8 phenotype of LyP and/or MF lesions. Long-term follow-up and repeated biopsy of selected skin lesions are necessary for correct diagnosis and proper treatment of both diseases.     

Epstein-Barr virus in CD30 anaplastic large cell lymphoma involving the skin and lymphomatoid papulosis in South Korea

BACKGROUND: Epstein-Barr virus (EBV)-associated cutaneous lymphoproliferative disorders are prevalent in Asia, and less frequent in Western countries. AIM: To elucidate the possible association of EBV with CD30+ anaplastic large cell lymphoma (ALCL) involving the skin and lymphomatoid papulosis (LyP) in South Korea. METHODS: In situ hybridization for EBV-encoded small RNA (EBER) and immunohistochemistry including viral latent membrane protein-1 (LMP-1) were performed on formalin-fixed, paraffin-embedded skin specimens of 26 cases of LyP and 16 cases of CD30+ ALCL involving the skin which were selected from six university hospital medical centers in South Korea. RESULTS: In situ hybridization studies showed positivity of the neoplastic cells for EBER in two of 16 cases of CD30+ ALCL and in none of the cases of LyP. One EBER-positive case was cutaneous CD30+ ALCL with concurrent lymph node involvement. The other was CD30+ ALCL involving the skin and other organs, including lymph nodes, bone, lung, and spleen. Immunostaining for LMP-1 was also positive only for the two cases of EBER-positive CD30+ ALCL. CONCLUSION: LyP and primary cutaneous CD30+ ALCL are very rarely associated with EBV in South Korea.     

Lymphomatoid papulosis localized to the face

We report a case of lymphomatoid papulosis (LyP) localized to the face. Only ten cases of localized LyP have been reported, and those involved the extremities. Three of those cases finally evolved to classic LyP with a widespread distribution of the lesions, the other cases remain limited. The evolution into lymphoma for the localized form appears to be equivalent to that reported with the generalized LyP (20 %), underlining the need for long-term followup of these patients. This is the first reported case of LyP localized to the face.     

Lymphomatoid papulosis in children: a retrospective cohort study of 35 cases

BACKGROUND: Lymphomatoid papulosis (LyP) is a rare entity, considered to be part of the spectrum of the CD30(+) cutaneous lymphoproliferative disorders. About 10% to 20% of the adult LyP patients will develop an associated lymphoid malignancy. Only a few cases of LyP have been described in children, and the risk of associated lymphoid malignancies in these patients is not known. OBJECTIVES: To study the association between childhood onset of LyP and other malignancies and to determine the clinical characteristics in this subgroup of patients. DESIGN: Retrospective cohort study. SETTING: Referral center at a university hospital. Retrospective registry for patients with LyP of childhood onset (< or =18 years).Patients Thirty-five patients with childhood-onset LyP (19 boys and 16 girls) were interviewed by telephone using a standardized questionnaire. The median duration of follow-up was 9.0 years. All included patients were confirmed by histologic examination. RESULTS: The age distribution was significantly different, with boys having an earlier onset of LyP (P =.03). Of the 35 LyP patients, 3 (9%) developed a malignant lymphoma; all were diagnosed as having non-Hodgkin lymphoma. Compared with the general population, patients with childhood-onset LyP have a significantly increased risk of developing non-Hodgkin lymphoma (relative risk, 226.2; 95% confidence interval, 73.4-697.0). More than two thirds of the patients reported being atopic, which is significantly more than the expected prevalence of atopy (relative risk, 3.1; 95% confidence interval, 2.2-4.3). CONCLUSIONS: Lymphomatoid papulosis presents similarly in children and adults, including the risk of lymphoid malignancies. Therefore, all LyP patients should be closely monitored throughout their lives.     

Nitrogen mustard gel-induced inflammation triggers lymphomatoid papulosis in patients with mycosis fungoides

Lymphomatoid papulosis (LyP) is a paraneoplastic primary cutaneous CD30⁺ lymphoproliferative disorder (LPD) that has been associated with malignant lymphomas, most commonly mycosis fungoides (MF). We observed 10 patients with MF who developed severe inflammation after using nitrogen-mustard (NM) gel from 1 to 8 months and who developed LyP. We hypothesized that NM gel produced local inflammation, which induced CD30 expression in malignant T cells in situ leading to the appearance of LyP papules. The high frequency of induction of LyP lesions in patients with severe inflammation while on treatment with NM gel suggests an association between inflammatory stimuli and development of LyP. Our observation provides insight into the pathogenesis of CD30⁺ LPD.     

Lymphomatoid papulosis associated with mycosis fungoides: a study of 21 patients including analyses for clonality

BACKGROUND: Although an association of lymphomatoid papulosis (LyP) with mycosis fungoides (MF) is recognized, our understanding of this relation is limited. OBJECTIVE: We sought to document the clinical experience at the University of California, San Francisco, in 21 patients who had both LyP and MF and to do clonality studies in 7 of those patients in whom this was possible. METHODS: We conducted chart review of the 21 patients and analysis for T-cell receptor-gamma gene rearrangements by the polymerase chain reaction. RESULTS: Of 54 patients, 21 (39%) with LyP had associated MF. LyP preceded MF in 14 (67%), MF preceded LyP in 4 (19%), and there was concurrent appearance in 3 (14%). Of the 21 patients, 20 (95%) were type A and only 1 (5%) was type B. An identical clone was found in lesions of both LyP and MF in all 7 patients in whom analysis was possible. CONCLUSION: Findings of this study strengthen the idea that LyP and MF are related T-cell lymphoproliferative disorders.     

Lymphomatoid papulosis and human herpesviruses--A PCR-based evaluation for the presence of human herpesvirus 6, 7 and 8 related herpesviruses

BACKGROUND: Lymphomatoid papulosis (LyP) is a chronic, recurrent lymphoproliferative disorder of the skin that belongs to the group of primary cutaneous CD30-positive T-cell lymphomas. Ultrastructural and clinical features of LyP suggest that it has a viral etiology. Human herpesviruses have been proposed as causative cofactors for LyP because of their oncogenic potential and their association with other lymphomas. METHODS: LyP skin lesions and a LyP-derived cell line were examined for the presence of the recently discovered oncogenic human herpesvirus 8 (HHV-8) and the two T-lymphotropic human herpesviruses 6 and 7 (HHV-6 and HHV-7) by nested polymerase chain reaction (PCR) using virus-specific oligonucleotide primers. Furthermore, a recently described method involving degenerate PCR primers was applied to detect highly conserved DNA sequences shared by a variety of herpesviruses, especially oncogenic gamma-herpesviruses, in an attempt to identify a yet undiscovered herpesvirus associated with LyP. RESULTS: HHV-6 and 8 could not be found in 26 archival and 11 snap-frozen LyP lesions and a LyP tumor cell line. HHV-7 DNA sequences were detected in 14% (5 of 37) of LyP samples. HHV-6 was found in 23% (3 of 13) and HHV-7 in 8% (1 of 13) of normal skin samples from healthy individuals, respectively. Using degenerate PCR primers to amplify the highly conserved polymerase region of herpesviruses, no DNA sequences related to human herpesviruses could be detected. CONCLUSIONS: LyP is not associated with HHV-6, HHV-7 and HHV-8. In addition, the studies using degenerate PCR primers do not indicate the presence of a previously undescribed human herpesvirus in LyP.     

Lymphomatoid papulosis: is a second lymphoma commoner among East Asians?

Background. Lymphomatoid papulosis (LyP) is a low‐grade cutaneous lymphoma, which lies within the spectrum of primary cutaneous CD30‐positive lymphoproliferative disorders. Around 10–20% of LyP cases are associated with a second lymphoma. Aim. To analyse a cohort of Asian patients with LyP, diagnosed from 1987 to 2007 at the National Skin Centre (NSC), Singapore, in terms of epidemiology, treatment and association with a second lymphoma. Methods. Patients were identified through the NSC clinical and histological databases. Results. During this period, 13 patients were diagnosed with LyP based on clinicopathological criteria. The mean age at diagnosis was 41 years, the male : female ratio was 2.3 : 1, and 92% of the patients were Chinese. Recurrent papulonecrotic lesions were present for a mean of 3 years before diagnosis. Treatment of LyP comprised monotherapy (n = 4) or combination therapy (n = 9) using corticosteroids, oral antibiotics, methotrexate and/or phototherapy. Mean duration of follow‐up was 6.4 years. Eight patients (61.5%) were diagnosed with a second lymphoma, either before (n = 2), concurrently with (n = 1) or after (n = 5) the diagnosis of LyP. Mycosis fungoides (MF) was the commonest lymphoma (78%, n = 7), followed by primary cutaneous anaplastic large‐cell lymphoma (12%, n = 2). There was one death (mortality rate 7.7%), which occurred in a patient who had developed stage IIA MF after LyP, which subsequently progressed to systemic T‐cell lymphoma. Conclusions. LyP is a chronic, relapsing disease with considerable morbidity, but an overall good prognosis. A strikingly large proportion of our Asian patients (61.5%) had a second lymphoma, compared with previous studies. This emphasizes the importance of regular lifetime surveillance for associated lymphomas in all patients with LyP.     

Follicular lymphomatoid papulosis with follicular mucinosis: a clinicopathologic study of 3 cases with literature review and conceptual reappraisal

Lymphomatoid papulosis (LyP), characterized by recurring, waxing and waning, cutaneous papulonodules, is increasingly recognized to represent a heterogeneous collection of pathologically dissimilar subtypes. Recently, a follicular LyP variant was proposed, featuring folliculotropism. Folliculotropism by atypical lymphocytes is conventionally associated with follicular mucinosis and mycosis fungoides (MF), and review of the literature suggests co‐occurrence of folliculotropism and follicular mucinosis in LyP to be rare, with only 3 cases identified to date. Herein we describe 3 additional cases, each manifesting a typical LyP clinical picture, with the additional element of folliculotropism and follicular mucinosis on pathology. These cases suggest that LyP should be considered alongside MF in the differential diagnosis of follicular mucinosis with accompanying atypical lymphocytic infiltration. As LyP can occur with other lymphoproliferative disorders such as MF, the finding of follicular mucinosis in LyP may further represent a conceptual intersection between the 2 disease processes.     

Histopathological aspects and differential diagnosis of CD8 positive lymphomatoid papulosis

Lymphomatoid papulosis (LyP) belongs to CD30+ lymphoproliferative disorders with indolent clinical course. Classic histological subtypes, A, B and C are characterized by the CD4+ phenotype, while CD8+ variants, most commonly classified as type D, were reported in recent years. We present 14 cases of CD8+ LyP. In all patients, self‐resolving or treatment‐sensitive papules were observed. Of 14 cases 7 produced results with typical microscopic features of LyP type D mimicking primary cutaneous aggressive epidermotropic CD8+ T‐cell lymphoma. The infiltration pattern in 4 of 14 cases were consistent with classic LyP type B, without CD30 expression in two cases, resembling mycosis fungoides (MF). The morphology of 2 of 14 cases shared a certain consistency with classic type A and C, lacking eosinophils and neutrophils. Extensive folliculotropism characteristic to type F was observed in 1 of 14 case. Significant MUM1 and PD1 expression were detected in 2 of 14 and 3 of 14 cases, respectively. We concluded that CD8+ LyP may present with different histopathological features compared with type D, similar to CD4+ LyP variants. Differential diagnoses include CD8+ papular MF, folliculotropic MF and anaplastic large cell lymphoma in addition to primary cutaneous aggressive epidermotropic T‐cell lymphoma. We emphasise that rare CD8+ LyP cases may exist with CD30‐negativity.     

Immunophenotypic and genotypic characterization of lymphomatoid papulosis

BACKGROUND: Lymphomatoid papulosis (LyP) is a chronic dermatosis that histologically resembles malignant lymphoma. Thus far, only a few cases of LyP have been characterized in detail with regard to immunophenotype, genotype, and karyotype. OBJECTIVE: Our purpose was to study seven patients with LyP and compare the results to those reported in the literature. METHODS: Skin biopsy specimens were analyzed by frozen section immunohistochemical and molecular biologic techniques. Cytogenetic analysis was also performed in three cases. RESULTS: The atypical lymphoid cells consisted of activated helper T cells; four of the seven patients had lesions with a detectable clonal T-cell population. A peripheral T-cell lymphoma developed in one patient before the emergence of a genotypically different LyP T-cell clone. Cytogenetic studies were abnormal in one case of LyP and normal in another, whereas the karyotype of the lymphoma was abnormal. CONCLUSION: LyP is a preneoplastic proliferation of activated helper T cells, which is often clonal and may regress and expand with the development of new LyP clones or lymphoma.     

Cutaneous CD30‐positive lymphoproliferative disorders with CD8 expression: a clinicopathologic study of 21 cases

Lymphomatoid papulosis (LyP) and cutaneous anaplastic large cell lymphoma (ALCL) belong to the spectrum of cutaneous CD30+ lymphoproliferative disorders, an indolent form of T‐cell lymphoproliferative disease. We reviewed 21 cases of CD30+ lymphoproliferative lesions expressing cytotoxic profile (CD8+). Seven cases of cutaneous ALCL, 2 cases of systemic ALCL involving the skin, and 12 cases of LyP. The cases of LyP were predominated by small lymphocytes exhibiting a prominent epidermotropic pattern consistent with either type B or type D LyP. Four cases showed co‐expression of CD56. The ALCL cases included myxoid features, pseudoepitheliomatous change, and an intravascular component. In all cases that were primary in the skin an indolent clinical course was seen while one patient with systemic myxoid ALCL is in remission following systemic multiagent chemotherapy. The paucity of other neutrophils and eosinophils and concomitant granulomatous inflammation were distinctive features in cases of type B and type D LyP. CD30 and CD45 Ro positivity and a clinical course typical of LyP were useful differentiating features from an aggressive cytotoxic CD8+ T cell lymphoma. In all cases that were primary in the skin an indolent clinical course was observed. CD30 and CD45 Ro positivity and a clinical course typical of LyP were useful in preventing a misdiagnosis of an aggressive cytotoxic CD8+ T cell lymphoma.     

New variant lymphomatoid papulosis type E preceding and coexisting with mycosis fungoides – a case report and review of the literature

Angioinvasive (type E) lymphomatoid papulosis (LyP) is a recently described subtype of LyP presenting with eschar‐like lesions that can be mistaken for aggressive forms of angiocentric cutaneous T‐cell lymphoma. None of the cases of angioinvasive LyP described thus far have been associated with mycosis fungoides (MF). Herein, we describe a case of angioinvasive LyP type E coexisting with MF. The patient presented with an eschar on his chest and over time developed new nodules and large plaques with eschar formation, all of which resolved spontaneously over a period of a few weeks without intentional therapy. Biopsy revealed a CD30+ atypical inflammatory cell infiltrate with marked angiocentricity. Later, he developed erythematous annular scaly patches histologically consistent with MF. Our patient's clinical course confirms the indolent behavior characteristic of LyP despite the aggressive clinical and histologic appearance of lesions. The co‐occurrence of angioinvasive LyP and MF in our patient highlights the propensity for LyP type E to coexist with MF, as is characteristic of other LyP subtypes, and supports the theory that LyP and MF are related T‐cell lymphoproliferative disorders. Patients with LyP can present with large lesions exhibiting eschar formation and an atypical angiocentric/angiodestructive lymphoid infiltrate and should be spared overtreatment.     

Contribution of longitudinal follow up and clinical pathological correlation in the diagnosis CD30‐positive skin infiltrates

The diagnosis of a CD30+ cutaneous infiltrate is often difficult and requires clinicopathologic correlation. To further evaluate this challenge, initial clinical and histopathologic diagnoses were correlated with final clinicopathologic diagnosis in 44 cases with CD30 immunopositivity. Dermatopathologic evaluation confirmed the initial clinical diagnosis in 65% of the suspected benign cases, all cases of suspected lymphomatoid papulosis (LyP), and 72% of clinically malignant cases. In the 25 patients with clinical suspicion for lymphoma, the histopathologic diagnoses included lymphoma in 18, LyP in 2, CD30+ lymphoproliferative disorder (CD30 LPD) in 3 and hypersensitivity reaction in 2 patients. Clinicopathologic correlation led to a change in three cases diagnosed histopathologically as anaplastic large cell lymphoma (ALCL) reclassified as LyP type C, and one patient diagnosed as CD30 LPD clinically evolved as herpes virus infection. Furthermore, five cases reported as CD30 LPD received more specific diagnoses after clinicopathologic correlation (LyP type C in three, and ALCL in two patients). Clinicopathologic correlation is essential in establishing the correct diagnosis of CD30 LPD, in particular the distinction of ALCL from LyP type C. In this setting, the histopathologic diagnosis of CD30 LPD is advisable in the absence of clinical data.     

Differential expression of TRAF1 aids in the distinction of cutaneous CD30-positive lymphoproliferations

Lymphomatoid papulosis (LyP), primary cutaneous anaplastic large T-cell lymphoma (cALCL), and cutaneous infiltrates of systemic anaplastic large cell lymphoma (sALCL) are CD30-positive lymphoproliferative disorders of the skin that overlap clinically, histopathologically, immunophenotypically, and genetically but differ considerably in their prognosis. In particular, lesions of LyP regress spontaneously, whereas those of cALCL and sALCL persist and may progress and spread to extracutaneous sites. In contrast to patients with cALCL, LyP patients do not benefit from an aggressive radio- and/or chemotherapeutic approach. We generated a novel tumor necrosis factor receptor (TNFR)-associated factor 1 (TRAF1) antibody that recognizes a formalin-resistant epitope (Ber-TRAF1A) and investigated the expression of TRAF1, an intracellular component of TNFR signaling, in LyP and ALCL. We could show a strong TRAF1 expression in the tumor cells of most LyP cases (42/49, 84%). In contrast, tumor cells of primary and secondary cALCL revealed TRAF1 expression in only a few cases (3/41, 7%) as shown for sALCL without skin manifestation. The data indicate that TRAF1 expression reliably distinguishes LyP from primary or secondary cALCL. This might be of crucial diagnostic importance and has a strong impact on the treatment decision for patients with cALCL and LyP.     

Lymphomatoid papulosis: a study of 18 cases*

Lymphomatoid papulosis (LyP) is a cutaneous eruption that is clinically benign but histologically malignant. To date, more than 300 cases have been published. About 10–20% of the patients develop a lymphoma. The purpose of this study was to make a clinicopathological study of 18 patients diagnosed with LyP in our hospital from 1973 to 1990, to characterize cellular infiltrates in the lesions, to find clonal populations of T-cells and to look for predictive factors of malignant lymphoma in LyP patients. Mean age was 48.7 years. The most frequent clinical lesions were papules (88.8%) followed by plaques (38.8%). The localizations were on extremities (100%), trunk (88%), face (22%), palms or soles (11%), perigenital (11%) and scalp (5%). Two patients have been free of disease for more than 5 years. IgA levels are increased in LyP patients. Neither HTLV I nor III can be considered as a cause of the LyP in any of our patients. Associated diseases were found in 6 cases (1 mycosis fungoides, 1 Hodgkin's disease, 2 anaplastic large-cell lymphoma and 2 large plaque parapsoriasis). Some types of parapsoriasis should be included in the ‘spectrum of Ki-1 lymphomas’. 52 skin biopsies were studied. 17% were type A of Willemze, 67% were type B and 15% were transitional. In 12 of the samples follicular or perifollicular infiltration was found. Follicular LyP should not be considered as a distinct type of LyP. Vasculitis is an uncommon finding in LyP. In all the cases studied, large atypical cells were CD30 +; 5/7 cases had lost CD5 and 4/5 cases had lost CD7. In one case, all T-cell antigens were negative. Cerebriform mononuclear cells were always recognized by T-cell antibodies and they were CD30 positive in only two cases. In one case there were more CD8 + than CD4 + cells. In 5 patients skin and blood samples for genetic rearrangement (beta-T) were taken. Only germinal line was found. We did not find any significant difference between those cases in which malignant lymphoma developed and those in which it did not.     

Iron deficiency and hair loss: the jury is still out

We report 3 cases of lymphomatoid papulosis (LyP) with a CD56+, cytotoxic immunophenotype. All 3 patients presented with clinical histories typical of LyP, with one patient having associated mycosis fungoides. Histologically, two cases were type A LyP and one was type B. All 3 cases demonstrated a T-cell receptor clone in lesional skin without evidence of blood involvement. The atypical lymphocytes in each of the 3 cases expressed cytotoxic granules (T-cell intracellular antigen-1+ and granzyme B+) and were CD8+ and CD56+. Expression of CD56 is associated with a poor prognosis in subcutaneous panniculitis-like T-cell lymphoma and blastic natural killer cell lymphoma. However, the two cases of CD56+ LyP previously reported and the 3 cases in this series all appear to be pursuing an indolent course with no evidence of systemic disease.