Cutaneous T‐cell lymphoma and atopy: is there an association?

BACKGROUND: Case reports have suggested a relationship between atopic diatheses and Sézary syndrome, pre-Sézary syndrome or mycosis fungoides. However, Sézary and pre-Sézary syndromes are rare entities, and this association has never been analysed in greater detail for specific subtypes of cutaneous T-cell lymphoma (CTCL). OBJECTIVES: To evaluate the prevalence of atopy in subjects with Sézary syndrome, pre-Sézary syndrome or mycosis fungoides, and to compare the rates with the reported prevalence of atopy in the general population. METHODS: We retrospectively reviewed the records of 157 patients with the diagnosis of Sézary or pre-Sézary syndrome seen between 1965 and 2000, and 102 patients with the diagnosis of mycosis fungoides evaluated from 1994 to 2000 at Mayo Clinic. RESULTS: Of 157 subjects with Sézary or pre-Sézary syndrome and 102 subjects with mycosis fungoides, 18 and 12, respectively, were identified as having a history of atopic dermatitis, asthma or allergic rhinitis. The prevalence rates of atopy in Sézary or pre-Sézary syndrome and mycosis fungoides were 11.5% (95% confidence interval 6.9-17.5%) and 11.8% (6.2-19.7%), respectively. CONCLUSIONS: No significant difference exists in the prevalence of atopy in Sézary or pre-Sézary syndrome compared with that in mycosis fungoides (chi2-test, P = 1.00). Furthermore, the rates of atopy in Sézary or pre-Sézary syndrome and mycosis fungoides are not significantly different from the prevalence of atopy in the general population (17-40%). On the basis of these observations, no evidence currently implicates a causal association of CTCL with atopy.     

CD56 staining in Merkel cell carcinoma and natural killer‐cell lymphoma: magic bullet,diagnostic pitfall,or both?

Background: Antibodies to CD56 label natural killer (NK) cell lymphomas and neuroendocrine tumors such as Merkel cell carcinoma (MCC). In MCC altered by crush artifact or obscured by lymphocytes, the histologic features coupled with CD56 positivity can lead to an erroneous impression of NK‐cell lymphoma. Methods: Eighteen cases of MCC were stained for CD56, CK20, MNF116, and pankeratin. The results were compared to histologic features and CD56 staining pattern of three NK‐cell lymphomas. Results: Three of 18 cases of MCC histologically resembled lymphoma, and CD56 positivity with CD3 and CD20 negativity initially raised the possibility of NK‐cell lymphoma. Two additional cases were obscured by dense inflammation, again suggesting the diagnosis of lymphoma. Seventeen of 18 MCC labeled for CD56 and an equal number stained for CK20. All MCC tested were positive for CAM5.2 (14/14) and MNF116 (17/17). Antibodies to pankeratin labeled only one of 18 MCC. Staining for CD56 was stronger in MCC than NK‐cell lymphomas. Conclusions: CD56 is a sensitive marker for MCC as well as for NK‐cell lymphoma, but is not specific. Importantly, CD56 positivity in crushed or inflamed biopsies of MCC may lead to an erroneous impression of NK lymphoma. Awareness of this potential pitfall will prevent misdiagnosis.     

Rate of growth—A novel surrogate marker for high‐risk cutaneous squamous cell carcinoma? A case report and review of the literature

Cutaneous squamous cell carcinoma (cSCC) is one of the most common nonmelanoma skin cancer worldwilde, with a more invasive growth pattern and higher potential to metastatize than basal cell carcinoma. Although several risk factors have been linked to a high metastatic potential of cSCC, no widely accepted classification system for this common subtype of cancer exists. Herein we report an emblematic case of rapidly growing and metastatic cSCC and discuss the rate of growth of the tumour (ROG) as novel prognostic high risk surrogate marker.     

Fondaparinux: a suitable alternative in cases of delayed‐type allergy to heparins and semisynthetic heparinoids? A study of 7 cases

Hypersensitivity to unfractionated and low-molecular-weight heparins and semisynthetic heparinoids is increasingly common. 7 female patients between 30 and 74 years with delayed-type allergy to heparins and semisynthetic heparinoids were investigated for (cross)-reactivity to fondaparinux, a new pentasaccharide with selective factor Xa inhibition. All patients showed delayed-type reactions to heparins and some additional cross-reaction to a heparinoid on intracutaneous testing. 6/7 tolerated fondaparinux on intradermal testing as well as on subcutaneous challenge testing. However, the 7th patient developed a characteristic delayed-type reaction to both skin tests with fondaparinux. Fondaparinux is a new synthetic pentasaccharide with a molecular weight of 1.728 Da. In some patients with cross-reactivity between various heparins and semisynthetic heparinoids, lepirudin, a recombinant hirudin, may be a safe and effective alternative. However, combined allergy to hirudin and heparins has been reported. Sometimes, intravenous administration of heparins or heparinoids may be tolerated. However, these patients are at risk of developing a systemic reaction. The pathogenesis of heparin hypersensitivity is not fully understood. Heparins may act as haptens by binding to dermal and/or subcutaneous structural proteins. The chemical structures of heparins and fondaparinux are different concerning their alpha- and beta-configuration and the molecular weight. However, some of their functional groups are nearly identical and therefore similar chemical and pharmacological reactivity is to be expected. Fondaparinux seems to be a valuable alternative in most cases of heparin and hirudin hypersensitivity. The clearly rare cross-reaction between fondaparinux and heparins, now confirmed by us, may be due to differences in the response to haptens.     

Histiocytoid and signet‐ring cell carcinoma of the axilla: a type of cutaneous apocrine carcinoma equivalent to histiocytoid lobular carcinoma of the breast?

There is a histopathological similarity between cutaneous apocrine carcinoma (CAC) and breast carcinoma. Cutaneous histiocytoid or signet-ring cell (SRC) carcinoma is a rare neoplasm, which usually occurs on the eyelid, and less commonly on the axilla. The precise histogenesis of this carcinoma remains controversial. We report the case of a man with a cutaneous histiocytoid SRC carcinoma of the axilla having histopathological and immunohistochemical features that were quite similar to histiocytoid lobular carcinoma (histiocytoid LC) of the breast, which is a subtype of classic invasive lobular carcinoma of the breast with apocrine differentiation. We consider this case to be a type of CAC equivalent to histiocytoid LC of the breast, based on the features and the occurrence on the axilla. The patient was treated with adjuvant chemotherapy according to the general guidelines for the treatment of breast carcinoma.     

An increased risk of non‐Hodgkin lymphoma and chronic lymphocytic leukemia in US patients with Merkel cell carcinoma versus Australian patients: A clinical clue to a different mechanism of pathogenesis?

The US and Queensland populations both demonstrate an increased risk of secondary malignancies following the diagnosis of Merkel cell carcinoma (MCC). A recent Queensland study failed to demonstrate a significantly increased risk of developing non‐Hodgkin lymphoma (NHL) or chronic lymphocytic leukaemia (CLL) in these patients. In contrast, using the US Surveillance, Epidemiology, and End Results database, we demonstrate there is an increased risk in CLL and NHL following the diagnosis of MCC in the USA. We hypothesise that this difference may be a result of a differing pathogenesis.     

Can simultaneous contact allergies to phenyl glycidyl ether and epoxy resins of the bisphenol A/F‐types be explained by contamination of the epoxy resins?

Background: Simultaneous contact allergies to epoxy resins based on diglycidyl ether of bisphenol A (DGEBA‐R) or epoxy resins of the bisphenol F‐type and the reactive diluent phenyl glycidyl ether (PGE) have been reported. The reason might be cross‐reactivity, exposure to an epoxy resin system with PGE as a component, or contamination by PGE in the epoxy resin. Aims: To study contamination by PGE, 20 commercial epoxy resins were analysed for the presence of PGE. To study contact allergy to PGE and its relation to epoxy resins by inserting PGE in the standard series. Results: Among 2227 patients, 7 reacted to PGE. Of 23 (30%) patients, 7 with contact allergy to DGEBA‐R and 7/19 (37%) with contact allergy to an epoxy resin of the bisphenol F‐type reacted to PGE. All 7 patients with contact allergy to PGE reacted both to the DGEBA‐R and to the epoxy resin of the bisphenol F‐type. PGE was found in 90% of the investigated resins. The amounts of PGE ranged between 0.004% w/w and 0.18% w/w. Conclusion: Most probably, the presence of PGE as a contaminant in epoxy resins is of minor importance for the sensitization, but possibly the contamination of PGE might elicit contact dermatitis in individuals with a high reactivity to PGE.     

Severe flucloxacillin‐induced acute generalized exanthematous pustulosis (AGEP), with toxic epidermal necrolysis (TEN)‐like features: does overlap between AGEP and TEN exist? Clinical report and review of the literature

Acute generalized exanthematous pustulosis (AGEP) and Stevens–Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) are rare but severe cutaneous adverse drug reactions. Especially in TEN, large areas of the skin and mucosae may become detached. Although AGEP and SJS/TEN are distinct entities with a different clinical picture, pathogenesis, prognosis and treatment, they may share some features, raising the hypothesis of overlap between both entities. We present a severe case of AGEP, caused by flucloxacillin, clinically presenting with TEN‐like features and pronounced systemic symptoms with haemodynamic and respiratory instability. Furthermore, we present a review of the literature on cases of AGEP with features resembling SJS/TEN or a supposed overlap with SJS/TEN.     

Does deprivation of area of residence influence the incidence,tumour site or T stage of cutaneous malignant melanoma? A population‐based and clinical database study

This study aimed to document the incidence of malignant melanoma at specific subsites in men and women, stratified by deprivation of area of residence in southeast England, and to explore the association between deprivation and tumour thickness at diagnosis. Data were extracted on 6468 cases from the Thames Cancer Registry for the years 1998 to 2002, and data on, and 508 cases were extracted from the clinical database of the Skin Tumour Unit, St Thomas' Hospital, for the years 1996 to 2004. The postcode of residence was used to assign quintiles of deprivation based on the income domain stated in the Indices of Deprivation 2000. For both males and females, the incidence was higher for those living in the most affluent areas. The trunk was the most common site in males and the lower limbs in females. All sites showed an affluence gradient, although this was least pronounced for head and neck tumours. Distribution of T stage at diagnosis did not differ by deprivation of area of residence.     

Primary cutaneous marginal zone B-cell lymphoma may exhibit both the t(14;18)(q32;q21) IGH/BCL2 and the t(14;18)(q32;q21) IGH/MALT1 translocation: an indicator for clonal transformation towards higher-grade B-cell lymphoma?

Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is a recently proposed entity and constitutes the cutaneous counterpart of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT). The t(14;18)(q32;q21) involving the IGH and the MALT1 gene has previously been described in PCMZL, whereas the t(14;18)(q32;q21) IGH/BCL2 seems to be restricted to follicular lymphoma and diffuse large B-cell lymphoma. We screened 30 PCMZLs of 13 patients by fluorescent in situ hybridization analysis for the presence of the t(14;18)(q32;q21) IGH/BCL2 and the t(14;18)(q32;q21)IGH/MALT1. The t(14;18)(q32;q21) IGH/MALT1 was detected in 10 PCMZLs of eight patients, with four patients showing the t(14;18)(q32;q21) IGH/MALT1 exclusively. The t(14;18)(q32;q21)IGH/BCL2 was detected in 16 PCMZLs of seven patients, with four patients showing the t(14;18)(q32;q21) IGH/BCL2 exclusively. Six lymphomas of four patients showed both translocations in the same lesion. In seven lymphomas, neither of the two translocations occurred. One patient developed multiple lesions without either of the two translocations. Our results underline that both the t(14;18)(q32;q21)IGH/BCL2 and the t(14;18)(q32;q21) IGH/MALT1 may occur in PCMZL, albeit in an irregular distribution. Therefore, the etiopathogenetic relevance of either translocation in PCMZL remains a matter of debate.