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Background
This is rare skin rash that has been confusingly classified as a variant of mycosis fungoides, a skin lymphoma, or a reactive process. Part of the problem has been the lumping of cases of mycosis fungoides and cutaneous T-cell lymphomas, confusing the clinical-pathologic presentation of these cases.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION INCIDENCE Rare
PATHOGENESIS CHARACTERIZATION GENERAL
Microenvironment of Woringer-Kolopp's disease.Geerts ML, Kaiserling E, Kint A.
Dermatologica 1982 Jan;164(1):15-29 Abstract quote A localized form of 'Pagetoid reticulosis' (Woringer-Kolopp's disease) is described morphologically. It can be shown that ultrastructurally the 'Pagetoid cells' are heterogenic and that different cell types can be distinguished: there are lymphoid cells, which often look like Lutzner cells, and nonlymphoid cells which can be characterized as indeterminate cells or Langerhans' cells. Often a close topographical relation between lymphoid and nonlymphoid cells can be demonstrated. This finding is suggestive for a functional interaction between the lymphoid cells, which are most probably T cells, and indeterminate cells and Langerhans' cells, which are known to be important for the thymus-dependent immune reaction.
The presence of the nonlymphoid cell population in the epidermis in Woringer-Kolopp's disease seems to build up the specific microenvironment for lymphoid cell reaction.
REACTIVE
Woringer-Kolopp disease. A lymphomatoid hypersensitivity reaction.Crowson AN, Magro CM.
Central Medical Laboratories, Misericordia General Hospital, Winnipeg, Canada.
Am J Dermatopathol 1994 Oct;16(5):542-8 Abstract quote We report two cases of unilesional pagetoid reticulosis (Woringer-Kolopp disease). Histopathological, immunohistological, ultrastructural, and genotypic studies showed both lesions to be lymphomatoid hypersensitivity reactions, as characterized by polyclonal proliferations of T-lymphocytes; CD8+ T cytotoxic-suppressor (Tc/s) lymphocytes were predominant in one case, whereas CD4+ T helper (Th) lymphocytes were predominant in the other.
The nosology of Woringer-Kolopp disease is discussed.
T-CELL LYMPHOMA
Woringer-Kolopp disease (localized pagetoid reticulosis) or unilesional mycosis fungoides? An analysis of eight cases with benign disease.Burns MK, Chan LS, Cooper KD.
Department of Dermatology, University of Michigan, Ann Arbor.
Arch Dermatol 1995 Mar;131(3):325-9 Abstract quote BACKGROUND: The controversial nosology of Woringer-Kolopp disease (localized pagetoid reticulosis, unilesional mycosis fungoides) is being clarified by the systematic immunophenotypic and immunogenetic examination of infiltrating lesional T lymphocytes. The clinical course and immunohistochemical characteristics of eight cases of Woringer-Kolopp disease are described.
OBSERVATIONS: Lesions measured 0.8 x 0.5 to 16.0 x 15.0 cm. Histologically, all cases resembled mycosis fungoides-type cutaneous T-cell lymphoma and phenotypic analysis supported their designation as an epidermotropic T-cell process. Phenotypic aberrancy was not noted on immunohistochemical analysis of paraffin-embedded tissue. Three of four patients with available fresh-frozen tissue specimens demonstrated reduced or absent expression of CD7 (Leu-9) and/or Leu-8, while loss of the pan-T-cell markers CD2, CD3, and CD5 was not observed. Only in half these patients was a lesional predominance of CD4+ T-cells revealed. Germline DNA was detected in a lesional skin specimen obtained from one patient tested for T-cell receptor gene rearrangements. After treatment, the observation of disease-free periods ranging from 18 months to 17 years (mean, 5.9 years) reinforces the view that Woringer-Kolopp disease is a focal pathologic event with a favorable prognosis. No patient experienced a local recurrence or distant spread of the disease.
CONCLUSION: This and previous studies suggest that Woringer-Kolopp disease is a unique, benign unilesional T-cell lymphoproliferative process with certain histologic and phenotypic similarities to both early epidemotropic mycosis fungoides-type cutaneous T-cell lymphoma and other T-cell lymphoproliferations.
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION GENERAL
- Ketron-Goodman disease, Woringer-Kolopp disease, and pagetoid reticulosis.
Steffen C.
Am J Dermatopathol. 2005 Feb;27(1):68-85. Abstract quote
In this historical review I will synopsize the original articles by Lloyd W. Ketron and M.H. Goodman who described Ketron-Goodman disease, by Frederic Woringer and Pierre Kolopp who described Woringer-Kolopp disease, and by Otto Braun-Falco and colleagues who described pagetoid reticulosis. In their publications, each of these authors reported on one patient.
I will review the clinical picture of the three patients, their histopathology, and the pathogenesis of each disease as suggested by the above authors. Then the views of others that have written on the subject recently will be reviewed particularly as to their conception of the diseases. Publications that describe the histopathology of the patch (early) stage of mycosis fungoides will be redacted to compare it to the histopathology of Ketron-Goodman disease, Woringer-Kolopp disease, and pagetoid reticulosis.
I will discuss whether any or all of them are diseases sui generis, whether they are one, two, or three entities, or whether any or all are but forms of mycosis fungoides.
Pagetoid reticulosis. A further case report with a review of the literature.Braun-Falco O, Schmoeckel C, Burg G, Ryckmanns F.
Acta Derm Venereol Suppl (Stockh) 1979;59(85):11-21 Abstract quote A further case of pagetoid reticulosis (PR) was studied, and the findings were compared with 18 previous case reports in the literature.
There seem to be two variants: The generalized type (Ketron-Goodman) with disseminated lesions and a poor prognosis, and the localized type (Woringer-Kolopp), with a single lesion usually located on the extremities with a good prognosis (if the lesion can be totally cleared by excision or highdose-radiation).
The basic criterion of diagnosis is a very strong cellular infiltrate within the epidermis, but usually weak in the upper dermis. The etiology of PR remains unclear: In some cases a T-cell lymphoma seems a likely interpretation. In others, epidermal infiltrating cells are not typically lymphoid, but remain morphologically unclassifiable.
WORINGER-KOLOPP DISEASE Localized KETRON-GOODMAN Generalized
Disseminated pagetoid reticulosis (Ketron-Goodman disease): Six-year follow-up.Nakada T, Sueki H, Iijima M.
Department of Dermatology, Showa University School of Medicine.
J Am Acad Dermatol 2002 Aug;47(2 Pt 2):S183-6 Abstract quote Pagetoid reticulosis consists of 2 types: Woringer-Kolopp and Ketron-Goodman disease (K-G). Compared with the former, K-G may have disseminated lesions and a guarded prognosis.
We encountered a case of K-G in a 67-year-old man with disseminated plaques on the neck, the trunk, and both extremities. Histologic specimens demonstrated medium- to large-sized, atypical cells infiltrating in the lower epidermis. The phenotype (CD3(+), CD4(-), CD8(-), CD45RO(-), CD45RA(+)) and ultrastructural findings suggest that these cells were immature T cells. Although PUVA was initially effective, new plaques in which atypical cells are still present histologically, have appeared through a 6-year follow-up. Literature review revealed the high rate of recurrence.
These findings suggest that K-G is an epidermotropic or immature T-cell variant of mycosis fungoides. In patients with K-G, therefore, long-term observation is necessary: Disappearance of cutaneous lesions may not mean cure.
Disseminated pagetoid reticulosis (Ketron-Goodman disease): Six-year follow-up.Nakada T, Sueki H, Iijima M.
Department of Dermatology, Showa University School of Medicine.
J Am Acad Dermatol 2002 Aug;47(2 Pt 2):S183-6 Abstract quote Pagetoid reticulosis consists of 2 types: Woringer-Kolopp and Ketron-Goodman disease (K-G). Compared with the former, K-G may have disseminated lesions and a guarded prognosis.
We encountered a case of K-G in a 67-year-old man with disseminated plaques on the neck, the trunk, and both extremities. Histologic specimens demonstrated medium- to large-sized, atypical cells infiltrating in the lower epidermis. The phenotype (CD3(+), CD4(-), CD8(-), CD45RO(-), CD45RA(+)) and ultrastructural findings suggest that these cells were immature T cells. Although PUVA was initially effective, new plaques in which atypical cells are still present histologically, have appeared through a 6-year follow-up. Literature review revealed the high rate of recurrence.
These findings suggest that K-G is an epidermotropic or immature T-cell variant of mycosis fungoides. In patients with K-G, therefore, long-term observation is necessary: Disappearance of cutaneous lesions may not mean cure.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL
Pagetoid reticulosis (Woringer-Kolopp disease). Histopathologic and ultrastructural observations.Medenica M, Lorincz AL.
Arch Dermatol 1978 Feb;114(2):262-8 Abstract quote Histopathologic and ultrastructural observations in a case of Pagetoid reticulosis (Woringer and Kolopp disease) are reported.
The most important clinical feature of this disease is manifestation as a single very slowly enlarging verrucoid, plaque-type skin lesion without internal organ involvement. Histologically, the epidermis is extremely acanthotic and densely infiltrated with numerous atypical appearing cells that are entirely absent from the dermis, which simply contains a banal dense chronic inflammatory infiltrate.
Ultrastructural study showed that there are two major types of unusual cells infiltrating the epidermis. One type is a large lymphoid cell similar to the stimulated lymphocyte. The second type differs from the first in having paler staining of the nucleus and cytoplasm and showing cell membrane damage. These cells probably derive by degeneration from the large lymphoid cells. The presence of transitional forms favors this interpretation. Occasionally, degeneration of these cells proceeds to their complete necrosis.
Pagetoid reticulosis (Woringer-Kolopp disease).Wood WS, Killby VA, Stewart WD.
J Cutan Pathol 1979 Apr;6(2):113-23 Abstract quote Two patients with solitary lesions of pagetoid reticulosis are presented. A third case with disseminated lesions and similar microscopic detail is also reported. Lighg microscopic findings include an abnornal cell infiltrate confined to the epidermis and distributed diffusely in a pagetoid pattern. In ultrathin sections, the abnormal cells are seen to have an abundant cytoplasm with numerous organelles and a convoluted nucleus, often with a prominent nucleolus.
The nature of the abnormal cells is discussed. The infiltrate is differentiated from that of mycosis fungoides.
Pagetoid reticulosis (Woringer-Kolopp disease). An ultrastructural and immunocytological study.Takahashi H, Takahashi K, Tanno K, Iijima S.
Acta Pathol Jpn 1982 May;32(3):513-26 Abstract quote Histopathological, immunocytological and ultrastructural observations are reported in the first case of pagetoid reticulosis (Woringer-Kolopp disease) in Japan. The patient was a 61-year-old woman with multiple skin lesions running a chronic and apparently benign clinical course.
Histology of the skin biopsies revealed typical pagetoid appearance of the epidermis due to intraepidermal infiltration of abnormal cells. Ultrastructural investigation showed that the intraepidermal abnormal cells were classified into mycosis fungoides cells, Sezary cells, lymphoblast-like cells, and large blastoid cells and that the mycosis fungoides cells were a major cell population. Intermediate or transitional cells were found between these cells and large blastoid cells were mostly situated in the basal cell layer. By the rosetting assays of the free cell suspensions prepared from the epidermis of the biopsied skin lesions, 93% of the suspended cells were positive for spontaneous rosette formation with sheep erythrocytes. The immunoperoxidase technique demonstrated no cytoplasmic immunoglobulins in almost all the intraepidermal abnormal cells. These results indicate that the intraepidermal abnormal cells are T-lymphocytes.
Thus, it is concluded that the present case is a cutaneous T-cell lymphoma of low-grade malignancy showing a prominent epidermotropism. This case is the first description of the disease in Japan.
Woringer-Kolopp disease (pagetoid reticulosis). Four cases with histopathologic, ultrastructural, and immunohistologic observations.Deneau DG, Wood GS, Beckstead J, Hoppe RT, Price N.
Arch Dermatol 1984 Aug;120(8):1045-51 Abstract quote Four patients had localized lesions of Woringer-Kolopp disease (pagetoid reticulosis). The clinical appearances and histopathologic features with light microscopy of the four cases were similar. However, electron microscopy in all four cases and enzyme histochemistry studies and immunophenotyping with monoclonal antibodies in three cases disclosed two subgroups of patients. The features of the predominant cells in the intraepidermal infiltrate of three cases were most consistent with those of helper T lymphocytes with cerebriform nuclei, as found in many cases of mycosis fungoides. In one case, however, the epidermis contained a predominant population of small, medium, and large lymphoid cells of cytotoxic/suppressor T-lymphocyte cell phenotype.
It remains to be determined whether Woringer-Kolopp disease is a variant of mycosis fungoides (ie, within the spectrum of the cutaneous T-cell lymphomas), a benign reactive process, or a syndrome with various causes.
VARIANTS
SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHERCHARACTERIZATION SPECIAL STAINS IMMUNOPEROXIDASE CD8 positive
Pagetoid reticulosis (Woringer-Kolopp disease): an immunophenotypic, molecular, and clinicopathologic study.Haghighi B, Smoller BR, LeBoit PE, Warnke RA, Sander CA, Kohler S.
Department of Pathology, Stanford University Medical Center, California 94305-5302, USA.
Mod Pathol 2000 May;13(5):502-10 Abstract quote Pagetoid reticulosis (PR), also known as Woringer-Kolopp disease, is a form of cutaneous T-cell lymphoma that demonstrates striking epidermotropism on histologic examination.
We present the histologic, immunologic, and molecular findings for seven patients who had PR. The patients ranged in age from 33 to 67 years. All patients presented with one or several thick plaques involving the distal extremities except for one patient, who presented with a tongue lesion.
Immunohistochemical staining of the atypical lymphoid cells demonstrated a T-cell phenotype in all cases. In one of four frozen cases, the neoplastic cells were of T-helper cell phenotype (CD4 positive). Four of seven cases demonstrated a T-cytotoxic/suppressor cell phenotype (CD8 positive). The T-cell subset for the remaining two cases could not be determined. CD30 positivity and a high growth fraction as indicated by staining with Ki-67 were seen in three of seven and three of four cases, respectively. Genotypic analysis performed on three of our cases revealed T-cell receptor (gamma and/or beta) rearrangement, indicating a clonal proliferation.
The clinical follow-up ranged from 15 months to 13 years. Four of seven patients are alive and free of disease after treatment with excision or local irradiation. One patient relapsed twice after treatment with radiation and photochemotherapy with 8-methoxypsoralen and UVA and was then lost to follow-up. The lesions of another patient resolved spontaneously but recurred at the same and in an additional site 5 years later. One patient recurred after electron beam therapy. The recurrent lesion improved with radiation therapy and local wound care but never resolved completely. The patient died of unrelated causes.
Our findings suggest that PR is a distinct clinicopathologic entity, separate from unilesional mycosis fungoides, demonstrating a slow disease course. The disease is a clonal cutaneous T-cell lymphoma with relatively consistent clinical and histopathologic findings but a heterogeneous immunophenotypic profile.
CD30
Department of Dermatology, University of Pretoria, Pretoria, South Africa.
Pagetoid reticulosis (PR) is a low-grade primary cutaneous T-cell lymphoma that usually presents as a solitary, slowly enlarging erythematous or hyperkeratotic plaque on the distal areas of the extremities.
Histopathologically, it is characterized by a dense, band-like infiltrate of atypical lymphocytes with prominent epidermotropism within a hyperplastic epidermis, and immunophenotypic studies show in most cases, a CD4-positive T-helper phenotype for the neoplastic lymphocytes.
We describe an African man with a more than 20-year history of an acral lesion of PR, which was histopathologically characterized by lymphocyte immunophenotype consisting of CD8- and CD30-positive cells. We discuss the differential diagnosis with other primary cutaneous lymphoproliferative disorders showing similar immunophenotype. This case shows that CD30-positive PR should be included as a rare variant within the spectrum of CD30-positive primary cutaneous lymphoproliferative disorders.
As in other primary cutaneous CD30-positive lymphoproliferative processes, lesions of CD30-positive PR show an indolent course and a benign biological behavior.CD45
Loss of leucocyte common antigen (CD45) on atypical lymphocytes in the localized but not disseminated type of Pagetoid reticulosis.Sterry W, Hauschild A.
Department of Dermatology, University of Kiel, Germany.
Br J Dermatol 1991 Sep;125(3):238-42 Abstract quote Atypical intraepidermal T cells in Pagetoid reticulosis did not express CD45RO, the low molecular isoform of CD45. These cells did express other CD45 isoforms in the disseminated aggressive variant (Ketron-Goodman type).
The complete CD45 family was lost from the cell surface in the benign localized variant (Woringer-Kolopp type). Because the presence of CD45 is crucial for the function of the lymphocyte-specific tyrosine kinase p56lck, which is associated with lymphocyte growth and transformation, this may be the molecular basis for the non-aggressive nature of this clonal T-cell proliferation.
ELECTRON MICROSCOPY
The ultrastructure of mycosis fungoides, of Sezary's syndrome, and of Woringer-Kolopp's disease (pagetoid reticulosis).Braune-Falco O, Schmoeckel C, Wolff HH.
Bull Cancer 1977;64(2):191-208 Abstract quote Skin lesions of 13 cases of mycosis fungoides, 2 cases of Sezary's syndrome and one case of Woringer-Kolopp's disease were studied.
In mycosis fungoides two main cell types were observed: small atypical lymphoid cells, and large atypical lymphoid cells. The small atypical lymphoid cells were characterized by dense nuclei with varying degrees of nuclear lobulation. Normal-appearing lymphocytes and typical mycosis cells were in this cell group. The large atypical lymphoid cells were characterized by large nuclei with little lobulation and by less chromatin condensation, as well as by a more abundant cytoplasm. Their identification is discussed and indications of their possibly being neoplastic or proliferating lymphoblasts are given.
In Sezary's syndrome Sezary cells with more lobulated nuclei than in mycosis cells and lymphoblast-like cells as in mycosis fungoides could be found. In Woringer-Kolopp's disease cells similar to Sezary cells aggregated mainly in the epidermis. No interdigitating reticulum cells and no other morphological signs of a specific lymphocyte microenvironment were found in any of the cases.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES GAMMA-DELTA T-CELL LYMPHOMA
Primary cutaneous gamma/delta T-cell lymphoma presenting as disseminated pagetoid reticulosis.Berti E, Cerri A, Cavicchini S, Delia D, Soligo D, Alessi E, Caputo R.
Istituto di Clinica Dermatologica Prima e Dermatologia Pediatrica, University of Milan, Italy.
J Invest Dermatol 1991 May;96(5):718-23 Abstract quote The first case of primary gamma/delta cutaneous T-cell lymphoma (CTCL) with a fatal outcome is reported. The patient had the clinical and histopathologic features of disseminated pagetoid reticulosis, a rare form of CTCL characterized by a strong epidermotropic lymphoid infiltrate.
Extensive immuno-cytochemical studies showed that the neoplastic cells were almost exclusively localized in the epidermis, expressed the gamma/delta variant of the T-cell receptor (CD3+, TCR-delta-1+) and were CD5+, CD7+, CD27+, CD29+, CD43+, CD44+, CD45+, CD45RA+, CD54+, CD69+, but beta F1-, Ti gamma a-, BB3-, A13-, CD2-, CD4-, CD8-, CD11a-, CD49d-, CD25-, CD30-, and HLA-DR-. A comparison of our results with those of the literature, which have not included gamma/delta T-cell receptor analysis, suggests that some reported cases of pagetoid reticulosis may have phenotypes similar to our case. Electron microscopy studies demonstrated that the gamma/delta T lymphocytes were villous, containing dense and multivesicular bodies, and formed close contacts with the surrounding keratinocytes, suggesting that these cells should have a role in the skin-associated lymphoid tissue.
The proliferating cells in our case might represent the neoplastic counterpart of the recently reported CD2- subset of normal human peripheral blood gamma/delta T lymphocytes.
MYCOSIS FUNGOIDES
Woringer-Kolopp disease. An epidermotropic variant of mycosis fungoides.Degreef H, Holvoet C, Van Vloten WA, De Wolf-Peeters C, Desmet V.
Cancer 1976 Nov;(5):2154-65 Abstract quote Woringer-Kolopp disease is characterized by the presence of a monomorphic, tumorous infiltrate in the epidermis. A light- and electron microscopic study, and cytophotometry, of one case were performed and the results are discussed. No explanation has been found for the epidermotropic character of this condition, although an intraepidermal origin of the tumor infiltrate seems improbable. The cytomorphologic characteristics of the tumor cells shows a striking analogy to the atypical cells in the Sezary syndrome and mycosis fungoides.
These findings support the hypothesis that Woringer-Kolopp disease represents a clinical variant of mycosis fungoides and the Sezary syndrome.
Localized mycosis fungoides not manifesting as Woringer-Kolopp disease.Ringel E, Medenica M, Lorincz A.
Arch Dermatol 1983 Sep;119(9):756-60 Abstract quote A 68-year-old man had a solitary, eczematiform lesion of two years' duration on the digital dorsal surface of his right foot. Despite its innocuous appearance, the lesion showed histologic characteristics of advanced, plaque-type mycosis fungoides.
The patient's clinically benign yet histologically malignant disease manifestation is discussed. The distinction between localized mycosis fungoides and Woringer-Kolopp disease is also reviewed.
Pagetoid reticulosis, epidermotropic mycosis fungoides and mycosis fungoides: a disease spectrum.Tan RS, MacLeod TI, Dean SG.
Br J Dermatol 1987 Jan;116(1):67-77 Abstract quote Using a standard technique involving monoclonal antibodies against T-cell subsets, we have shown that almost all the infiltrating T-cells in the epidermis of a patient with Pagetoid reticulosis (PR), one with epidermotropic mycosis fungoides (EMF) and one with poikiloderma atrophicans vasculare (PAV), were OKT8 positive (presumed cytotoxic/suppressor) T-cells. The infiltrating T-cells in the epidermis of a patient with limited plaque stage mycosis fungoides (MF), however, were almost exclusively Leu 3a-positive (presumed helper/inducer) T-cells as is usually found in this condition. The keratinocytes in the patients with PR, EMF and PAV were HLA-DR-positive whilst those in the patient with MF were HLA-DR-negative.
We consider these four diseases to be part of the spectrum of mycosis fungoides, the first three conditions representing the early or benign end of the spectrum.
Solitary skin lesions with histopathologic features of early mycosis fungoides.Cerroni L, Fink-Puches R, El-Shabrawi-Caelen L, Soyer HP, LeBoit PE, Kerl H.
Department of Dermatology, University of Graz, Austria.
Am J Dermatopathol 1999 Dec;21(6):518-24 Abstract quote Mycosis fungoides (MF) is a cutaneous T-cell lymphoma that usually begins with cutaneous patches that evolve into plaques and tumors. A few recent reports describe a solitary variant of MF distinct from localized pagetoid reticulosis, a disease in which solitary verrucous lesions occur on acral skin. Solitary skin lesions with some of the histopathologic features of MF rarely occur during treatment with several drugs, especially antidepressants or antihistamines.
We analyzed the clinicopathologic features of 20 patients with solitary skin lesions showing histopathologic features of patch- or early plaque-stage MF. Eight men and 12 women (mean age 50.6, range 23-82, median 49) had solitary, small erythematous patches or plaques located on the trunk (16 cases, 6 of them on the breast), upper extremities (3 cases), and inguinal region (1 case). Ten patients were treated with one or more drugs; only two of them received antidepressants or antihistamines.
Histopathologic examination revealed in all cases a band-like infiltrate in the upper dermis, frequently with epidermotropism of solitary lymphocytes. Atypical lymphocytes were present in a minority of cases. Immunohistology showed a predominance of CD3+ T lymphocytes, in most cases admixed with clusters of CD20+ B-cells. Only a small proportion of the infiltrate was CD8+. Molecular analysis of the rearrangement of the T-cell receptor genes was performed in 16 cases using the polymerase chain reaction (PCR) technique and revealed a monoclonal band in 8 of them.
After surgical excision, 2/14 patients had a recurrence near the surgical scar. In 18 patients with complete follow-up data, no evidence of "classic" MF could be observed after a mean follow-up of 31.9 months. Solitary skin lesions with the histopathologic features of MF can be considered as a distinct clinicopathologic entity, probably representing a solitary variant of mycosis fungoides.
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSTIC FACTORS
Localized epidermotropic reticulosis (Woringer-Kolopp disease).Mandojana RM, Helwig EB.
J Am Acad Dermatol 1983 Jun;8(6):813-29 Abstract quote In order to clarify the biologic behavior and establish clinical and histologic criteria for the diagnosis of a cutaneous disease originally described by Woringer and Kolopp, we examined fifteen new cases and reviewed thirteen reports.
The disease presents as a single, chronic, asymptomatic, circinated, scaly plaque. It preferably involves distal extremities and affects middle-aged individuals, as well as a considerable number of youths. The male to female ratio is 2:1. The treatment of choice is surgical excision or radiotherapy. One lesion resolved with an intralesionally administered steroid. Follow-up data of the twenty-eight cases suggest that this is a benign lesion. Paradoxically, its atypical microscopic features contrast with its benign clinical appearance and biologic behavior. The lower epidermis appears focally infiltrated by large atypical mononuclear cells that spare the dermis. Ultrastructural observations suggest that these are stimulated T lymphocytes, which sometimes are mixed with histiocytes.
We propose the term "localized epidermotropic reticulosis" for this distinct clinicopathologic entity, which is different from mycosis fungoides.
Disseminated pagetoid reticulosis: need for long-term follow-up.Yagi H, Hagiwara T, Shirahama S, Tokura Y, Takigawa M.
Department of Dermatology, Hamamatsu University School of Medicine, Japan.
J Am Acad Dermatol 1994 Feb;30(2 Pt 2):345-9 Abstract quote A 54-year-old woman had an erythematous plaque on the left index finger for 2 years. Histologically there was a dense infiltrate of atypical mononuclear cells in the epidermis that produced a pagetoid appearance. A diagnosis of localized pagetoid reticulosis was made. Three years after total removal of the finger lesion, erythematous plaques appeared on her left thigh, followed by the development of similar plaques on the right thigh and lower abdomen.
Histologically these skin lesions had the same pagetoid appearance as the plaque on the finger. This suggested development of the disseminated type of pagetoid reticulosis, long after removal of the initial skin lesion.
Macpherson and Pincus. Clinical Diagnosis and Management by Laboratory Methods. Twentyfirst Edition. WB Saunders. 2006.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.
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