30 años del Grupo Español de Dermatopatología / The Spanish Dermatopathology Group-30 Years On

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Pero ¿hubo alguna vez una clasificación de Clark de los melanomas? / But was there ever a Clark classification of melanomas?

Desde hace 40 años, la inmensa mayoría de los dermatólogos y los patólogos de todo el mundo ha aceptado y ha empleado la clasificación de Clark de los melanomas cutáneos. Sin embargo, tras una cuidadosa relectura de los artículos fundamentales de Clark y sus colaboradores, hemos podido comprobar que tal clasificación ha sido en realidad muy efímera. Tras distinguir en 1968 entre melanoma nodular, melanoma de extensión superficial (MES) y melanoma del lentigo maligno (MLM), la inclusión en 1979 del melanoma lentiginoso acro (MLA) como un nuevo subtipo de melanoma fue la primera avería seria de la clasificación, ya que un melanoma lentiginoso y de aparición tardía (como el MLM) se localizaba, a diferencia de éste, en las zonas menos fotoexpuestas de la piel. Posteriormente, los mismos autores comprobaron que, contrariamente a su idea inicial, el pronóstico del MLM era el mismo que el de los demás subtipos a igualdad de espesor, según Breslow. Finalmente, diversas observaciones de los mismos autores fueron poniendo de manifiesto su creciente dificultad para distinguir al microscopio entre MLM, MES y MLA, salvo que tuviesen en cuenta la localización. Es decir, que hoy por hoy las posibles diferencias morfológicas entre uno y otro caso de melanoma cutáneo no conllevan demostradas diferencias pronósticas, y las diferencias morfológicas que puedan encontrarse se deben más a la diferente localización que a la propia neoplasia (AU)

For the past 40 years, the Clark classification of cutaneous melanoma has been accepted and used by the vast majority of dermatologists and pathologists throughout the world. However, after careful rereading of the most relevant articles by Clark and his collaborators, we can affirm that the classification was only ever of passing validity. After distinguishing between nodular melanoma, superficial spreading melanoma (SSM), and lentigo maligna melanoma (LMM) in 1968, the inclusion of acral-lentiginous melanoma (ALM) in 1979 as a new subtype was the first serious setback for the classification; in contrast to ALM, late-onset lentiginous melanomas, such as LMM, were situated on areas of skin with less exposure to sunlight. Later, the same authors found that, contrary to their initial belief, the prognosis of LMM was the same as that of other subtypes with the same Breslow thickness. Finally, a number of observations by the same authors made ever clearer the increasing difficulty for distinguishing microscopically between LMM, SSM, and ALM, except by taking their localization into consideration. This means that, today, the possible morphological differences between one case of cutaneous melanoma and another are of no proven prognostic implication. In addition, the morphological differences that can be found are much more closely related to the different localization that to the tumor itself (AU)

[But...was there ever a Clark classification of melanomas?]. / Pero...hubo alguna vez una clasificación de Clark de los melanomas?

For the past 40 years, the Clark classification of cutaneous melanoma has been accepted and used by the vast majority of dermatologists and pathologists throughout the world. However,after careful rereading of the most relevant articles by Clark and his collaborators, we can affirm that the classification was only ever of passing validity. Today, the possible morphological differences between one case of cutaneous melanoma and another are of no proven prognostic implication. In addition, the morphological differences that can be found are much more closely related to the different localization that to the tumor itself.

¿Tienen ustedes una definición de nevo melanocítico típico? / Do You Have a Definition for Typical Melanocytic Nevus?

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Análisis de la producción científica nacional e internacional de los dermatólogos españoles (1988-2000) / Analysis of the international and national scientific production of Spanish dermatologist (1998-2000)

Objetivo. Comparar la producción científica de los dermatólogos españoles en la revista Actas Dermo-Sifiliográficas (AD) con la publicada en revistas internacionales (RI) incluidas en Medline entre 1988 y 2000. Los principales parámetros bibliométricos analizados han sido: tipo documental, áreas temáticas fundamentales, principales centros productores y distribución geográfica de los artículos. Material y métodos. Se revisaron manualmente todos los artículos científicos publicados en los números ordinarios de la revista AD. Los documentos de autores españoles publicados en RI fueron recuperados a partir de la base de datos Medline. Resultados.En AD se publicaron 1.864 artículos y en RI recuperamos 1.645 documentos. La extensión media de los documentos publicados eN AD fue superior (4,46 ± 2,57) a la de los publicados en RI (3,29 ± 2,33 páginas) (p < 0,001). En los documentos de AD había un predominio de los temas de oncología (13,3% frente a 9,0%) (< 0,001), pediatría (12,7% frente al 8,7%) (p < 0,001) y cirugía (2,4 % frente a 0,9%) (p = 0,001) respecto a los de las RI. En las RI se observó un predominio de los temas de contacto (14,6 % frente a 4,5%) (p < 0,001), fisiopatología (4 % frente a 2,3%) (p = 0,001), reacciones adversas (6,6 % frente a 4,3 %) (p = 0,001) y dermatopatología (18,7% frente a 16,1%) (p = 0,01). Las Comunidades Autónomas (CCAA) de Andalucía (16,4 % frente a 8,6%) (p < 0,001), Aragón (3,9 % frente a 0,7 %) (p < 0,001), Asturias (2,1 % frente a 0,2 %) (p < 0,001), Castilla y León (8,5 % frente a 3,6 %) (p < 0,001) y Madrid (37,1% frente a 30,6%) (p < 0,001) publicaron más en AD. En cambio, las CCAA de Cataluña (26,3% frente a 9,6 %) (p < 0,001), Navarra (5,6% frente a 1,8%) (p < 0,001) y País Vasco (4,2 % frente a 2,0 %) (p < 0,001) lo hicieron en RI. Conclusión. Se han observado diferencias temáticas y regionales de los documentos publicados en dermatología por investigadores españoles

Objective. To compare the scientific production in articles published in Actas Dermo-Sifiliográficas (AD) with papers published by Spanish dermatologists in international journal (IJ) included in Medline data base between 1988 and 2000. The main features studied were: type and extension of document, subject, authors’ place of work, speciality and geographical distribution. Material and methods. Data were obtained by consulting the articles published in the journal AD. Also Spanish papers in dermatology were retrieved from Medline data base. Results. A total of 1,864 articles were published in AD and 1,645 papers were retrieved from IJ. The extension of documents in AD was higher (4.46 ± 2.57) than in IJ (3.29 ± 2.33). In the journal AD there is a predominance of topics related to oncology (13.3 % vs 9.0 %) (< 0.001), pediatrics (12.7% vs 8.7%) (p < 0.001) and surgery (2.4 % vs 0.9%) (p = 0.001) compared to the papers published in IJ. In IJ there was a predominance of topics related to contact dermatitis (14.6% vs 4.5%) (p < 0.001), physiopathology (4% vs 2.3%) (p = 0.001), drug-induced reactions (6.6 % vs 4.3%) (p = 0.001) and dermatopathology (18.7 % vs 16.1 %) (p = 0.01). Andalucía (16.4% vs 8.6 %) (p < 0.001), Aragón (3.9 % vs 0.7 %) (p < 0.001), Asturias (2.1 % vs 0.2 %) (p < 0.001), Castilla- León (8.5 % vs 3.6 %) (p < 0.001), and Madrid (37.1% vs 30.6%) (p < 0.001) published more in AD than in IJ. However, Cataluña (26.3% vs 9.6 %) (p < 0.001), Navarra (5.6 % vs 1.8%) (p < 0.001) and País Vasco (4.2% vs 2.0%) (p < 0.001) published more in IJ. Conclusion.We have observed differences in the topics and the geographical distribution of papers published by Spanish researchers in dermatology

Basal cell carcinoma on the vermilion border of the lip: a study of six cases.

BACKGROUND: The vermilion border of the lower lip is a frequent location of squamous cell carcinoma (SCC), but it is very rarely mentioned within the published series of basal cell carcinomas (BCCs). OBJECTIVE: We present 6 cases of BCC involving either mainly or exclusively the vermilion border of a lip. METHODS: We reviewed from our files all the cases of BCC diagnosed in a period of 11 years. RESULTS: A number of 3,477 BCCs were histologically diagnosed in that period; 2,872 (82.6%) of them were located on the head, and 66 (2.3%) of the latter (1.9% of all BCCs) were on the lips. In 6 cases, the neoplasm involved either mainly or exclusively the vermilion border of either the lower (5 cases) or the upper (1 case) lip. CONCLUSION: Not every carcinoma of the vermilion border of the lip is a SCC.

Panniculitis. Part II. Mostly lobular panniculitis.

UNLABELLED: The second part of our review of panniculitis summarizes the clinicopathologic features of the mostly lobular panniculitides. Erythema induratum of Bazin (nodular vasculitis) represents the most common variant of lobular panniculitis with vasculitis, although controversy persists about the nature of the involved vessels. Mostly lobular panniculitides without vasculitis comprise a series of disparate disorders. These include sclerosing panniculitis that results from chronic venous insufficiency of the lower extremities; panniculitis with calcification of the vessel walls such as calciphylaxis and oxalosis; and inflammatory diseases with crystals within the adipocytes such as sclerema neonatorum, subcutaneous fat necrosis of the newborn, and poststeroid panniculitis. Connective tissue diseases, such as systemic lupus erythematosus and dermatomyositis, pancreatic diseases, and alpha(1)-antitrypsin deficiency may also show a mostly lobular panniculitis with characteristic histopathologic features. Lobular panniculitis may also be an expression of infections, trauma, or factitial causes involving the subcutaneous fat. Lipoatrophy refers to a loss of subcutaneous fat due to a previous inflammatory process involving the subcutis, and it may be the late-stage lesion of several types of panniculitis. In contrast, lipodystrophy means an absence of subcutaneous fat with no evidence of inflammation and often the process is associated with endocrinologic, metabolic, or autoimmune diseases. Finally, cytophagic histiocytic panniculitis is the term that has been used to describe two different processes: one is inflammatory, a lobular panniculitis, and the other one is neoplastic, a subcutaneous T-cell lymphoma. The only common feature of these two different processes is the presence of cytophagocytosis in the lesions. (J Am Acad Dermatol 2001;45:325-61.) LEARNING OBJECTIVE: At the completion of this learning activity, participants should be familiar with the pathogenesis, clinical manifestations, histopathologic findings, and treatment options for the most frequent variants of the lobular panniculitides.

Primary cutaneous large B-cell lymphoma: the relation between morphology, clinical presentation, immunohistochemical markers, and survival.

The histogenesis, morphology, immunophenotype, and clinical behavior of cutaneous large B-cell lymphomas (CLBCL) are largely a matter of controversy. We performed an investigation to determine whether CLBCL have features that differentiate them from other large B-cell lymphomas and whether CLBCL is itself a heterogeneous group. To this end, we reviewed the main characteristics of a series of 32 cases of LBCL found in the skin. We reviewed the clinical findings and paraffin sections of the tumors from these 32 patients. The immunohistochemical study performed included p53, MIB1, Bcl2, Bcl6, and CD10 markers. We carried out statistical analysis of these data (univariate and multivariate), seeking an association between the features of the tumors and clinical outcome, as defined by failure-free survival time. Only one patient died as a consequence of the lymphoma. Nevertheless, the accumulated probability of survival without failure at 48 months was 0.46. The number, type, and localization of the lesions were not associated with variations in either survival or failure-free survival. The expression of p53 was negative in this group of CLBCL, whereas Bcl-2 expression or localization in the lower leg did not relate to any other significant feature. Histologic examination of the cases disclosed three different groups: Grade III follicular lymphomas (FLs), monomorphous large B-cell lymphomas (LBCL type I), and LBCL with an admixed component of small B-lymphocytes (LBCL type II). Grade III FL (11 cases) tended to be found in the head and neck and showed CD10 expression in a majority of cases. A higher probability of lymph node relapses was associated with cases located in the head and neck and with CD10+ tumors. Cutaneous large B-cell lymphomas are indolent tumors, but follow an insidious course. Our data support the interpretation that CLBCL is a heterogeneous condition; comprises some LBCL derived from CD10+ germinal center cells which manifests more frequently as tumors in the head and neck region, with an increased probability of relapse in lymph nodes [1] and has some distinctive morphologic features. The existence of a component of small B-cells within the other CLBCL could lend support to the theory that some of these tumors, more than arise de novo, may have originated in preexistent small B-cell lymphomas, but no firm evidence of this is provided in this study.

Lupus erythematosus with an erythema multiforme-like eruption.

We describe a patient diagnosed with lupus erythematosus (LE) who developed an acute generalized eruption characterized by erythema multiforme (EM)-like lesions. Biopsy specimen showed foci of vacuolar alteration at the dermo-epidermal junction and frequent necrotic keratinocytes. Laboratory tests disclosed a speckled-homogenous antinuclear antibody titer of 1:640, leucopenia and hypocomplementemia. Anti-Ro/anti-La antibodies and rheumatoid factor were negative. Treatment with high-dose oral prednisone and azathioprine led to complete remission of the cutaneous lesions, although eruption recurred two years later. We believe that this patient presented a subacute cutaneous lupus eythematous with a distinctive erythema multiforme-like eruption. This case could be included in the so-called Rowell's syndrome, although it does not fit all the immunological characteristics reported in the original description, as in many of the previously reported cases. At the present time there seems to be enough evidence to classify Rowell's syndrome within the subacute cutaneous lupus erythematosus subset. Finally, a coexistence of LE and EM can not be completely discarded in our patient, although no causative factor was found.

Solitary keratoacanthoma: a self-healing proliferation that frequently becomes malignant.

To discuss the relation between solitary keratoacanthoma (KA) and crateriform squamous cell carcinoma (cSCC), the clinical and histologic features of cutaneous crateriform squamous cell proliferations were studied. Two hundred twenty cases of wholly excised crateriform squamous cell proliferations were studied both clinically (age, sex, location, and duration) and histologically (hematoxylin-eosin-stained sections). For comparison, we studied 100 consecutive cases of wholly excised noncrateriform squamous cell carcinoma (ncSCC). One hundred forty-four of the 220 cases of crateriform squamous cell proliferations were histologically classified as KA. In 47 other cases, a relatively large area of the KA showed frank histologic and cytologic malignant transformation (mKA); this event could happen during every stage of the KA. Twenty-nine lesions were cSCCs without remnants of KA. The patients in the KA group were significantly younger (p = 0.000) than those in the other three groups. The ages of the patients in these three groups were not significantly different (p = 1.0). More KAs (16%) were located in areas that are not usually exposed to the sun than was the case with the other groups of neoplasms considered (2%, 3%, and 3%, respectively), and this difference was statistically significant (p = 0.001). Regarding the duration of the lesion, only the differences between KA and cSCC, KA and ncSCC, and mKA and ncSCC were statistically significant. Not every cutaneous crateriform squamous cell proliferation is a KA; in KA, the crater must be multilocular, the "lips" must be perforated, and the cornified contents do not usually project out of the "mouth." At least a quarter of KAs undergo malignant transformation, which occurs more frequently in older patients and photoexposed areas. This transformation is a focal event and may happen at any stage of KA development. Consequently, a suspected KA must be wholly excised and studied in serial paraffin blocks so as to disclose any focus of malignant transformation.

Trichoblastoma is the most common neoplasm developed in nevus sebaceus of Jadassohn: a clinicopathologic study of a series of 155 cases.

Nevus sebaceus of Jadassohn is a hamartoma that combines epidermal, follicular, sebaceous, and apocrine gland abnormalities. Classically, several types of cutaneous neoplasms have been associated with this hamartoma, with basal cell carcinoma being the most frequently described malignancy. We studied a series of 155 examples of nevus sebaceus of Jadassohn with clinicopathologic correlation. Several histopathologic changes related to the age of the patients were found. In our series, we could not identify any cases of authentic basal cell carcinoma. In contrast, several examples of primitive follicular induction and of trichoblastomas were seen. Other cutaneous hamartomas, hyperplasias, and neoplasms found in our series of nevus sebaceus of Jadassohn included syringocystadenoma papilliferum, sebomatricoma, apocrine gland cyst, poroma, different histopathologic variants of warts (classic warts, tricholemmoma, and desmoplastic tricholemmoma), primitive follicular induction, and ductal induction. In our series, no examples of malignant neoplasms were identified. On the basis of these findings, the classically recommended treatment for this hamartoma, which consists of early excision to prevent the development of malignancy, seems to be inappropriate.

Lichenoid, erosive and ulcerated dermatofibromas. Three additional clinico-pathologic variants.

On the occasion of a case of dermatofibroma with histological lichenoid features, we reviewed from our files all the cases in which the epidermis, usually hyperplastic in dermatofibroma, was, in some way, partially or completely destroyed. Among a total of 484 dermatofibromas, we found three lichenoid, six erosive and two ulcerated cases. In the three lichenoid cases, the columnar epidermal basal cells were lacking (squamotization of the basal layer) and in two of them there was a cleft between the epidermis and the dermatofibroma. Three of the six eroded cases were large pedunculated dermatofibromas with inflammatory phenomena of variable intensity. One case was in the center of a plaque of lichen simplex chronicus with some eroded area. In the other two cases, as well as in the two ulcerated lesions, neither inflammation nor epidermal changes usually attributed to rubbing or scratching were seen. Only in three of the eleven cases dermatofibroma was proposed (with question mark) as a clinical diagnosis. Both follow-up and histopathology supported the benign nature of these cases. We may conclude that: i) Lichenoid, erosive and ulcerated changes in dermatofibroma are infrequent phenomena which may make a clinical diagnosis difficult; and ii) in the presence of an otherwise histopathologically typical dermatofibroma, erosion and ulceration should not be considered as suspicious of malignancy.

Lichen nitidus of the palms: a case with peculiar histopathologic features.

Palmar involvement in lichen nitidus is infrequent. In such cases, the histopathologic findings of palmar lesions are usually identical to those of extrapalmar ones. We report on the case of a patient with multiple tiny papules located on the palms and elbows. A biopsy specimen from the elbow showed the typical features of lichen nitidus, but a biopsy from the palm disclosed an inflammatory infiltrate mostly disposed around the bases of rete ridges and composed of lymphocytes and histiocytes with some giant cells both in the dermis and in the epidermis. This location of the infiltrate is similar to that found in hypertrophic lichen planus, a combination of lichen planus and lichen simplex chronicus. We conclude that this histopathologic feature in palmar lichen nitidus could be the result of the superimposition of lichen nitidus on normal palmar skin, resulting in a picture resembling hypertrophic lichen planus.