Could Jessner's lymphocytic infiltrate of the skin be a dermal variant of lupus erythematosus? An analysis of 210 cases.

BACKGROUND AND OBJECTIVE: The objective of this study was to evaluate the relationship between Jessner's lymphocytic infiltration of the skin (JLI) and lupus erythematosus (LE), which has been the subject of debate since its initial description in 1953. MATERIAL AND METHODS: This is a retrospective study including all patients with a histopathologically ascertained diagnosis of JLI performed at the Laboratoire d'Histopathologie Cutanée of the Strasbourg University Hospital between 1993 and 2003. Information about patient characteristics and follow-up data were retrieved between 2004 and 2005. Special attention was paid to features indicative of LE. RESULTS: 210 consecutive patients (102 women and 108 men) with a mean age 42 years were diagnosed with JLI in the reference period. 175 patients (83%) had multiple lesions and 32 patients (15%) had only a single lesion at the time of diagnosis (data not available in 3 patients). The head, neck and upper part of the thorax were involved in 171 patients (81%). An annular or arciform configuration and/or arrangement were present in 111 patients (53%). Lesions consisted of red (100%) papules or plaques (98%). Mean follow-up was 4 years. Sixteen patients (7.6%) had proven LE. Only 2 patients (1%) developed >4 ACR criteria of systemic LE. Furthermore, 1 patient had antiphospholipid antibody syndrome and 2 patients had rheumatoid arthritis. CONCLUSIONS: This high frequency of patients with typical features of LE strongly argues that JLI could be a dermal variant of LE and not an autonomous entity. It might be the cutaneous marker of a subset of LE patients with excellent prognosis.

Granuloma faciale: a clinicopathologic study of 66 patients.

BACKGROUND: Only case reports or studies of small series of patients have focused on granuloma faciale (GF). OBJECTIVE: We sought to describe the clinicopathologic characteristics of GF in a large series of patients. METHODS: We conducted a retrospective analysis of 66 patients and 73 skin specimens. RESULTS: GF mostly presented as reddish plaques or nodules in middle-aged adults. One third of patients had multiple sites involved and 5 patients had extrafacial lesions. A clinical diagnosis of GF was made in only 10 cases; sarcoidosis, lymphoma, lupus, and basal cell carcinoma were the main differential diagnoses. The most frequent histopathologic features were the presence of a grenz zone, neutrophils, and telangiectases. Vascular changes were frequent, although necrotizing vasculitis appeared to be rare. There was often an association of acute and chronic inflammatory patterns, suggesting that GF follows a chronic history with recurrent acute phases, rather than distinct successive acute and chronic stages. LIMITATIONS: In this retrospective study, a reliable analysis of the outcome of patients could not be performed. Results of direct immunofluorescence tests and laboratory investigations were lacking in many cases. CONCLUSION: GF is often clinically misdiagnosed; its morphologic spectrum is broader than usually described and includes a lack of eosinophils, the presence of vascular changes with rare vessel wall necrosis, and associated acute and chronic inflammatory patterns.

On the regulation of hair keratin expression: lessons from studies in pilomatricomas.

Human hair follicles exhibit a complex pattern of sequential hair keratin expression in the hair matrix, cuticle, and cortex. In pilomatricomas, that is, benign skin tumors thought to arise from germinative matrix cells of the hair follicle and retaining morphological signs of cortical differentiation, this differential hair keratin pattern has been shown to be faithfully preserved in the lower and upper transitional cell compartments of the tumors. Here we show that also the co-expression of hair keratin hHa5 with its regulatory nuclear homeoprotein HOXC13 in matrix cells of the hair follicle is maintained in lower transitional cells of pilomatricomas. In contrast, the nuclear co-expression of LEF1 and beta-catenin, which in the hair follicle has been postulated to initiate cortex cell differentiation through the induction of hair keratin hHa1 expression (Merill et al, Genes Dev 15:1688-1705, 2001), is not preserved in upper transitional cells of pilomatricomas. Although these cells correctly express hHa1, they are completely devoid of LEF1 and nuclear LEF1/beta-catenin co-expression is shifted to a subpopulation of hair keratin-free basaloid cells of the tumors. These data imply that unlike the normal hair follicle, cortical differentiation in pilomatricomas is not under the control of the canonical Wnt signaling pathway.

[Epidemiological features of tumors of the skin and mucosal membranes in the department of dermatology at the Yalgado Ouedraogo National Hospital, Ouagadougou, Burkina Faso]. / Tumeur cutanéo-muqueuses: aspects épidémiologiques dans le service de dermatologie du centre hospitalier national Yalgado Ouedraogo de Ouagadougou, Burkina Faso.

We conducted a retrospective study of the files of all patients seen from 1 January 1992 through 31 December 1996 with tumors of the skin and mucosal membranes at the Yalgado Ouédraogo National Hospital in order to determine the epidemiologic features of this disease. The records revealed 988 patients presented 1024 tumors, which could be classified into 33 categories. Most of the patients (60.6%) were in the age bracket of 20 to 39 years. Nearly all cases (988 or 96.5%) were benign skin tumors, mainly of infectious origin, especially viral (51.7%). We observed a substantial number of sexually transmissible infections, such as condylomata. We also found 36 cases (3.5%) of malignant tumors, including 29 cases of Kaposi sarcoma, five skin carcinoma (13.8%), three spinocellular and two basocellular; we also noted two borderline malignant tumors: a dermatofibrosar-coma protuberans and a nodular hidradenoma. The elevated prevalence of condyloma (151 cases) may explain the predominance of the 20-39 year age group, which is the most sexually active. Our series also confirmed the relative rarity (3.5%) of cutaneous cancers among African blacks. The predominance of Kaposi sarcoma may be explained by the high prevalence of HIV infection in our country.

Ocular manifestations in the inherited DNA repair disorders.

Deoxyribonucleic acid (DNA) repair is a fundamental process designed to keep the integrity of genomic DNA that is continuously challenged by intrinsic or environmental induced alterations. Numerous genes involved in DNA repair have been cloned and are involved in different DNA repair pathways: base excision repair, nucleotide excision repair, mismatch repair, DNA recombination. Inherited conditions due to mutations in DNA repair genes include mainly: xeroderma pigmentosum, Cockayne syndrome, Trichothiodystrophy, Bloom syndrome, Rothmund-Thomson syndrome, and Werner syndrome. Minor to major ocular manifestations occur in these syndromes. For example, eyelid skin cancers in xeroderma pigmentosum and retinal dystrophy in Cockayne syndrome are major features of these syndromes. This review focuses on the DNA repair pathways, the general and ocular features of the related syndromes, the laboratory tests useful for diagnosis, and the general processes implied with DNA repair (ultraviolet sensitivity, carcinogenesis, apoptosis, oxydative stress, and premature aging).

The AESOP (adenopathy and extensive skin patch overlying a plasmacytoma) syndrome: report of 4 cases of a new syndrome revealing POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome at a curable stage.

We describe an easily recognizable and previously not individualized clinical syndrome that can reveal solitary plasmacytoma of bone. We report 4 patients with a slowly extending violaceous skin patch overlying a solitary plasmacytoma of bone, associated with enlarged regional lymph nodes. Biopsies of the cutaneous lesion and the lymph nodes were not specific, although increased dermal mucin deposition and vascular proliferation were present in all skin specimens. Three patients had associated polyneuropathy. One patient had POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, and Skin changes) syndrome at the time the plasmacytoma was diagnosed. Another patient developed POEMS syndrome, from which he died, 4 years after excision of the plasmacytoma. The 3 other patients were treated either with irradiation or with a combination of irradiation and surgery, and recovered completely, including from the associated neuropathy and/or POEMS syndrome. We suggest calling this unique and distinctive clinical presentation the for

Inter-observer variation in the histopathological diagnosis of clinically suspicious pigmented skin lesions.

When a biopsy is taken of a suspicious pigmented skin lesion, histological examination is expected to establish the definitive diagnosis. This study evaluated the inter-observer variation of 20 pathologists in the histological diagnosis of a randomly selected set of suspicious pigmented skin lesions (PSLs), by comparing their diagnoses to a reference diagnosis. Overall sensitivity for melanoma was 87%, ranging from 55% to 100% between the observers. Sensitivity was significantly lower for thin (Breslow thickness <1 mm) than for thick melanomas (83% versus 97%, p=0.005). Overall melanoma specificity was 94%, ranging from 83% to 100% between observers. Dysplastic naevus was the most important source of false-positive diagnoses, mainly in situ melanomas. Positive and negative predictive values in the given test set were 75% and 97%, respectively. In the case of melanoma, there was quite some variation in measured Breslow thickness. This would have led to a different therapeutic approach in 12% of the readings. Some of the variation seemed to be due to a different interpretation of the presence of a co-existent naevus. In 9% (3/35) of the readings, participants did not agree on the presence of ulceration. These results reflect a tendency to overdiagnose mainly thin melanomas in general histopathological practice. They also demonstrate variation in the assessment of major prognostic factors of melanoma.

Stromelysin 3 expression: a useful marker for the differential diagnosis dermatofibroma versus dermatofibrosarcoma protuberans.

BACKGROUND: Stromelysin 3 (ST3) is a member of the metalloproteinase family, which is expressed in tissue remodeling processes such as scarring, embryogenesis, or tumoral invasion. Although the prognosis of breast cancers and extracutaneous squamous cell carcinomas is correlated with the level of expression of ST3, this staining has not yet found a routine application in dermatopathology. OBJECTIVE: Our purpose was to study by immunohistochemistry the expression of ST3 in dermatofibromas and dermatofibrosarcoma protuberans (DFP). METHODS: We selected 40 cases of dermatofibromas, 40 histologically typical DFPs, and 10 giant dermatofibromas. Immunohistochemistry was carried out by means of the LSAB method, with monoclonal anti-ST3 antibody (provided by MC Rio, IGBMC Strasbourg). A semiquantitative scale (0-3) was used to evaluate the level of ST3 expression. RESULTS: Positively stained cells were observed in all cases of dermatofibromas (100%), including the 10 giant cases, but never in DFP (0%). The staining was intense (class 2 or 3) in 39 of the 50 dermatofibromas. The CD34 staining used as a control proved to be less efficient; 6 DFP were CD34 negative, whereas some of the dermatofibromas showed a marginal CD34 positivity. CONCLUSION: Our results are consistent with those obtained by in situ hybridization in previous studies of smaller series of fibrous tumors. The study of ST3 expression in fibrous tumors of the skin shows that this immunostaining could be a useful tool in the purpose of differentiating DFP from giant or invasive dermatofibromas. Although ST3 is a "negative" marker for DFP and therefore does not demonstrate the margins of the neoplasm, it is more reliable than CD34 staining in differentiating this tumor from a dermatofibroma.