Familial ACD with no recognizable enzyme abnormalities.
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Adult↗
Biomedical subjects
Bernard Cribier
Publications and source records attributed to Bernard Cribier.
Neural hyperplasia in chondrodermatitis nodularis chronica helicis.
BACKGROUND: Chondrodermatitis nodularis chronica helicis (CNCH) is a common condition characterized by tender nodules of the helix, of uncertain mechanism. The pain is not explained by current physiopathological hypotheses. OBJECTIVE: We sought to investigate possible nerve hyperplasia in CNCH. METHODS: Thirty-seven cases of clinically typical CNCH were submitted to conventional microscopy after surgical excision and analyzed with immunohistochemistry using PS100 and neurofilament antibodies. As controls, we investigated 25 tumors of the ear with the same methods. RESULTS: Large nerves (>0.1 mm in diameter) were seen in 8 of 37 cases; these were close to the cartilage or at the upper part of the ulceration. Increased numbers of small nerve sections (0.01-0.06 mm in diameter) were seen in 35 of 37 cases, with more than 20 sections per low-power field in 18 cases. Nerve hyperplasia was not observed in tumors of the ear, even in ulcerated cases. LIMITATIONS: Hypothesis based on morphologic observations. CONCLUSIONS: Nerve hyperplasia is present in CNCH, but is often masked by intense vascular and inflammatory reactions. This finding may explain the induction of pain by light pressure, whereas ulcerated auricular malignant tumors are generally painless. Certain authors believe that CNCH is an equivalent of prurigo nodularis, which interestingly also shows nerve hyperplasia.
Adult↗
Expression patterns of hair and epithelial keratins and transcription factors HOXC13, LEF1, and beta-catenin in a malignant pilomatricoma: a histological and immunohistochemical study.
BACKGROUND: We have previously shown that benign pilomatricomas not only maintain the sequential expression of the hair matrix and precortex keratins hHa5 and hHa1 of normal hair follicles in their transitional cell compartment, but also preserve the association of hHa5 expression with that of its regulatory homeoprotein HOXC13 in the lower transitional cell compartment. In contrast, hHa1 expression in the upper transitional cell compartment is uncoupled from the nuclear co-expression of the LEF1/beta-catenin complex seen in normal hair follicles (Cribier et al., J Invest Dermatol 2004; 122: 1078). METHODS: Formalin-fixed paraffin sections of the tumor were examined using a panel of mono- and polyclonal hair and epithelial keratin antibodies as well as antibodies against HOXC13, LEF1, and beta-catenin. RESULTS: Morphologically, the malignant pilomatricoma investigated here clearly deviated from the described major tumor type by a large number of differently sized parakeratotic squamoid whorls emerging within the mass of basaloid cells and surrounded by cells remembering transitional cells, but only rarely containing shadow cells and signs of calcification. We show that hHa5/HOXC13 co-expression was maintained in transitional cell areas, in which hHa1 expression was much stronger than in benign pilomatricomas, but again uncoupled from concomitant nuclear LEF1/beta-catenin expression. Surprisingly, however, and in clear contrast to benign pilomatricomas, these transitional cells co-expressed the epithelial keratins K5, K14, and K17, with the latter being as strongly expressed as hHa1, both also staining the entire inner mass of the parakeratotic whorls. CONCLUSIONS: Although the malignant pilomatricoma investigated here was distinctive in that it contained a multitude of parakeratinizing whorls and no signs of calcification, it shared both hHa5/HOXC13 co-expression and disrupted hHa1/beta-catenin-LEF1 expression in its transitional cell compartment around the whorls with benign pilomatricomas. However, in clear contrast to the latter, transitional cells of the malignant tumor also strongly expressed the epithelial keratins K5, K14, and K17. We speculate that the observed dominance of the epithelial differentiation pathway over the competing conventional shadow cell differentiation pathway may prevent massive calcification of the tumor.
Aged, 80 and over↗
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.
Adult↗
Urticaria and hepatitis.
Acute urticaria is commonly observed in the prodromic stage of hepatitis A and B infection as well as in hepatitis C infection, although only rare cases have been published regarding the latter. Urticaria is considered one of the pre-icteric symptoms of viral hepatitis and is related to immune-complex deposits; subsequently, it may be associated with arthritis and headache (Caroli's triad). The absence of specific presentation of acute urticaria in patients with viral hepatitis is not surprising because many other viral infections can induce similar cutaneous symptoms. On the other hand, no convincing evidence exists in which hepatitis virus infection caused chronic urticaria. Data are lacking for hepatitis B, but several series and one controlled study showed the absence of a link between hepatitis C and chronic urticaria. Systematic hepatitis virus investigations in patients with chronic urticaria probably are not cost-effective. Hepatitis B or C can occasionally induce urticarial vasculitis, but this is probably related more to vasculitis than to true urticaria.
Hepatitis A↗
Safety, efficacy, and dosage of 1% pimecrolimus cream for the treatment of atopic dermatitis in daily practice.
INTRODUCTION: Although several controlled clinical trials have demonstrated the efficacy and good tolerability of 1% pimecrolimus cream for the treatment of atopic dermatitis, the results of these trials may not apply to real-life usage. The objective of this study was to evaluate the safety and efficacy of a pimecrolimus-based regimen in daily practice. METHODS: This was a 6-month, open-label, multicenter study in 947 patients aged >or=3 months with atopic dermatitis of all severities. The investigators incorporated 1% pimecrolimus cream into patients' standard treatment protocols on the basis of their clinical diagnosis. Use of topical corticosteroids was allowed at the discretion of the physician. Safety and tolerability were evaluated by monitoring adverse events. Efficacy was evaluated by recording changes in the Investigators' Global Assessment scores and pruritus scores at each visit. RESULTS: No clinically unexpected adverse events were reported. The discontinuation rate for adverse events was 2.3%. The disease improvement rate was 53.7% at week 1 and 66.9% at week 24. The pimecrolimus-based regimen was particularly effective for the treatment of lesions involving the face (improvement rate: 61.9% at week 1 and 76.7% at week 24). The greatest therapeutic response was experienced by pediatric patients with mild or moderate disease. Nonetheless, 64% and 65% of infants and children, respectively, with severe/very severe facial disease at baseline were clear/almost clear of signs of atopic dermatitis on their face at week 24. In patients aged <18 years, most of the improvement occurred within the first week of treatment, while in adults a progressive improvement was observed over the entire study period. Worsening of disease by the end of the study occurred in 9.5% of patients and was most frequent in adults (12.6%). The discontinuation rate for unsatisfactory therapeutic effect was 4.8%. The mean number of treatment days was 135.6 (SD 53.2). The mean drug consumption (non-US centers only) was 4.2 g per treatment day. Drug consumption decreased over time as disease improved. In total, 47% of patients who completed the study never used topical corticosteroids over 6 months. CONCLUSION: In daily practice, incorporation of 1% pimecrolimus cream into patients' standard treatment regimen is well tolerated and improves atopic dermatitis in approximately two-thirds of patients. Disease improvement is particularly evident on the face. The greatest therapeutic response is experienced by pediatric patients with mild or moderate disease. In these patients, most of the improvement is observed within 1 week from the start of treatment.
Administration, Topical↗
[Lupus panniculitis].
Lupus panniculitis is characterized by a T-cell lymphocyte infiltration of the fat, with fibrosing and cicatricial progression. The lesions are predominantly found on the head and the upper part of the body; they are more frequent in young women. Diagnosis is based on the integration of the clinical and histological data, which renders a deep cutaneous biopsy indispensable. Histopathology is the key to the differential diagnosis, which essentially includes subcutaneous T-cell lymphoma. The lupus panniculitis lesions can be isolated or, more often, integrated within a known lupus disease, usually of good prognosis. Treatment relies above all on synthetic anti-malarials, occasionally associated with local or general corticosteroids.
Adrenal Cortex Hormones↗
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.
Adult↗
History: Frederic Woringer (1903-1964) and Woringer-Kolopp disease.
The two most famous dermatologic eponyms born in Strasbourg are Pautrier microabscess and Woringer-Kolopp disease. Frederic Woringer (1903-1964) was one of Pautrier's students, who had been in charge of the Laboratoire d'Histopathologie Cutanée in Strasbourg from 1930 until his death. He achieved a brilliant career in the field of dermatopathology and was very active during the great period between World War I and II. His name is linked to a rare disease, pagetoid reticulosis, which he actually misunderstood, as he was at first convinced that the characteristic intraepidermal changes were due to Paget cells. In this article, we show for the first time the original correspondence between Kolopp (who sent the case to Strasbourg) and Woringer, including discussion with masters such as Pautrier and Civatte. Civatte suggested that these mysterious intraepidermal cells might have common morphologic features with the recently described Sezary cells. After the first publication of pagetoid reticulosis, it took almost 40 years to establish a clear link with mycosis fungoides. It was Braun Falco who named the disease after Woringer and Kolopp in 1974. Woringer would certainly be surprised to know that his name is still world famous, thanks to a rare disease he was the first to extensively describe in 1939, a few days before the declaration of war.
Dermatology↗
Characterization of a novel human type II epithelial keratin K1b, specifically expressed in eccrine sweat glands.
In this study, we show that a novel human type II epithelial keratin, K1b, is exclusively expressed in luminal duct cells of eccrine sweat glands. Taking this luminal K1b expression as a reference, we have used antibodies against a plethora of epithelial keratins to systematically investigate their expression in the secretory globule and the two-layered sweat duct, which was divided into the intraglandular, intradermal, and intraepidermal (acrosyringium) segments, the latter being further subdivided into the sweat duct ridge and upper intraepidermal duct. We show that (i) each of the eccrine sweat gland tissue compartments expresses their own keratin patterns, (ii) the peripheral and luminal duct layers exhibit a sequential keratin expression, with both representing self-renewing cell layers, (iii) the intradermal duct and the sweat duct ridge display hitherto unknown length variations, and (iv) out of all cell layers, the luminal cell layer is the most robust layer and expresses the highest number of keratins, these being concentrated at the apical side of the cells to form the cuticle. We provide evidence that the cellular and intercellular properties of the peripheral and the luminal layers reflect adaptations to different functions.
Amino Acid Sequence↗
Primary cutaneous cribriform carcinoma: a rare apocrine tumour.
BACKGROUND: Primary cutaneous cribriform carcinoma (PCCC) is a rare apocrine tumour occurring in middle-aged people. This neoplasm is often located on the limbs. The histopathological diagnosis is difficult, mainly because this tumour is exceptional. We, in this study, report a patient with PCCC. CASE REPORT: The patient was a 37-year-old man who presented with a nodule of the left knee. RESULTS: Histopathologic findings showed an asymmetrical deep dermal tumour with a cribriform pattern. The aggregations of neoplastic cells were interconnected and varied in size and shape. The cells were arranged in solid nests or tubular structures. In the lumina of tubules, some papillary protrusion of basophilic cells was seen. The ductal elements were lined by cuboidal or cylindric cells with images of decapitation secretion. The nuclei of the neoplastic cells were pleomorphic. A wide excision was performed with sentinel inguinal node biopsy. After a 2-year follow-up, neither persistence at the local site nor metastasis was observed. CONCLUSIONS: Clinical and pathological features of PCCC are reviewed. Differential diagnoses, including cutaneous metastasis of adenocarcinoma, adenoid basal cell carcinoma and adenoid cystic carcinoma, are discussed.
Adenocarcinoma↗
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.
Cytoskeletal Proteins↗
[Facial dermatitis. Acne, rosacea, seborrheic dermatitis].
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Acne Vulgaris↗
Reliability of the histopathologic diagnosis of malignant melanoma in childhood.
OBJECTIVE: To assess interrater reliability in the diagnosis of malignant melanoma in children. DESIGN, SETTING, AND PARTICIPANTS: We collected 85 slides of melanomas diagnosed in patients younger than 17 years through a network of dermatopathologists and dermatologists. The slides were classified into 3 categories: (1) slides from children with metastatic melanoma; (2) slides from disease-free children with a follow-up of less than 5 years; (3) slides from disease-free children with a follow-up of 5 years or longer. Category 1 was considered the gold standard. Four pairs of expert dermatopathologists reviewed the slides and classified them into melanoma, nevus (including Spitz nevus), or ambiguous tumors. INTERVENTION: None. MAIN OUTCOME MEASURE: Concordance between pairs of experts. RESULTS: For category 1 slides (n = 20), the concordance was weak to moderate. For category 2 slides (n = 47), the concordance was weak. For category 3 slides (n = 18), the concordance was poor to moderate. CONCLUSION: This study demonstrates that the reliability of diagnosis of melanoma in childhood is poor, even when submitted to experts.
Adolescent↗
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.
Adult↗
Inflammatory vitiligo-like macules that simulate hypopigmented mycosis fungoides.
Two cases of an inflammatory vitiligo-like condition that simulated mycosis fungoides are reported. Both patients presented acquired hypopigmented macules sharply limited by an erythematous and papular border. The clinical aspect was suggestive of inflammatory vitiligo. Mycosis fungoides was suspected on skin specimens showing a dense band-like lymphocytic infiltrate with discrete nuclear atypias and marked exocytosis. This infiltrate was made of CD3 positive lymphocytes. CD8 positive lymphocytes were numerous in one case, few in the other. There was a loss of melanocytes in the lesional skin and absence of dominant T-cell clones in both cases. No repigmentation was observed after PUVA or local chemotherapy. The authors emphasized that erythematous and papular borders surrounding hypopigmented macules, CD8 positive lymphocytic infiltrate, absence of T-cell clonal rearrangement are helpful to rule out mycosis fungoides.
Adult↗
Retinoic-acid receptor beta expression in melanocytes.
Retinoic acid receptors (RAR) and retinoic X receptors (RXR) are critical for skin homeostasis. In epidermis, RXRalpha and RARgamma isoforms are highly expressed but only weak or no expression of RARbeta has been reported. Here, we re-examined RARbeta in situ expression in comparison with that of the RXRalpha, in both normal skin and melanocytic tumours. In normal skin, RXRalpha was localized in epidermis, sebaceous glands and hair follicles, while RARbeta was detectable only in melanocytes and in stratum granulosum. RXRalpha was never detected in melanocytic tumours, neither in nevi, nor in melanomas. RARbeta was also absent from melanoma cells but was present in nevus cells. These results indicate that melanoma are characterised by simultaneous decrease of RARbeta and absence of RXRalpha that may be responsible for the RA-resistance of most melanoma cell lines.
Biopsy, Needle↗