Cutaneous lymphomas other than mycosis fungoides: follow-up study of 52 patients.

Cutaneous lymphomas other than mycosis fungoides (MF) represent a rare and heterogeneous group of lymphomas. Their clinical behavior remains largely unknown. In this study, the clinical and immunohistologic characteristics and follow-up data of 52 well-documented cases of cutaneous lymphomas other than MF, presenting with initial cutaneous lesions, were reviewed. Twenty-seven patients presented with skin disease alone (stage IE), and 25 patients had concurrent cutaneous and extracutaneous disease (stage IV). The tumors were grouped into high-grade lymphomas (HGLs; 21%), intermediate-grade lymphomas (IGLs; 58%), and low-grade lymphomas (LGLs; 21%). A B-cell phenotype was most often expressed by cutaneous lymphomas (73%), particularly by stage IE lymphomas (85%). Among 13 cases of T-cell lymphomas, loss of one of the pan-T-cell antigens was detected in all cases but one. The clinical course of cutaneous lymphoma was closely dependent on stage and histologic subtype but not on T-cell or B-cell phenotype. Of 20 patients with stage IV HGL or IGL, 13 were treated by polychemotherapy with curative potential. Their median survival was 37 months. Fourteen patients with stage IE HGL or IGL were treated by radiotherapy alone. Nine patients (69%) relapsed within 2 years posttreatment. Seven of them relapsed in the skin outside the initial site involved, suggesting that radiotherapy alone is not an adequate treatment for these patients. Preliminary results concerning seven other patients with stage IE IGL or HGL treated by an initial third-generation polychemotherapy regimen are presented.

Immunolocation of TNF-alpha/cachectin in human melanoma cells: studies on co-cultivated malignant melanoma.

We have investigated the ability of metastatic cells to produce the macrophage cytokine, TNF-alpha/cachectin, as these cells have macrophage-like properties such as infiltration and migration. We looked for TNF-alpha/cachectin in three tumor cell lines derived from human malignant melanomas and six co-cultivated malignant melanomas derived, in vitro, from these three cell lines plus angioma fibroblasts. Immunohistochemistry with an anti-TNF-alpha/cachectin monoclonal antibody showed that TNF-alpha/cachectin was produced by two of the three parent melanoma cell lines. All the tumor cells in both the co-cultivated malignant melanomas and their in vitro tumorous nodules produced TNF-alpha/cachectin, even those derived from the melanoma cell line, which originally did not. The results clearly show that TNF-alpha/cachectin can be produced by non-hematopoietic tumor cells. A co-cultivated tumor model prepared from other types of human tumor cell lines promises to provide a useful tool for exploring the relationship between TNF-alpha/cachectin and oncogenesis.

Management of alarming hemangiomas in infancy: a review of 25 cases.

During the past 10 years, 25 infants with alarming hemangiomas--lesions that impaired important functions and were life threatening, especially when there was visceral involvement--have been treated. A vascular mark was present at birth in 68% of these infants. Visceral hemangiomas were associated with bulky cervicocephalic hemangiomas or with small hemangiomas scattered over the body. Among the 25 infants, 12 had laryngeal hemangiomas, 3 had hepatic hemangiomas, and 1 had gastrointestinal hemangiomatosis. Ocular sequelae, malocclusion, and cutaneous distortion were the most important functional problems. Corticosteroid treatment was used for 23 of 25 infants with alarming hemangiomas. There was a varied treatment response: total failure (30% of the patients); excellent, dramatic, rapid improvement (30% of the patients); and moderate, doubtful response, with the natural course of the disease remaining unaltered (40% of the infants). Arterial embolization, used in 6 infants, gave inconstant results. Cardiac failure, frequently associated with large cutaneous hemangiomas and always seen with hepatic multinodular hemangiomas, required digitalization. In some cases arterial embolization reduced the increased cardiac output. Liver hemangiomas had a high mortality; all 3 infants with hepatic involvement died.

Prevalence of human herpesvirus 8 infection measured by antibodies to a latent nuclear antigen in patients with various dermatologic diseases.

BACKGROUND: Human herpesvirus 8 (HHV-8) has been detected in all epidemiological forms of Kaposi sarcoma (KS). The role of HHV-8 in dermatologic diseases other than KS is controversial. Some studies based on polymerase chain reaction findings suggest an association between HHV-8 and epithelial tumors of the skin, lymphoproliferative disorders, or pemphigus. OBJECTIVE: To assess the prevalence of antibodies against a latent nuclear antigen of HHV-8 in patients with various dermatologic diseases. DESIGN: An indirect immunofluorescence assay was used to search for HHV-8 antibodies. SETTING: Ambulatory or hospitalized patients from a university hospital associated with a research laboratory. PATIENTS: Eighty-three patients with various non-KS dermatologic diseases and 16 patients with KS who were seronegative for the human immunodeficiency virus. Controls were 100 healthy subjects living in the same area. RESULTS: Antibodies to HHV-8 were found in 100% (16/16) of the patients with KS and 3.6% (3/83) of the patients with non-KS dermatologic diseases: 1 patient with pemphigus vulgaris, 1 with discoid lupus erythematosus, and 1 with bullous pemphigoid. The prevalence of antibodies to HHV-8 in controls was 2% (2/100) and was not significantly different than the prevalence in patients with dermatologic diseases other than KS (P =.28). CONCLUSIONS: Our serologic study confirms the higher prevalence of HHV-8 antibodies in patients with KS and demonstrates that contrary to other human herpesviruses, HHV-8 is not a ubiquitous virus in France. We could not determine any causal association between HHV-8 and pemphigus or lymphoproliferative disorders of the skin.

Dioxins in adipose tissue of non-occupationally exposed persons in France: correlation with individual food exposure.

We evaluated individual adipose tissue (subcutaneous lipomas) dioxin contamination in non-occupationally exposed persons living in France (adult patients undergoing a surgical ablation of benign lipomas), in relation to the corresponding individually evaluated mean daily dietary dioxin intake (DDDI). The diet survey (questionnaire) included information on consumption of meat, fish, milk and dairy products, from which the individual DDDI was calculated. Sixteen subjects participated in this study. DDDI ranged between 1.06 and 3.31 pg I-TEQ/kg body weight, bw (mean value: 2.05+/-0.72). Adipose tissue polychlorinated dibenzo-p-dioxins (PCDD)/polychlorinated dibenzofurans (PCDF) levels ranged between 18.5 and 76.9 pg I-TEQ/g lipids (mean value: 35.6+/-14.8). No relation was found between the DDDI and adipose tissue PCDD/PCDF concentrations. The mean DDDI in France does not fundamentally differ from those found in other industrialised countries, is within the range of 1-4 pg I-TEQ/kg/day recently suggested by WHO-ECEH/ICPS for the tolerable daily intake of dioxins. Adipose tissue PCDD/PCDFs levels are similar to levels in other European countries and USA without relation to sex or age, and can be considered representative European background concentrations. Globalisation of alimentary production leads to a similar food exposure in Western European countries, in spite of dioxins accidental selective contaminations that are epiphenomenon and thus do not have any impact in human dioxin background levels.

Angio-tumoral complex in human malignant melanoma characterised by free laminin: ultrastructural and immunohistochemical observations.

Neo-vessel density in primary tumours as a prognostic factor for metastasis has been questioned. For this reason we have investigated qualitative aspects of tumour vascularity and particularly the association of cancer cells with endothelium, focussing on the peri-endothelial matrix. We have examined the matrix between endothelium and tumour cells in human invasive and metastatic malignant melanoma using transmission electron microscopy and immunohistochemistry. We have identified a hitherto unrecognised image (the angio-tumoral complex) in which the tumour cell and endothelium are in direct contact via an amorphous matrix. This amorphous matrix lacks an organised lamina and contains predominantly laminin with noticeably less collagen type IV. In this image endothelial cells showed no signs of physiological damage, no tumoral intravasation, and tumour cells occupied a pericytic location. This typical image was absent from naevi. We regard the laminin in this amorphous matrix as "free' laminin as distinct from laminin integrated into an organised lamina. We discuss the role of this free laminin in promoting the migration of melanoma cells in contact with vessels and suggest the possibility that this angio-tumoral complex represents a marker for metastasis.

Ultrastructural and immunohistochemical observations concerning laminin in B16 melanoma. Is an amorphous form of laminin promoting a non hematogenous migration of tumor cells?

The gravity of cancer is related to the propensity of tumor cells to migrate from a primary site to distant organs. It is generally accepted that tumor migration occurs in the vascular stream, via the endothelial basement membrane or lamina. A recent study identified in human malignant melanomas an angio-tumoral association (termed the angio-tumoral complex) characterized by an amorphous material juxtaposed between endothelial cells and tumor cells that contained laminin. The absence of any sign of intravasation and the pericytic location of tumor cells in this typical image raised the question of the role of these complexes in promoting tumorigenesis. Using the mouse B16 melanoma model, we observed an increase of angio-tumoral complexes with tumor progression, again without any evidence of intravasation. Given the role of laminin in migration and metastasis, we discuss a non hematogenous mechanism of tumor migration along the abluminal surface of endothelium.

Angio-tumoral laminin in murine tumors derived from human melanoma cell lines. Immunohistochemical and ultrastructural observations.

Cells in tissues interact with each other and with the extracellular matrix as part of a structural and informational unit. During cancer progression, tumor cells participate in the formation of a neotissue involving other cells and matrix. We recently observed in melanoma an association between tumor and endothelial cells via an amorphous matrix containing free laminin. The pericytic location of melanoma cells in this angio-tumoral complex raised the question of an intramesenchymal migration of metastatic melanoma cells promoted by free laminin along the endothelium. However the respective roles of melanoma cells and endothelial cells in laminin secretion were not clear. In an attempt to clarify the latter issue, we injected into mice three human melanoma cells lines, one secreting laminin and two that did not, in order to identify the source of laminin secretion in the subsequent interactions between tumor cells and vascular endothelium. Using immunohistochemistry and electron microscopy we observed in all three cases an amorphous matrix containing laminin between tumor and endothelial cells. The fact that two cell lines did not secrete laminin suggests that the periendothelial/peritumoral laminin could be of endothelial origin. Given the presence of laminin alone during intramesenchymal angiogenesis and embryogenesis, we propose an analogous role for endothelial laminin in facilitating the migration of melanoma cells along the abluminal surface of the endothelium.

[Kala-azar in immunosuppressed patients. Apropos of a case disclosed by skin lesions]. / Kala-azar chez l'immunodéprimé. A propos d'un cas révélé par des signes cutanés.

Kala-azar was revealed by skin lesions in a patient treated for recurrent Hodgkin's disease. These lesions, extremely discreet, consisted of papules on the forehead and in the peribuccal area. Histological examinations initially showed nodular infiltration of the dermis with very few Leishman-Donovan bodies; subsequently, foamy, Virchow-type histiocytes appeared, and Leishman-Donovan bodies became numerous. This clinical and histological similarity with leprosy has already been noted for post-kala-azar dermal leishmaniasis which is frequent in India and in Ethiopia. Skin lesions in kala-azar appear to be exceptional since they were not observed in the 7 cases of kala-azar in immunodepressed patients previously published. The fact that they are discreet may account for their apparent rareness. Easily accessible, they make it possible to diagnose visceral leishmaniasis--an often difficult diagnosis in these patients owing to the underlying disease. Immunodepression has little influence on the clinical and biological manifestations of kala-azar, but reserves must be made concerning the usual criteria for cure.

[Role of corticoid-antibiotic combinations in local treatments. Critical study]. / Place des associations corticoïdes-antibiotiques dans les traitemen'ts locaux. Etude critique.

Current literature about steroid antibiotic combinations has been critically reviewed. The use of such combinations may be considered in infected steroid sensitive dermatosis. Definition of super-infection is however impossible on clinical data only. A single study demonstrated the superiority of the combination upon the steroid alone in well defined experimental conditions. This superiority is not demonstrated beyond 8 days of treatment or in case of pretreatment with local antiseptic solutions or baths. Theoretical side effects of local antibiotics include, sensitization, selection of antibiotic resistant germs, systemic toxicity. They are not troublesome in current practice. However adjunctions of an antibiotic can diminish the steroid efficacy when infection is not present. The real usefulness of the combination in current practice is still to be demonstrated.

[Proteus syndrome. 8 cases]. / Syndrome Protée: huit cas.

INTRODUCTION: Proteus syndrome most often includes: corporal hemi-hypertrophia, gigantism of the extremities with hyperplasia of the palms and soles, vascular abnormalities and varied types of other hamartomatous tumours. The dysmorphic growth generates functional orthopaedic and orthognatic disabilities that increase with age. OBSERVATIONS: We report, on eight cases, our experience in management of Proteus syndrome abnormalities and the benefits of new techniques in imaging and interventional vascular radiology. The correction of limb orthopaedic abnormalities is complicated with elephantiasic enlargement of soft tissues. In some patients the absence of a deep venous network contra-indicates transcutaneous occlusion of dysplatic vessels. Facial asymmetry and orthognatic anomalies are better managed after permanent teeth have appeared. COMMENTARIES: The management of these disabilities is carried out by staff of different specialities but should be organized by a practitioner who regularly follows the child and detects early the functional disturbances. Aesthetic corrections are performed later.

[Hirsutism. Two familial cases of 21-hydroxylase deficiency]. / Hirsutisme. Deux observations familiales de déficit en 21-hydroxylase.

INTRODUCTION: One to six percent of women with hirsutism have 21-hydroxylase deficiency. Beyond these classical congenital forms, the most frequent cases present non-specific signs of hyperandrogenism. The diagnosis of the deficiency is based on 17-hydroxy-progesteron (17-OHP) assay before and after ACTH stimulation. CASE REPORT: We observed 2 sisters with a non-classical deficiency in 21-hydroxylase. Their phenotypes were very different: the elder sister had severe hirsutism and clitoromegaly and was overweight; the younger sister had more moderate hirsutism with no other signs of hyperandrogenism. Hormone patterns were also different. Both baseline and stimulated plasma 17-OHP were higher in the elder sister (47 ng/ml to 126 ng/ml and 5.2 ng/ml to 42.8 ng/ml respectively). The genotype was however identical. Both were homozygous for CYP21A duplication and short C4B and had a Val281Leu mutation on exon 7 of the CYP21B gene. DISCUSSION: This phenotypic variability in subjects with the same genotype could be explained by increased sensitivity to androgens or, more likely, to the presence of currently undetectable mutations. Genetic counselling is often requested due to the high frequency of non-classical forms and the large number of heterozygous subjects with the 21-hydroxylase trait. It is thus important to screen for 21-hydroxylase deficiency in women with hirsutism. In addition, molecular assessment of CYP21B gene should be proposed for all subjects with a non-classical form as well as family members (at least the father and mother).

[Diffuse normolipidemic plane xanthomatosis and monoclonal dysglobulinemia. A case with anti-lipoprotein activity of monoclonal immunoglobulin and hypocomplementemia]. / Xanthomes plans disséminés normolipémiques et dysglobulinémie monoclonale. Un cas avec activité anti-lipoprotéine de l'immunoglobuline monoclonale et hypocomplémentémie.

INTRODUCTION: Diffuse normolipaemic plane xanthomatosis associated with monoclonal dysglobulinaemia has been recognized as a clinical entity for more than 30 years. Antilipoprotein activity has been reported in certain cases with monoclonal immunoglobulinaemia or hypocomplementaemia. CASE REPORT: A 64-year-old woman developed simultaneously diffuse normolipaemic plane xanthomatosis and monoclonal IgG kappa dysglobulinaemia. This paraprotein was initially associated with monoclonal gammapathy of undetermined signification (called "benign") before evolving into a myeloma. This aggravation was associated with an expansion of the skin lesions. Immunological exploration demonstrated the presence of IgG-lipoprotein complexes in the plasma and hypocomplementaemia suggesting incomplete activation of the classical pathway (low CH50 and fraction C4). DISCUSSION: According to the literature, the most probable hypothesis for the pathogenesis of the xanthomatosis-dysglobulinaemia association is a specific interaction between the monoclonal immunoglobulin and lipoprotein metabolism. In normolipaemic forms, the immunoglobulin-lipoprotein complexes are recognized by "waste-receptors" and accumulate in the dermal macrophages while the mechanism of lipoprotein captation in the other types of cells remains normal, explaining the absence of hyperlipidaemia.

[Congenital cutaneous Langerhans histiocytosis. Apropos of 7 cases]. / Histiocytoses langerhansiennes congénitales cutanées. A propos de 7 cas.

Seven cases of congenital Langerhans' cell histiocytosis (LH) are reported, with emphasis on clinical and immunohistochemical features. This is a polymorphic disease at birth. In 4/7 cases, the diffuse, generalized rash could be classified as cutaneous Letterer-Siwe disease (LSD); 3/4 remained purely cutaneous and healed in less than 3 months; whereas the fourth-one persisted, pulmonary lesions appeared, and the infant died on his 40th day. In 3/7 cases, the clinical diagnosis at birth was either a Blueberry Muffin Baby (BMB) or Hashimoto-Pritzker type LH (HPLH); the lesions healed rapidly, although one cas was contradictory: typical BMB at birth, histology mimicking a monoblastic cutaneous leukemia, no T.O.R.C.H. syndrome, normal bone marrow, immunophenotyping of LH, auto-involution; 2/3 were MZ twins, both with few lesions. We would like to stress the fact that the clinical spectrum of LH should include BMB, which, however, in most cases must be considered a differential diagnosis. Regarding cutaneous congenital LH, an eponymic classification (LSD, HPLH) is difficult to follow strictly, because overlapping pictures are observed. There is a wide spectrum of cutaneous congenital LH. The main problem at birth is the lack of prognostic criteria. Neither the presence of the rash at birth, nor its type and extension, is necessarily evidence of risk of systemic disease. Cases of HPLH involute, as also do cases of cutaneous LSD, and the "Blueberry Muffin" type of LH; overlapping clinical aspects exist. Histopathological data, electron microscopy or immunohistochemistry, define LH, but they do not enable the outcome to be predicted.(ABSTRACT TRUNCATED AT 250 WORDS)

[Androgenic alopecia revealing an androgen secreting ovarian tumor]. / Alopécie androgénétique révélant une tumeur ovarienne androgéno-secrétante.

INTRODUCTION: Androgen-producing tumors of the ovary are rare in postmenopausal women and are revealed by severe virilization. Leydig hilus cell tumors are the most frequent postmenopausal virilizing tumors. In this report, an unusual and rare cause of alopecia due to Leydig cell hyperplasia within the wall of a simple cyst and in the ovarian hilus is described. OBSERVATION: An 80 year-old woman complained of a 10-year history of severe androgenic alopecia associated with very mild facial hirsutism, without others signs of virilization. Hormonal blood levels showed markedly elevated testosterone. Computed tomographic scan of the adrenals and the ovaries revealed an enormous left ovarian cystic mass. Bilateral hystero-ophorectomy was performed. Histological examination demonstrated bilateral Leydig cell hyperplasia within the wall of the cyst and in the right ovarian hilus. Two months postoperative hormonal evaluation demonstrated dramatically decreased plasma levels of testosterone. COMMENTARY: The clinical, X ray and histologic aspects of this case, although rare, show that the presence of virilization should lead to a search for an androgen-secreting ovarian or adrenal tumor.

[Cranial fasciitis of childhood]. / Fasciite du cuir chevelu (cranial fasciitis) de l'enfant.

INTRODUCTION: A nodule of the scalp in a child of less than eleven years should evoke a cranial fasciitis among other serious diagnoses. OBSERVATION: A four-month old infant had a firm and pink nodule at the left parietal level, exhibiting a slow growth since two months. It was excised. The pathologic sample showed spindle-shaped cells within a myxoïde matrix, with a strong reactivity for smooth muscle actin (immunohistochemical analysis). Diagnosis of cranial fasciitis was made. Due to the results of pathology, it was possible to rule out the diagnosis of sarcoma, therefore, no complementary work-up was performed. Evolution was favorable. DISCUSSION: Cranial fasciitis is a diagnosis to be considered when confronted with a firm nodule of the scalp in a infant or a young child, with or without bone involvement. This is a benign lesion but worrying pathological signs may exist, making diagnosis of benignity difficult. Exeresis of the lesion, even incomplete, protects the child from possible recurrence. Evolution is always good.

[Cutaneous malacoplakia: a pediatric case]. / Malakoplakie cutanée: un cas pédiatrique.

INTRODUCTION: Cutaneous malakoplakia is an inflammatory disease characterized by granulomatous accumulation of distinctive phagocytic macrophages. It occurs mainly in visceral or orificial areas; the condition is rarely purely cutaneous, and appears to be extremely rare in childhood. CASE REPORT: A facial cutaneous crusted lesion was diagnosed as cutaneous malakoplakia in an immunocompetent child. The lesion had been excised twice and it had recurred, and the diagnosis was made possible only with a third biopsy, after a 2-year chronic expansion. This third biopsy revealed a dense granulomatous inflammation with numerous phagocytic histiocytes containing abundant fine granules and round Michaelis-Gutmann bodies, both staining with PAS, Perls and von Kossa. Biopsy cultures revealed only growth of two different streptococcus (group B) strains. The lesion resolved after a 4-month period of antibiotic therapy, including roxithromycin, ampicillin and trimethoprim-sulfamethoxasole. DISCUSSION: Diagnosis of malakoplakia is mainly made by histopathologic examination of tissue excision or biopsies. There are no specific clinical features. Most reported cases of this uncommon phagocytic reaction to common bacteria have developed in the genitourinary areas (71 p. 100); purely cutaneous localisation, as in our patient, are rare (4 p. 100). Intracytoplasmic granules may result from phagolysosomes and incomplete bacterial killing, with subsequent deposit of iron and calcium in the phagocytic macrophages. A number of reported cases have affected immunocompromised patients with either congenital immunodeficiency or secondary immunodeficiency. The most effective treatment option is based on a protracted antibiotherapy, using drugs that easily permeate the macrophages, e.g. quinolones and trimethoprim-sulfamethoxasole. Lesion may recur after surgical excision.

[Multifocal epithelioid hemangioendothelioma with partial remission after interferon alfa-2a treatment]. / Hémangioendothéliome épithélioïde multifocal en rémission partielle sous traitement par interféron alpha 2a.

INTRODUCTION: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumour of the soft tissue having an intermediate malignancy. Cutaneous presentations have exceptionally been reported. OBSERVATION: A young woman, initially affected by an EHE with cutaneous and bone involvement, then with pulmonary and hepatic localizations, was in partial remission after one year of treatment with alpha 2a interferon. DISCUSSION: In a critical review of the literature, we describe the main features of this tumour and the difficulties to classify this entity in the vascular tumors' spectrum. Histologically, epithelioid hemangioendothelioma has a very characteristic appearance and is clearly different from other vascular tumors as angiolymphoid hyperplasia with eosinophilia and Kimura's disease. The assumption of a multicentric or a metastatic origin when multiple tumor deposits are found, is still not elucidated. Different therapeutic approaches are proposed: surgery, interferon, interleukin-2, retinoids. Randomized studies would be necessary to confirm our results with alpha 2a interferon but are probably difficult to realize because of the lack of cases.

[Paget disease of the vulva. 36 cases]. / Maladie de Paget vulvaire: 36 cas.

Thirty six patients with Paget's disease of the vulva were reviewed. The median age of the patients at diagnosis was 67 years (range: 45-91 years). One patient had a history of previous mammary adenocarcinoma. Screening for malignancy revealed two colonic tumours. Two patients with negative screening at presentation developed, 12 and 18 months respectively after vulvectomy, an ovarian carcinoma stage IIc and a cervical and urethral adenocarcinoma. All patients were treated by surgery based on extent of the disease. The operations performed included total vulvectomy (n = 11), partial vulvectomy (n = 14) and wide local excision (n = 4). Out of the 36 patients, 29 were available to follow-up. The median follow-up period was 74 months (range 2-204 months). Three patients died of metastatic disease due to vulval adenocarcinoma and breast carcinoma, or of liver metastases from an unknown adenocarcinoma. Eighteen of the 29 patients followed up remained free of disease. Five out of the 16 patients with positive margins recurred, as did 5 out of 9 patients with negative margins. Treatment of Paget's disease of the vulva is surgical. In order to prevent recurrence, some authors have proposed surgical excision extending beyond the visible clinical lesions with intraoperative frozen sections. The data we recorded show that free margins do not seem to correlate with recurrence, so that large excision beyond the clinical lesion is not useful.

[Recurrent nodular panniculitis associated with pancreas divisum]. / Panniculite nodulaire récidivante associée à un pancreas divisum.

We report the case of a 46-year old woman who, over a 12-year period, had 6 episodes of nodular panniculitis. Each lesion regressed and disappeared within four to six weeks. Fistulization before formation of a cupula-shaped scar was observed on two occasions. These episodes of hypodermitis seemed to be isolated when they began, but histological examination of the skin showed cytosteatonecrosis of the hypodermis pointing to a pancreatic origin. Signs of pancreatic abnormality appeared progressively: first an increase of amylasaemia concomitant with the cutaneous flares, then morphological abnormalities of the pancreatic ducts suggestive of pancreas divisum, and finally dilatation of the main pancreatic duct associated with upstream pancreatitis. No radical treatment of the pancreatic abnormality was carried out.