Anatomoclinical study of 30 cases of sclerosing sweat duct carcinomas (microcystic adnexal carcinoma, syringomatous carcinoma and squamoid eccrine ductal carcinoma).

BACKGROUND: Microcystic adnexal carcinoma (MAC), syringomatous carcinoma (SC) and "Squamoid eccrine ductal carcinoma" (SEDC) are rare sclerosing adnexal tumours. OBJECTIVE: To understand the histogenesis of these tumours and possible clinical implications. METHODS: We conducted a retrospective study of 30 cases, 18 MAC, 5 SC and 7 SEDC reviewed and classified by a panel of dermatopathology experts, with immunohistochemical analysis of keratins, including K77, a new keratin specific of eccrine ducts, and PHLDA1 expressed in adnexal structures. RESULTS: There was a strong female predominance, with only five cases occurring in men. Patients with MAC and SC were younger (mean age 56 and 47 years) than those with SEDC (mean age 81 years). The most common localization was the cheek in SC and SEDC and the periocular area in MAC. Two cases of SEDC were found in organ transplant patients. No recurrence or metastases were observed after complete surgery of MAC, or SC (mean follow-up 7.2 years and 4.7 years), whereas one case of SEDC recurred and another could not be fully excised. MAC and SC had similar histological features, except for cysts. In MAC, calcifications, granulomas, connection to follicles, keratin expression pattern, PHLDA1 positivity and K77 negativity indicated a follicular histogenesis, whereas in SC, K77 positivity and keratin expression pattern were consistent with a differentiation towards eccrine apparatus. SEDC was composed of strands centred by ducts and nests with squamous differentiation and displayed K77 ductal positivity in all cases, a finding consistent with an eccrine origin. CONCLUSION: Our study demonstrated that MAC and SC have similar clinical characteristics, although histogenesis differs and show arguments for the individualization of SEDC.

[Urticarial vasculitis associated with essential thrombocythaemia progressing to myelofibrosis]. / Vascularite urticarienne associée à une thrombocytémie essentielle avec myélofibrose secondaire.

BACKGROUND: Urticarial vasculitis (UV) is a rare form of leukocytoclastic vasculitis in which skin lesions resemble urticaria. UV comprises hypocomplementemic and normocomplementemic subtypes. To date, only 4 cases of UV associated with myeloproliferative disorders have been described, including 3 cases with essential thrombocythaemia (ET) and one case with polycythaemia vera. PATIENTS AND METHODS: We describe the case of a 59-year-old male patient with JAK2-positive TE and secondary myelofibrosis and who developed multiple urticarial papules persisting for more than 24hours. Skin biopsy showed perivascular neutrophilic infiltrate with margination of neutrophils in the lumen of vessels and some leukocytoclastic patterns, and with red cell extravasation consistent with UV. Treatment with ruxolitinib (a JAK2 inhibitor) induced transient and partial control of the haematological symptoms but did not prevent UV flare. Prednisolone 20mg once daily was added, with good clinical response. DISCUSSION AND CONCLUSION: To our knowledge, this is the fourth reported case of UV associated with ET and the first case associated with MF.

[Nevoid acanthosis nigricans or RAVEN (rounded and velvety epidermal nevus): three cases]. / Acanthosis nigricans nævoïde ou "RAVEN" (rounded and velvety epidermal nevus): trois cas.

BACKGROUND: We report three cases of a peculiar rash with mixed clinical features of both epidermal nevus and acanthosis nigricans. Their characteristics have been compared to those of very rare but similar cases found in the medical literature. PATIENTS AND METHODS: Two young adults (one male, one female) and a 7-year-old boy consulted for hyperchromic asymptomatic plaques located respectively on the right scapula, the left upper arm and the right frontotemporal area of the face. None had any noticeable familial medical history. None were overweight or had a personal history of diabetes or endocrine dysfunction. The plaques had appeared soon after puberty in the adults and at the age of 4 years in the child and they had remained stable for years. They were polycyclic in shape, sharply demarcated, and with a linear distribution. Their surface was slightly papular, with a velvety appearance and texture. No other skin or mucous membrane lesions were observed elsewhere. The physical examination was otherwise normal. Skin biopsy specimens showed mild acanthosis with papillomatosis, hyperorthokeratosis with elongated rete ridges and slight thinning of the roof of the dermal papilla. DISCUSSION: The striking clinical features of this rash rule out confusion with any other epidermal nevus (because of age of onset, shape and texture) or with acanthosis nigricans (because of its topography and the lack of associated neoplastic or endocrine disease). A dozen similar case reports can be found in the medical literature, mostly under the term "nevoid acanthosis nigricans". Such a rash may be linked to postzygotic mosaicism with the same mutations of the FGFR3 gene. However, since the physiopathology of this rash remains hypothetical, we propose to name it "RAVEN", for "rounded and velvety epidermal nevus", which is simply a descriptive acronym and nothing more.

Exacerbation of lupus panniculitis following anti-hepatitis-B vaccination.

Even though benefits of vaccination policies have been widely demonstrated, vaccine injections might be associated with rare side effects. In this setting, the potential role of vaccines, mostly against hepatitis B virus, in the induction of autoimmunity has been a matter of controversy. We report the case of a woman followed for a lupus panniculitis which had been in remission for 3 years, who developed a lupus flare following an anti-hepatitis-B vaccine injection. The topography of recurring lupus lesions, the chronology of the flare and the increase in the antinuclear autoantibody serum level all supported a causal role for vaccination in the relapse of the lupus lesions. We believe that the present case might provide a first observation of lupus panniculitis possibly induced by hepatitis B vaccination, and this should be added to the range of dysimmune manifestations caused by vaccinations.

[Confluent and reticulate papillomatosis of Gougerot and Carteaud: a retrospective study of 9 cases]. / Papillomatose confluente et réticulée de Gougerot et Carteaud: étude rétrospective de neuf cas.

BACKGROUND: Confluent and reticulate papillomatosis of Gougerot and Carteaud is a rare entity and a subject of controversy in terms of nosology. It has occasionally been regarded as a peculiar variant of acanthosis nigricans, pityriasis versicolor or amyloidosis. OBJECTIVES: To discuss confluent and reticulate papillomatosis and its diagnostic criteria as a distinct entity. METHODS: Retrospective study from 1994 to 2005 based on photographic files from 2 dermatology wards at the Saint Louis Hospital, Paris. Files containing a precise clinical description of the rash and at least one mycological element were included. RESULTS: Nine cases were included (6 females, 3 males). These patients showed clinical features comprising elementary lesion, distribution and topography corresponding to the classical description; histology was also consistent with confluent and reticulate papillomatosis, with no signs of amylosis and negative fungal samples or failure of antifungal treatment. One patient was presenting associated acanthosis nigricans. Doxycycline was efficacious in 4 cases in which it was evaluated. CONCLUSION: Confluent and reticulate papillomatosis is a definite entity and is not a superficial fungal disease. It must be distinguished from pityriasis versicolor as well as acanthosis nigricans and cutaneous amyloidosis. The therapeutic efficacy of oral cyclines (doxycycline or minocycline) appears to be an important distinguishing feature that can serve as a diagnostic criterion.

[Acute generalized exanthematous pustulosis induced by drugs with low-digestive absorption: acarbose and nystatin]. / Pustulose exanthématique aiguë généralisée induite par des médicaments à faible absorption intestinale: l'acarbose et la nystatine.

INTRODUCTION: Acarbose and nystatin are usually well-tolerated drugs because of their minimal intestinal absorption. We report herein two cases of acute generalized exanthematous pustulosis induced by these two molecules. CASES REPORT: A 43 year-old man with a history of insulin-deficient diabetes was admitted to our department for a febrile generalized cutaneous pustular erythema, that had appeared 48 hours after acarbose (Glucor) introduction. Acarbose was discontinued and the eruption resolved in one week. A 29 year-old man developed a flexural erythema twenty four hours after nystatin (Mycostatin) treatment, progressing towards a febrile pustular erythroderma, with elevated neutrophilic and eosinophilic counts. The lesions regressed rapidly with topical steroid treatment. The patch tests performed a few months later with Mycostatin and nystatin were positive. DISCUSSION: The clinical presentation of these two patients was typical of acute generalized exanthematous pustulosis, according to the EuroSCAR group criteria and acarbose and nystatin were the most likely factors that caused the disease according to the French unexpected or toxic drug reaction assessment. The minimal intestinal absorption of these two molecules explains their usual good tolerance. However, some cases of toxiderma have already been reported. There is the first described case of acute generalized exanthematous pustulosis with acarbose. Our two observations underline the possibility of severe toxiderma induced by low-absorbed and low-blood concentration molecules and focus on the need to take them in account in the toxiderma anamnesis.

Reactive angioendotheliomatosis secondary to dermal amyloid angiopathy.

Reactive angioendotheliomatosis (RAE) is a rare benign cutaneous vascular proliferation characterized by intravascular hyperplasia of endothelial cells and tuft-like proliferation of vessels. A 75-year-old man had erythematous and violaceous macules, some stellate and others arranged in a livedoid pattern, evolving toward necrosis with central areas having an "atrophie blanche" appearance spread on the trunk, inguinal folds, and right thigh. He was on hemodialysis and had a benign monoclonal gammopathy. Cutaneous biopsy revealed RAE characterized by the proliferation of epithelioid and spindle-shaped cells in superficial and middermis lining vascular channels, arranged in clusters, and sometimes displaying an intravascular growth pattern. These cells stained for CD31, CD34, and actin. Interestingly, prominent amyloid deposits were found in the wall of some vessels in deep dermis, often causing obstruction of their lumina. The cause of RAE is unknown, but it can be associated with infections, antiphospholipid syndrome, dysglobulinemia, cryoproteinemia, and lower extremities arteritis, and it may occur near arteriovenous fistulas. In this patient, we believe that RAE was caused by obliteration of dermal vessels by amyloid deposits. Indeed, it is thought that RAE could be caused by ischemia secondary to vascular obstruction. This is the first reported patient with RAE associated with amyloid deposits.

Clinical and histopathologic features and immunologic variables in patients with severe chilblains. A study of the relationship to lupus erythematosus.

We investigated 33 patients affected with chilblain lesions following a persisting course of more than 1 month. We focused on the incidence of an underlying connective tissue disease, mostly lupus erythematosus (LE), and we analyzed features of idiopathic chilblains compared with those of chilblain lesions associated with connective tissue disease. We also carried out a prospective follow-up of patients. Eleven patients included in the study were free of any clinical and/or laboratory abnormality suggestive of connective tissue disease, while 22 of 33 patients showed 1 or several abnormalities raising suspicion for connective tissue disease, and among them 8 had a diagnosis of systemic lupus erythematosus (SLE) established at initial evaluation based on the American College of Rheumatology revised criteria. The comparative analysis of patients with idiopathic chilblains and patients with chilblains associated with LE showed that female sex and persistence of lesions beyond cold seasons were significantly associated with chilblain LE. Histopathologic studies of chilblain lesions did not reveal features typical of LE in any case, but revealed a higher incidence of a deep perisudoral infiltrate in idiopathic chilblains. In patients showing signs of connective tissue disease, positive cutaneous immunofluorescence was correlated with the presence of circulating antinuclear antibodies. Two patients had an ascertained diagnosis of SLE with severe manifestations during prospective follow-up, requiring treatment with oral steroids in both cases. Chilblains following a chronic course may reveal connective tissue disease, and patients affected with chilblains associated with autoimmune abnormalities may develop severe SLE. Accordingly, long-term follow-up of these patients is warranted.

[Cutaneous lupus erythematosus following argon laser treatment]. / Lupus érythémateux cutané déclenché par le laser argon.

BACKGROUND: It is well known that exposure to ultraviolet light can trigger lupus manifestations. Other light sources may have the same effect. We report a case of argon laser-induced lupus erythematosus. CASE REPORT: A 59-year-old women developed an erythematous edematous infiltrated and sensitive lesion over the right cheek ten days after an argon laser treatment of the retina. The lesion spread towards the chin despite antibiotic treatment. Histology examination of a biopsy specimen and direct immunofluorescence suggested the diagnosis of cutaneous lupus erythematosus. The lesions regressed in one month with hydroxychloroquine (400 mg/d) treatment. DISCUSSION: Our patient developed argon laser induced cutaneous lupus erythematosus. It is known that ultraviolet light and non-ultraviolet frequencies (x-rays, visible light) can induce lupus manifestations. One case of discoid lupus erythematosus after argon laser has been reported. In our case, due to a technical error the laser beam was directed onto the ipsilateral cheek during the laser treatment of the retina. The low-energy beams used in ophthalmology would explain the absence of local burn but would be sufficient to trigger lupus. This case demonstrates that argon laser, a visible blue or green beam, can provoke cutaneous lupus erythematosus even if there is no heat-induced burn. It is important to be aware of this adverse effect due to the widespread use of lasers in dermatology, particularly for the treatment of cutaneous lupus lesions.

[Cheyletiella dermatitis: an uncommon cause of vesiculobullous eruption]. / La cheyletellose: une etiologie inhabituelle d'eruption vésiculo- bulleuse.

BACKGROUND: Species of Cheyletiella mites are parasites hosted by dogs, cats and rabbits. In humans, they cause a dermatosis not well known by dermatologists. We report a case of an unusual, purely vesiculobullous eruption due to Cheyletiella blakei acquired from an infected cat. CASE REPORT: A 76-year-old woman presented a very pruritic eruption of vesicles and bullous lesions located on the trunk and external areas of the arms. Biopsy showed dermoepidermal cleavage and polynuclear infiltrate with prominent eosinophils, consistent with the diagnosis of bullous pemphigoid. We suspected a Cheyletiella dermatitis due to the aspect and distribution of the elementary lesions and the fact that prior to the eruption the patient had acquired a cat that sometimes slept in her bed. The diagnosis was confirmed by a veterinary examination and isolation of Cheyletiella blakei from the cat's skin. The cat was treated successfully with ivermectin, while the household was disinfected with permethrin. A treatment with benzyl benzoate (Ascabiol) resolved all the patient's symptoms. DISCUSSION: This case is particularly interesting due to the purely vesiculobullous pattern of the rash and by the difficulty and considerable delay of diagnosis. Patients who have recently acquired a cat or dog before developing a pruritic dermatosis may indeed have cheyletiellosis.

[Erythema annulare centrifugum and relapsing polychondritis]. / Dermatose à type d'érythème annulaire centrifuge et polychondrite atrophiante.

BACKGROUND: Relapsing polychondritis is a rare systemic disease. Skin involvement occurs in 20 to 50 % of cases. Cutaneous signs are most often related to a leukocytoclastic vasculitis. Association of relapsing polychondritis with neutrophilic dermatosis have also been reported. We report the first case of an erythema annulare centrifugum-like dermatosis associated with relapsing polychondritis, with a two years delay between both conditions. CASE REPORT: A 74 year-old man was seen for papulo-erythematous centrifugal annular lesions that appeared 18 months earlier in a context of bad general conditions. Biological tests were normal. Several skin biopsies were performed, showing at the beginning features of drug reaction and then of lupus-lichen. Treatment with hydroxychloroquine, topical corticosteroids, dapsone and thalidomide were unsuccessful. In the following months, the patient developed fever and relapsing bronchitis. Suddenly, a chondritis of the ears appeared, leading to the diagnosis of relapsing polychondritis. All the cutaneous, chondritic and respiratory signs disappeared with oral steroid therapy. Two years after the diagnosis of relapsing polychondritis the patient developed refractory anemia. DISCUSSION: Cutaneous signs of relapsing polychondritis are frequent and may occur several months or years before the chondritis. They are polymorphous, but to the best of our knowledge, a clinical aspect of erythema annulare centrifugum has never been described. Our observation recalls the sometimes long delay between the cutaneous and the chondritic signs of relapsing polychondritis and the high frequency of dysmyelopoiesis in relapsing polychondritis with cutaneous involvement.

[Lichen pemphigoid associated with developing hepatitis B in a child]. / Lichen pemphigoïde associé à une hépatite B évolutive chez un enfant.

INTRODUCTION: Lichen planus pemphigoides is a rare acquired auto-immune bullous dermatosis which usually affects adults. Only four cases have been reported in children. We describe a new case of lichen planus pemphigoides in a child unusual by its association with an evolutive hepatitis B and by the occurrence of a lichen planus relapse. CASE REPORT: A 10-year-old African boy has been seen for a pruritic dermatosis with papular lichenoid lesions on the trunk and the limbs and blisters on the lower limbs, both arise on lichen planus lesions and normal skin. The diagnosis of lichen planus pemphigoides was confirmed by histology which showed the features of lichen planus on a papule and of a sub-epidermal split on a bulla and by direct and indirect immunofluorescent studies which revealed an IgG and C3 linear deposit at the dermo-epidermal junction and the presence of circulating IgG anti-basement membrane zone antibodies. Laboratory investigations showed an evolutive hepatitis B (HBsAg +). Healing was obtained by dapsone and topical steroid therapy. Eight months after withdrawal of treatment the patient presented a non-bullous relapse of lichen planus. The histology showed a typical aspect of lichen planus and the immunofluorescence studies were negative. The hepatitis B serology was unchanged. The lesions rapidly improved with topical steroid and coaltar. One year later the patient exhibited few slight lichen planus lesions on the limbs and the hepatitis B serology showed the onset of sero-conversion. DISCUSSION: Lichen planus and lichen planus pemphigoides are probably variants of the same disease. Their successive occurrence in our case report favours this hypothesis as does the association with an hepatitis B.

Blind loop syndrome: an unusual cause of panniculitis.

We describe a 52-year-old woman with panniculitis and blind loop syndrome. She had undergone a gastrectomy for peptic ulcer 4 years before. Tender erythematous nodules on her palms and soles were associated with diarrhea and weight loss. A biopsy specimen revealed septal and lobular panniculitis. A glucose hydrogen breath test was consistent with bacterial overgrowth. These results were consistent with panniculitis associated with a blind loop syndrome. Only four cases of this association have been reported previously.

Malignant melanoma and granulomatosis.

Sarcoidosis or granulomatous reactions have rarely been reported in association with malignant melanoma (MM). We describe seven patients who presented with both granulomatous disease and MM, and discuss the physiopathological and prognostic significance of this association. In three patients, the granulomatosis was diagnosed as true sarcoidosis and in one patient, as tumour-associated granuloma. In three cases, designated here as atypical tumour-associated granulomatoses, the presence of clear-cut pulmonary granulomatous nodules was typical neither for sarcoidosis nor for tumour-associated granuloma and was highly suggestive of melanoma metastases. Mediastinal lymphadenopathy was present in every patient. In all seven patients, the question of mediastinal or pulmonary involvement or relapse of the MM was raised, but could be confirmed in only one patient. MM can be associated with granulomatous disease. Knowledge of this association has implications in the management of patients with MM.

Disseminated varioliform pustular eruption due to Mycobacterium avium intracellulare in an HIV-infected patient.

Severe disseminated infection due to Mycobacterium avium intracellulare, with unusual cutaneous features, is reported in a patient with acquired immunodeficiency syndrome (AIDS). The eruption appeared as disseminated pustular lesions which showed necrotic features and which led to varioliform scarring. Bacterial culture from the skin, blood, and bone marrow, and ultimately from the bronchoalveolar fluid and sputum, was positive for M. avium intracellulare. The patient was successfully treated using a multiple agent anti-mycobacterial regimen including clarithromycin, which appeared to be the most effective drug. This resulted in resolution of the cutaneous and general symptoms. Our patient illustrates the wide spectrum of skin presentations that may be seen with mycobacterial infections in subjects infected with the human immunodeficiency virus (HIV). Clarithromycin is an important agent for the treatment of these severe infections.

Bacillary angiomatosis in HIV-infected patients: report of three cases with different clinical courses and identification of Rochalimaea quintana as the aetiological agent.

Three cases of cutaneous bacillary angiomatosis in HIV-infected patients are reported. They differed profoundly with respect to the extent of the lesions and the clinical course. In two cases, Rochalimaea quintana was identified by direct sequencing of the DNA amplified with the polymerase chain reaction (PCR), whereas an easy, rapid method based on the restriction length of polymorphism analysis of PCR products (PCR-RFLP) was used in the third case. This report illustrates the variations in clinical presentations and evolutive profiles in patients with bacillary angiomatosis, and confirms the causal role of R. quintana in this disease.