[Amicrobial pustulosis of the skin folds and autoimmune erythroblastopenia]. / Pustulose aseptique des plis et érythroblastopénie auto-immune.

INTRODUCTION: Amicrobial pustulosis of the skin folds represents a new entity within the spectrum of neutrophilic dermatoses. This disease is characterized by acute onset of pustular lesions in the skin folds, association with an autoimmune disorder, and improvement under systemic corticosteroids. OBSERVATION: A 24-year-old woman had been presenting pustular dermatosis for several months involving the inguinal folds and the scalp. The pustules coalesced to form oozing and crusting plaques. Eczematous lesions were located on the trunk. She also presented macrocytic anemia related to autoimmune erythroblastopenia. Bacteriological culture was negative. Antinuclear antibodies were found with anti-SSA specificity. Histopathological examination of a skin biopsy specimen showed pustules in the epidermis together with an inflammatory dermal infiltrate. Cutaneous direct immunofluorescence testing was negative. The patient responded to systemic corticosteroids. DISCUSSION: Thirty-six cases of amicrobial pustulosis of the skin folds have been reported in the literature. All but two previously reported patients were females with an autoimmune disorder (chiefly systemic lupus erythematosus). The clinical picture is characterized by aseptic pustular lesions of the major and minor skin folds of the scalp and the anogenital area associated with eczematous lesions. Diagnostic criteria have recently been proposed. This disease responds to systemic corticosteroids. We report a new case of amicrobial pustulosis of the skin folds associated with autoimmune erythroblastopenia, which to the best of our knowledge has been described only once in the literature.

[Cutaneous, mucosal and systemic pyoderma gangrenosum]. / Pyoderma gangrenosum cutanéomuqueux et systémique.

BACKGROUND: Extracutaneous and mucosal involvement is rare in neutrophilic dermatoses. We report a case of cutaneous-mucosal and systemic pyoderma gangrenosum (PG) revealing myelodysplasic syndrome. CASE REPORT: A 56-year-old man was hospitalised for an inflammatory abdominal plaque with an ulcerated centre that appeared rapidly in a setting of fever of 40 degrees C, odynophagia, weight loss and arthritis of the ankle. Despite surgical abdominal repair and combined dual antibiotics, the patient remained febrile and the ulcer size continued to increase. The edges were raised, purple and necrotic. Examination revealed vegetative legions with a purple edge on the lower lip and tongue. Histological examination of the skin and mucosal biopsy samples was consistent with a diagnosis of PG. A chest-abdomen CT scan showed mesenteric panniculitis and interstitial lung disease. The bone marrow sample revealed an appearance of chronic myelomonocytic leukaemia with myelofibrosis and excessively high blast levels. Systemic corticosteroids rapidly resulted in apyrexia with healing of the cutaneous, mucosal and visceral lesions. The patient presented secondary worsening of his acute myeloid leukaemia type-IV requiring bone marrow rescue. DISCUSSION: Extracutaneous involvement in neutrophilic dermatoses is rare but takes a number of different forms. We report a case of a patient presenting multifocal PG with cutaneous, mucosal, joint, mesenteric and pulmonary involvement. Oral lesions seen in PG generally affect the palate and they are commonly associated with inflammatory intestinal diseases. Our case is original in terms of the inaugural character of the mucosal lesions, their burgeoning appearance and their location on the lips and tongue.

[Basan's syndrome: Congenital absence of dermatoglyphs and milia]. / Absence de dermatoglyphes et grains de milium congénitaux: un nouveau cas de syndrome de Basan.

BACKGROUND: White micropapules are uncommon on the face newborns and are mainly due to sebaceous hyperplasia, or more rarely to milia. In some cases they may result from milia and profuse milia suggest rare diseases. CASE REPORT: We report the case of a newborn presenting with profuse congenital milia on the face that resolved spontaneously within a few months. DISCUSSION: This eruption revealed Basan's syndrome, a rare, autosomal dominant inherited dermatosis described as the association of profuse facial milia, acral bullae, absence of dermatoglyphs, and palmoplantar hypohydrosis.

[Pyodermatitis-pyostomatitis vegetans with nasal involvement]. / Pyostomatite-pyodermatite végétante avec atteinte nasale.

BACKGROUND: Pyodermatitis-pyostomatitis vegetans (PPV) is a rare chronic disorder often associated with inflammatory bowel disease. We report an isolated case involving the oral, labial and nasal mucosa. PATIENTS AND METHODS: A 28-year-old man, in good general condition, presented with a 2-year history of painless stomatitis. The physical examination revealed pustular and exophytic lesions of the jugal, gingival and palatine mucosa on an erythematous background, as well as some pustular and crusted lesions of the lower lip and nostrils. Histopathological analysis revealed epithelial hyperplasia and a suprabasal cleft with some signs of acantholysis and numerous neutrophils and eosinophils. Direct and indirect immunofluorescence assay was negative. There was no associated bowel disease. We concluded on a diagnosis of PPV of younger subjects. The lesions disappeared with oral corticosteroids but with steroid dependency. DISCUSSION: PPV is a rare dermatosis associated in more than 75% of cases with inflammatory bowel disease, usually ulcerative colitis. Lesions of the oral mucosa are a constant finding and are characterised by aseptic pustules on an erythematous background. Skin lesions are pustular and more or less exophytic. To our knowledge, there have been no reports to date of intranasal lesions of PPV.

[Sweet's syndrome as the presenting manifestation of relapsing polychondritis]. / Syndrome de Sweet révélant une polychondrite atrophiante.

Relapsing polychondritis (RP) is a rare disorder characterized by recurrent inflammatory episodes involving various cartilages. The clinical course of RP is variable, and dermatologic manifestations are uncommon. We report a 73-year-old patient who presented with a neutrophilic dermatosis (Sweet's syndrome) as the initial manifestation of RP. There was no evidence for a myelodysplastic syndrome, as it has been previously reported with RP, but the patient was followed-up for an indolent and untreated chronic lymphocytic leukaemia. Complete remission was obtained with oral corticosteroids. This report highlights the clinical spectrum of the RP.