Severe childhood lichen planus pemphigoides after hepatitis A vaccination.

Background: Lichen planus (LP) pemphigoides (LPP) is a very rare autoimmune bullous disorder, that is, exceptional in children. Case Report: We report a case of LP pemphigoides with severe cutaneous and mucosal involvement in an 8-year-old girl who consulted for multiple vesicular and bullous lesions associated with shiny erythematous-purple plaques. The eruption occurred 2 months after vaccination against hepatitis A virus. The diagnosis of LP pemphigoides was confirmed by histopathology and immunofluorescence examination. The patient received oral corticosteroid therapy with rapid improvement. Conclusion: To our knowledge, this is the first report of LPP following hepatitis A vaccination, among adults and children.

Lymphatic spread in an immunocompetent adult.

Cutaneous tuberculosis with lymphatic spread is rarely described. We report the case of a woman aged 38, immunocompetent, working in a fruit sorting center, who consulted us for ulceration of the left third finger that had persisted for four months, following a prick from a prickly pear. Physical examination revealed a nodular, erythematous, and ulcerative lesion of the left third finger, associated with inflammatory subcutaneous nodules arranged in a line along the ipsilateral upper extremity and an ipsilateral axillary lymph node. Laboratory tests and chest X-ray were normal. Pathological examination revealed granulomatous chronic inflammation without necrosis. PCR detected DNA from Mycobacterium tuberculosis. No extracutaneous tuberculosis locations were detected. A tuberculous chancre by direct inoculation was therefore diagnosed. The patient received quadruple therapy (rifampicin + isoniazid + pyrazinamide + ethambutol) for 2 months, followed by a combination therapy based on isoniazid and rifampicin for 6 months. Marked regression of the cutaneous lesions occurred after 1 month of first-line therapy. A tuberculous chancre is a rare form of cutaneous tuberculosis, with possible lymphatic spread, in immunocompromised patients but also in immunocompetent children and young adults.

[Chronic vulvar lymphedema revealing Crohn disease in a teenage girl]. / Lymphœdème vulvaire chronique révélant une maladie de Crohn.

INTRODUCTION: Cutaneous Crohn disease is a rare cutaneous manifestation of Crohn disease in children. Herein is reported a case of persistent vulvar lymphedema revealing Crohn disease in a teenage girl. CASE REPORT: A 14-year-old girl presented with an 8-month history of persistent vulvar swelling associated with chronic macrocheilia. Dermatologic examination showed an inflammatory vulvar lymphedema, associated with perianal fissures and hypertrophic gingivitis. Vulvar skin biopsy revealed non-necrotizing granulomatous inflammation. Gastrointestinal endoscopy yielded no significant findings. The diagnosis of Crohn disease presenting as vulvar lymphedema was established. Oral metronidazole therapy resulted in partial improvement of cutaneous lesions beginning the 1st week. CONCLUSION: The originality of this case lies in the presentation of chronic macrocheilia with persistent vulvar lymphedema in a child, revealing Crohn disease without gastrointestinal involvement.

[Thrombotic skin gangrene: A rare extra-intestinal manifestation of ulcerative colitis]. / La gangrène cutanée thrombotique : une manifestation extradigestive rare de la rectocolite hémorragique.

BACKGROUND: Thrombotic cutaneous gangrene is a rare extra-intestinal manifestation of ulcerative colitis with a severe prognosis. CASE REPORT: A 35-year-old woman with a 7-year history of ulcerative colitis presented with extensive ecchymotic lesions that began a few hours earlier. On examination, she was febrile with multiple necrotic lesions. Skin biopsy showed multiple microthrombi in the dermal vessels. A diagnosis of thrombotic cutaneous gangrene was established. The patient was treated with heparin and systemic corticosteroids. The majority of cutaneous lesions showed improvement after 1 month. Thrombophlebitis of the left lower limb occurred subsequently. CONCLUSION: Thrombotic cutaneous gangrene is attributed to microvascular thrombosis, which arises from the hypercoagulability observed in ulcerative colitis. Complete blood and coagulation tests must be performed and early anticoagulation with heparin must be considered in order to prevent the progression of cutaneous infarction.

[Bowel-associated dermatosis-arthritis syndrome during ulcerative colitis: A rare extra-intestinal sign of inflammatory bowel disease]. / Syndrome arthro-cutané lors d'une rectocolite ulcéro-hémorragique : une manifestation extra-intestinale rare des maladies inflammatoires de l'intestin.

INTRODUCTION: Bowel-associated dermatosis-arthritis syndrome (BADAS) is characterized by combined pustular skin eruption and arthralgia. It may be associated with inflammatory bowel disease or bowel bypass surgery. We report a case of BADAS in a patient with ulcerative colitis. CASE REPORT: A 39-year-old woman was being treated for a severe flare-up of ulcerative colitis present over the preceding 2 months and treated with prednisone, azathioprine and cyclosporine. She was also presenting a cutaneous eruption and arthralgia that had begun three days earlier. Dermatological examination revealed profuse vesicular and pustular lesions. Biopsy specimens showed mature neutrophilic infiltrate within the dermis. A diagnosis of BADAS was made and the same treatment was maintained. Systemic symptoms were resolved but the vesicular lesions were superseded by hypertrophic scars. DISCUSSION: Bowel-associated dermatosis-arthritis syndrome consists of a vesiculopustular eruption associated with arthralgia and/or arthritis and fever, as was the case in our patient. The histological picture is characterized by abundant neutrophilic infiltrate in the superficial dermis. The clinical and histological features and the course of BADAS allow this entity to be classified within the spectrum of neutrophilic dermatoses. Treatment chiefly involves systemic corticosteroids.

[Neutrophilic dermatosis of the hands: acral sweet syndrome?]. / Dermatose neutrophilique des mains : un syndrome de Sweet acral ?

INTRODUCTION: Neutrophilic dermatosis may have various clinical presentations but share common histopathological manifestations with an aseptic infiltrate of polymorphonuclears neutrophils. Neutrophilic dermatosis of the hands is a recently described disorder of which we present a case. CASE REPORT: A 60-year-old woman, without significant past medical history, presented with an acute and painful eruption of both hands with fever. Clinical examination showed erythematous edematous lesions surmounted by pustules that covered the thenar eminences of both hands. The patient was also presenting inflammatory joint pain. Histological findings were predominantly neutrophilic infiltration in the dermis with leukocytoclastic debris. A diagnosis of neutrophilic dermatosis was made and all the lesions disappeared rapidly without relapse under oral prednisone (0.5mg/kg/j). CONCLUSION: The eruption observed in our patient was clinically and histologically suggestive of neutrophilic dermatosis of the hands, confirming the existence of a homogenous entity which is still debatable if it constitutes a separate entity or a localized variant of Sweet syndrome.

Juvenile hyaline fibromatosis: a case report.

Juvenile hyaline fibromatosis is a rare, hereditary disease with distinct clinical and histopathological features. Clinically, it presents with gingival hypertrophy, pappulonodular skin lesions and joint contractures. Bone involvement is usually an uncommon finding. We report a case of a 2-year-old patient, daughter of consanguineous parents, who presented since the age of 2 months with impairment of mental development, multiple joint contractures, motion limitation and nodules on the scalp. The calvarian lesions were surgically removed, and histopathological examination concluded to juvenile hyaline fibromatosis.

Metastasizing pleomorphic adenoma of the submandibular gland: a case report.

Pleomorphic adenoma (PA), originally called mixed tumour, is the most common neoplasm of the salivary glands. It is usually a benign, slow-growing and well-circumscribed tumour. However, PA may occasionally give rise to metastases that usually occur after a previous recurrence. These tumours display benign histological features in both primary tumours and metastases. Such tumours have been termed metastatic PA or metastatic mixed tumours. We report a case of metastatic PA of the submandibular gland with metastasis to the cervical lymph nodes.

Growing Teratoma Syndrome After Treatment of a Nonseminomatous Germ Cell Tumor: A Case Report and a Review of Literature.

Growing teratoma syndrome is a rare condition among patients with nonseminomatous germ cell tumors who present with enlarging metastatic masses during appropriate systemic chemotherapy and normalized serum markers. Retroperitoneal residual masses are a common finding after chemotherapy for the nonseminomatous tumors of the testis. These might contain mature teratoma, fibrotic tissue, or tumor. Mature teratoma, which is unresponsive to chemotherapy, might result from evolution of a malignant lesion during treatment or it might represent a metastasis from a focus of mature teratoma in the primary testicular tumor. This article reviews a case of a growing teratoma syndrome.

Eosinophilic cystitis with eosinophilic cholecystitis: a rare association.

We describe a rare case of eosinophilic cystitis associated with eosinophilic cholecystitis in a 30-year-old patient who underwent bladder biopsy for irritative voiding symptoms and routine elective cholecystectomy for gallstones. Diagnosis was confirmed by histopathological examination. The rarity of this condition prompted us to report this entity in which no specific cause could be found.

[Tongue base schwannoma in a child]. / Schwannome de la base de la langue chez l'enfant.

INTRODUCTION: A schwannoma is a benign slow growing tumor originating from the peripheral nerve sheath. Its tongue base location is extremely rare, especially in very young patients. CASE REPORT: A 15-year-old female patient was admitted in emergency for moderate hematemesis. The clinical examination revealed swelling of the tongue base, causing the bleeding. This lesion had appeared when she was 3years of age and no treatment was done. A complete surgical excision was performed and the histopathological examination allowed diagnosing a tongue base schwannoma. The postoperative outcome was uneventful, and no recurrence was detected 6months after surgery. DISCUSSION: The particularities of this report are the patient's very young age and the tumor's tongue base location. The prognosis is usually good after complete excision, if there is only one tumor. Type 2 neurofibromatosis should be excluded, given the risk of malignant degeneration.