Widespread livedoid vasculopathy

A 37-year-old woman with a 13-year history of widespread livedo reticularis and recurrent, painful ulcerative skin lesions was referred to our department because of a relapse of cutaneous manifestations of the skin lesions involving almost the whole body surface; malar erythema and oedema, non-scarring alopecia and fever were also associated. Routine laboratory data, immunological investigations and coagulation parameters were normal or negative. Histology was consistent with livedoid vasculopathy. A good clinical response was obtained using intravenous methylprednisolone combined with pentoxifylline. Livedoid vasculopathy is a rare, distinctive dermatosis that can be associated with systemic autoimmune disorders or present in an "idiopathic" form. The latter is at present regarded as a non-inflammatory thrombotic disease that may occur in patients with coagulation abnormalities. It is noteworthy that, in the present case, despite long-standing and dramatic cutaneous features, serious systemic complications have not developed and the patient's seroimmunologic and coagulative profile has remained normal.     

Histologically specific skin lesions in disseminated cytomegalovirus infection

A diabetic renal transplant recipient developed fever, generalized rash, and genital ulcers which showed typical histologic changes of cytomegalovirus (CMV) infection in the dermal vascular endothelium. This infection was confirmed by rising serologic titer of specific antibody, positive viral cultures, and typical nuclear inclusion bodies in pulmonary and hepatic tissue at autopsy. Biopsy of the patient's skin lesions provided the opportunity for early diagnosis of this fatal systemic CMV infection. Two clinical patterns of specific cutaneous involvement in disseminated CMV infection are discussed.     

Malignant syphilis in patients with human immunodeficiency virus (HIV)

Malignant or noduloulcerative syphilis is an infrequent variant of secondary syphilis which appears in HIV+ patients. It is associated with fever, general malaise and skin lesions in the form of pustules or ulcerative scabs. Histology studies on the lesions show a perivascular infiltration of plasma cells and a formation of granuloma of giant and epitheloid cells. Most patients test positive for syphilis. We discuss the case of a 37-yar-old man who attended our service complaining of fever and scabby nodular lesions all over his skin. Blood testing confirmed that he had syphilis and HIV. The patient responded rapidly to penicillin treatment, with remittance of his fever. The skin lesions disappeared after 8 weeks. The rareness of malignant syphilis and its unusual clinical manifestation is a challenge to medical personnel. This diagnosis ought to be considered in HIV+ patients with fever and ulcerative skin lesions. Penicillin is the treatment of choice.     

Dermatitis artefacta: unusual appearance in an older woman

Dermatitis artefacta is a rare and difficult condition for diagnosis and treatment, with the highest incidence of onset in late adolescence to early adult life. Most patients are young women who have a personality disorder; borderline features are common and the patient's denial of psychological distress makes management and treatment difficult. Patients use a variety of means to cause the skin changes. Clinical presentation of the skin lesions does not conform to those of known dermatoses and are located on easily reached parts of the skin. We report an unusual case of a 72-year-old woman with symmetrical changes under the breasts and in the right inguinal region. The lesions were composed partly of haemorrhagic round lesions and partly of scars. A skin biopsy was taken and consultations with the psychiatrist, internist and the patient's family led to the diagnosis of self-induced dermatitis. The skin lesions were covered by occlusion techniques and the lesions improved very rapidly. The patient was discharged from the hospital under psychiatric and family care.     

Cutaneous aseptic abscesses, manifestations of neutrophilic diseases]

BACKGROUND: Neutrophilic skin disease includes several entities: Sweet syndrome, pyoderma gangrenosum, erythema elevatum diutium, Sneddon-Wilkinson sub-keratous pustulosis, and neutrophilic eccrine hidradenitis. We report two cases of aseptic abscesses which correspond to the deepest anatomoclinical form of neutrophilic dermatosis. CASE REPORTS: A 28-year-old man was hospitalized for fever and abdominal pain with bloody diarrhea in relation with Crohn's disease. The patient also presented two skin abscesses on the lower limbs. Bacteriology specimens were negative. The histology specimen of a skin lesion revealed neutrophil infiltration of the hypodermis without granulomatosis. Systemic corticosteroid therapy was given and rapidly led to resolution of the inflammatory bowel disease and the skin lesions. The patient developed inflammatory spondylarthropathy several months later. The second patient was a 36-year-old woman with a history of splenomegaly with asceptic abscesses. She was admitted for abdominal pain with non-bloody diarrhea, fever and multiple joint pain related to spondylarthropathy. She developed several simultaneous abscessed nodules on the legs. Biopsy revealed neutrophil infiltration of the hypodermis. The diagnosis of neutrophilic disease with aseptic cutaneous and visceral abscesses was retained. Nonsteroidal antiinflammatory drugs and dapsone were given leading to regression of the skin lesions and the abdominal and joint pain. DISCUSSION: Aseptic skin abscesses result from a deep localization of neutrophilic disease. They suggest the presence of inflammatory bowel disease, spondylarthropathy or other aseptic visceral localizations.     

Cutaneous cryptococcosis resembling molluscum contagiosum in a homosexual man with AIDS. Report of a case and review of the literature.

A 43-year-old homosexual man with the Acquired Immunodeficiency Syndrome (AIDS) developed cutaneous molluscum contagiosum-like lesions on face, ears, neck, hands and feet. He was admitted to our unit with fever, malaise and headache. Cytologic examination of skin brushing revealed numerous encapsulated budding yeasts, identified as Cryptococcus neoformans. Such a finding calls for a cytologic examination of skin lesions in patient with AIDS who present with fever and headache, in order to rule out a potentially life-threatening fungal infection.     

Drug rash with eosinophilia and systemic symptoms induced by valproate and carbamazepine: formation of circulating auto-antibody against 190-kDa antigen

Drug rash with eosinophilia and systemic symptoms (DRESS) is characterized by fever, rash and internal organ involvement after exposure to certain drugs. Most of the aromatic anticonvulsants, such as phenytoin, phenobarbital, and carbamazepine, can induce DRESS. Cross-sensitivity between not only the aromatic anticonvulsants, but also the aromatic and non-aromatic anticonvulsants, is possible. We report here a case of a Korean woman who had previously tolerated valproate, but developed DRESS due to valproate after carbamazepine intake, which indicates cross-sensitivity. Her skin lesions displayed diffuse oedematous patches on the entire body associated with tense bullae on her arms and legs. Circulating auto-antibody to 190-kDa antigen was detected in the patient's serum by indirect immunofluorescence and immunoblotting, which might contribute to a pathogenic role in DRESS.     

Exacerbation of skin lesions during fever in a patient with chronic infantile neurologic cutaneous articular (CINCA) syndrome

Chronic infantile neurologic cutaneous articular (CINCA) syndrome is a serious chronic systemic inflammatory disease that presents at a young age and that is characterized by skin, joint, and central nervous system disease. Skin symptoms are the first to appear, in the form of a longstanding nonpruritic urticarial rash, with exacerbations coinciding with episodes of fever, arthritis, and enlarged lymph nodes. The findings of biopsy of skin lesions are extremely variable but characterized by perivascular neutrophilic infiltrate. With the discovery of mutations in the CIAS1 gene, which encodes a protein known as cryopyrin, this entity has been classified as one of the cryopyrin-associated autoinflammatory diseases, along with familial cold urticaria and Muckle-Wells syndrome. This discovery has also made available new therapeutic options. We present the case of a boy diagnosed with CINCA syndrome who presented with an outbreak of painful skin lesions and fever. These lesions were thought to be an exacerbation of underlying lesions during an episode of fever.     

艾滋病并发皮肤播散性马尔尼菲青霉病

报告1例艾滋病并发皮肤播散性马尔尼菲青霉病.患者女,34岁.因间歇性发热、贫血4个月.面部及躯干丘疹、斑块1个月就诊.皮损表现为面部、躯干以及四肢广泛分布的疣状增殖性丘疹、斑块,上覆蛎壳样痂屑.皮损真菌培养及组织病理学检查确诊为马尔尼菲青霉病.经多次HIV抗体和抗原检测,最终确诊为艾滋病.予抗真菌及抗病毒治疗,患者皮损消退,一般情况迅速好转.     

Association of hyperimmunoglobulinaemia D syndrome with erythema eievatum diutinum

Hyperimmunoglobulinaemia D and periodic fever syndrome was observed in a female patient with erythema eievatum diutinum. The association of this skin disease with hyperimmunoglohnlinaemia D may indicate a pathogenetic relationship. The skin lesions responded to dapsone therapy.     

Porokeratosis with large skin lesions. Histologic, cytologic and cytogenetic study of three cases.

Three porokeratosis patients with large skin lesion(s) are reported. The histopathology of the large lesions revealed that the epidermis 1) frequently presented slight or marked acanthosis and/or elongation of the rete ridge, and 2) contained abnormal cells, e.g. hyperchromatic, large, multinucleated, and/or irregular shaped nuclei. The DAPI-DNA microfluorometric study revealed DNA polyploidy and/or an increased population of epidermal cells with hyperdiploid and/or tetraploid DNA content. These results indicate the proliferating potential of the epidermis and the existence of a neoplastic clone or clones therein. This finding may explain the enlargement of skin lesions and possibly the development of malignancy, as sometimes occurs in large skin lesions. Furthermore, cultured skin fibroblasts from a patient's skin lesion or its surrounding skin revealed various kinds of chromosomal structural abnormalities, which may serve as a basis for the development of abnormal neoplastic clones in the porokeratotic epidermis.     

艾滋病合并播散性马内菲青霉病1例

报告1例艾滋病合并播散性马内菲青霉病。患者女,31岁。因发热、咳嗽、乏力伴消瘦2个月入院。入院前半个月患者面部、躯干、上肢出现中央有凹陷或坏死的传染性软疣样皮损。入院后查人免疫缺陷病毒（HIV）抗体阳性。丹髓涂片、组织病理检查见细胞内外有大量酵母样细胞,部分中央有横隔（裂殖）;骨髓、皮损及淋巴结真菌培养均阳性（2℃和37℃双相培养）,经鉴定为马内菲青霉,25℃条件下培养为菌丝相,且有红色色素养产生,37℃条件下培养为酵母相。诊断：艾滋病合并播散性马内菲青霉病。确认后予以静脉滴注伊曲康唑,2周后发热消退,皮损缓解。     

An unusual case of coccidioidomycosis presenting with skin lesions

A 42-year-old white male without prior skin disease presented 8 months ago with chest pain, fever, and coughing to his primary physician. At that point, he had no skin lesions. The diagnosis was made by standard blood tests. The patient completed treatment and was symptom free for 8 months until he presented to his physician again with skin lesions on his right cheek and extremities. The patient was referred to our dermatology clinic for further evaluation. A complete skin examination revealed lesions on his face, extremities, and back. The lesions on his right face were 8 x 10-mm erythematous papules and nodules coalescing into a plaque. A biopsy was taken from his right cheek lesions.     

Pemphigus vegetans in a patient with lung cancer

A 66-year-old woman with a history of lung cancer treated with radiotherapy 2-years prior to admission, was seen for mucosal and skin lesions of 3-month's duration. She had pustules involving intertriginous areas and erosions involving the oral mucosa. Histopathology of skin lesions and the results of direct and indirect immunofluorescence studies were consistent with the Hallopeau-type pemphigus vegetans. Additionally, circulating antibodies against skeletal muscles were detected in patient's serum. The patient was treated with immunosuppressive therapy and had an almost complete remission of skin and mucosal lesions within 1 month; however, the patient developed pneumonia with pyothorax, apparently related to recurrence of lung cancer.     

A case of febrile ulceronecrotic Mucha-Habermann disease requiring debridement of necrotic skin and epidermal autograft

We report a case of febrile ulceronecrotic Mucha-Habermann disease (FUMHD) in a 21-year-old man. This disease is a severe form of pityriasis lichenoides et varioliformis acuta (PLEVA) and is characterized by the sudden onset of diffuse ulcerations associated with high fever and systemic symptoms. It is sometimes lethal especially in elderly patients. In the present case, intense generalized maculopapular erythematous plaques with central necrosis developed progressively in association with a high fever. Initial treatment with systemic betamethasone had been unsuccessful and the skin lesions, which covered about 50% of the body surface, became severely ulcerated. Although the development of new lesions had ceased spontaneously, widespread ulceration of the skin remained. Debridement of the necrotic skin and skin grafting using cultured epidermal autografts and meshed allografts of cadaver skin led to prompt reepithelization.     

Cutaneous cryptococcosis resembling molluscum contagiosum: a first manifestation of AIDS

A 30-year-old homosexual man developed multiple skin umbilicated lesions resembling molluscum contagiosum. Initially the lesions were on his face but they rapidly spread. Histopathology and mycologic cultures of a skin biopsy revealed cryptococcus neoformans which was also identified in cerebrospinal fluid and in bronchoalveolar washings. The patient had fever, weight loss, generalized lymph node enlargement, depletion of the T helper subpopulation and positive HIV-1 serology. During treatment with flucytosine and amphotericin B, the skin lesions regressed in 3 months (cryptococcus neoformans disappeared in the cerebrospinal fluid and skin within one and five weeks, respectively). Our case demonstrates that molluscum contagiosum-like skin manifestations may be caused by cryptococcal infections. So it is necessary to perform skin biopsy in HIV seropositive patients with skin lesions resembling molluscum contagiosum, to diagnose mycotic infections, and especially cryptococcosis. Cutaneous cryptococcosis was, in this case, the first symptom of AIDS.     

Marking patch tests sites: description of a practical, clean, durable and inexpensive method

We propose an original method to mark patch test sites. A transparency is applied on the patient's back, surrounding patch tests, immediately after their application. Marks are performed with an erasable pen on test sites and on permanent skin lesions like naevi. For further readings performed after patch test removal, the transparency applied on the patient's back permits the attribution of a positive reaction. This method is clean (without ink marks on skin and without contamination of clothing), more comfortable for the patient (allowing back washing immediately after the 1st reading, never inducing allergy to inks), durable (permitting delayed readings after several days or weeks) and inexpensive (because transparencies can be reused).     

Oral mucosa lesions as atypical manifestation of adult-onset Still’s disease

Adult-onset Still’s disease is a systemic inflammatory disorder of unknown etiology, characterized by skin rash, spiking fever, arthralgias or arthritis, and leukocytosis. The typical skin rash is evanescent, salmon-pink, nonpruritic and maculopapular, predominantly on the extremities. It is considered one of the major Yamaguchi’s criteria in adult-onset Still’s disease. However, atypical skin lesions are also described. Here, a 61-year-old woman with sore throat, spiking fever, polyarthritis and evanescent salmon-pink nonpruritic maculopapular skin rash on the extremities was diagnosed with adult-onset Still’s disease. In addition, atypical brown macules on oral mucosa, localized on the inner lips and tongue were also observed. Biopsy revealed a neutrophilic infiltrate. Despite treatment and improvement of the adult-onset Still’s disease, the atypical oral mucosal lesions persisted.     

Reacción granulomatosa a cuerpo extraño a sílice,silicona y ácido hialurónico,en paciente con sarcoidosis inducida por interferón

We report the case of a patient who developed sarcoid granulomas 11 months after starting treatment with pegylated interferon alfa and ribavirin for chronic hepatitis C. The sites of the lesions were related to 3 different foreign bodies: silica in old scars on the skin, hyaluronic acid that had been injected into facial tissues, and silicone in an axillary lymph node draining the area of a breast implant. Systemic sarcoidosis was diagnosed on the basis of a history of dry cough and fever and blood tests that revealed elevated angiotensin converting enzyme and liver enzymes. Interruption of the antiviral therapy led to normalization of liver function tests and disappearance of the skin lesions and lymphadenopathies. Dermatologists and cosmetic surgeons should be aware of the risk of sarcoid lesions related to cosmetic implants in patients who may require treatment with interferon in the future.     

Malignant histiocytosis with panniculitis--a case report

We report a case of malignant histiocytosis which began with the skin lesions of panniculitis. A 32-year-old woman presented with recurrent erythematous plaques, subcutaneous nodules, and ulcers on the trunk and the extremities and intermittent fever for 7 months. The cutaneous lesions consisted of erythematous and brownish irregular-shaped patches and tender cutaneous nodules 0.5-1.0 cm in diameter. Central necrosis and shallow ulcers were seen in the lesions. The patient also suffered from general fatigue, arthralgia, and weight loss. She was anemic and thrombocytopenic and had progressive impairment of liver function with coagulation defect. Histopathological study of skin lesions showed lobular panniculitis without vasculitis in the subcutaneous fat tissue. In the panniculitis lesion, moderate mixed cell infiltration consisting of lymphocytes and histiocytes was observed. Bone marrow aspiration revealed an increase in the number of histiocytes, mostly immature with active phagocytosis of erythroid cells, myeloid cells, and platelets. She was diagnosed as having malignant histiocytosis and treated with cyclophosphamide, vincristine, and prednisolone which she responded well; her fever subsided and the lesions healed with hyperpigmentation. In this patient, benign histiocytes with hemophagocytosis without immature forms were found in the skin lesions. According to our knowledge, this is the first Thai report of malignant histiocytosis with clinical features of panniculitis.