Linear Darier disease with herpes zoster superinfection treated successfully by brivudine.

We report the case of a human immunodeficiency virus-positive patient presenting linear Darier disease with varicella-zoster virus superinfection following the lines of Blaschko. The lesions healed after treatment with brivudine.

Acute pustulosis of the legs in diverticulitis with sigmoid stenosis: an overlap between bowel-associated dermatosis-arthritis syndrome and pustular pyoderma gangrenosum.

BACKGROUND: Bowel-associated dermatosis-arthritis syndrome denotes the occurrence of diarrhoea with arthritis and skin lesions related to bowel disease with or without bowel bypass. In this condition, the histological finding of cutaneous aseptic neutrophilic cell infiltrate is non-specific and common to a wide spectrum of neutrophilic dermatoses, including pyoderma gangrenosum. OBSERVATION: We describe a 78-year-old woman with fever, abdominal discomfort and arthralgias, who developed grouped pustular lesions on her shins with histologically spongiform pustule formation. Aetiological assessment disclosed diverticular disease with sigmoid stenosis. CONCLUSION: Although clinical and histological features in our case fit the diagnosis of bowel-associated dermatosis-arthritis syndrome, they may also correspond to a pustular variant of pyoderma gangrenosum. Our observation raises the question of the nosological classification of bowel-associated dermatosis-arthritis syndrome within the spectrum of neutrophilic diseases.

Erosive pustular dermatosis of the leg: report of three cases.

Erosive pustular dermatosis of the leg is a distinct form of spongiform amicrobial pustulosis. The disorder typically affects the lower limbs of elderly patients presenting with chronic venous insufficiency and stasis dermatitis, and has a chronic course. Three elderly patients with chronic venous ulcers are described, who developed pustules and moist eroded lesions on the leg. The clinical and histological features were typical for erosive pustular dermatosis. The lesions rapidly responded to topical treatment with either tacrolimus or corticosteroids. Of note, this condition was associated with a diverticular disease in two patients, while in another patient an epidermoid carcinoma of the tongue was present. Erosive pustular dermatosis of the leg is an uncommon but distinct skin disorder typically associated with trophic changes of the lower limbs. Our observations raise the question of the relation of erosive pustular dermatosis of the leg with the group of neutrophilic dermatoses. Topical immunotherapy with tacrolimus may constitute a novel therapeutic option for this frequently recalcitrant condition.

Chronic borreliosis presenting with morphea- and lichen sclerosus et atrophicus-like cutaneous lesions. a case report.

We report on a case of chronic cutaneous borreliosis with manifestations clinically compatible with morphea and lichen sclerosus et atrophicus. The histopathologic features of these lesions were those of acrodermatitis chronica atrophicans. Our case illustrates the concept that clinical aspects of morphea and lichen sclerosus et atrophicus pertain to the spectrum of cutaneous borreliosis.

Alopecia syphilitica: report of two cases in Geneva.

Two patients with alopecia syphilitica are presented. Moth-eaten alopecia is the most typical look of secondary syphilis on the scalp. With the decrease in syphilis cases in western countries, this rarer clinical manifestation tends to be misdiagnosed.

Cutaneous pseudolymphoma, lymphomatoid contact dermatitis type, as an unusual cause of symmetrical upper eyelid nodules.

We describe a 74-year-old woman who rapidly developed bilateral voluminous nodules on the upper eyelids, following 4 months' treatment for chalazion. Histological and immunohistochemical findings were suggestive of cutaneous pseudolymphoma (CPL), and extensive screening for malignant lymphoma (ML) remained negative. Cutaneous pseudolymphomas are inflammatory diseases that can simulate ML either clinically, histopathologically, or both. They are a rare cause of nodules of the upper eyelids, usually characterized by a benign evolution, that can be secondary to ultraviolet sensitivity, adverse reactions to systemic medications and contact sensitization, among others. In our case, epicutaneous patch tests were strongly positive for a series of allergens contained in the ophthalmological preparations used; therefore, they were suggestive that the patient had CPL, lymphomatoid contact dermatitis type. In addition, the patient had been treated previously with several drugs, known to cause CPL and immune dysregulation. Complete regression of the lesions required treatment with systemic steroids and chlorambucil. No relapse occurred within a 5-year follow-up period.

Takayasu arteritis and cutaneous necrotizing vasculitis.

Takayasu arteritis (TA) is an inflammatory arteriopathy involving predominantly the aorta and its main branches. The disease evolves in two phases: a first, nonspecific inflammatory stage and a late 'pulseless' stage, in which complications related to arterial stenosis and aneurysm formation predominate. In both phases, skin manifestations, such as inflammatory nodules, erythema-nodosum- and pyoderma-gangrenosum-like ulcers, have been described. We report 2 patients with TA, who had cutaneous necrotizing vasculitis as presenting manifestation of the disease. A review of the literature revealed 8 similar cases. TA does not only involve large arteries, but also small blood vessels. The observation that in TA the inflammatory process of the large arteries affects regions of the walls supplied by the vasa vasorum, the anatomy of which bears resemblance to the cutaneous vessel system, suggests that primary involvement of small vessels contributes to the development of the clinicopathological features of TA. Knowledge of the skin manifestations associated with TA remains important for its diagnosis and prompt instauration of life-saving treatment.

Adult-onset Still's disease with persistent plaques.

Adult-onset Still's disease (AOSD) is a systemic disorder characterized by intermittent fever, evanescent rash, arthralgias or arthritis and predominantly neutrophilic leucocytosis. We report on a 16-year-old woman with Still's disease who developed, in addition to the typical rash, persistent papular lesions on her face, neck and upper and lower back. Although the presence of fixed skin lesions is not a characteristic feature of AOSD, their appearance at the onset of the disease and their evolution suggest that they represent a specific manifestation of the disease.

Eccrine syringofibroadenoma in a patient with erosive palmoplantar lichen planus.

We report the case of an 82-year-old woman with a 6-year history of erosive palmoplantar lichen planus associated with eccrine syringofibroadenoma (ESFA). Examination revealed a well-demarcated patche writish of reticulated whitish papular lesions in skin of otherwise normal appearance at the border of erosive lichen planus plaques. These lesions had the histological appearance of ESFA. We suggest that these lesions are induced by the inflammatory remodelling associated with erosive lichen planus and propose to consider ESFA in the context of skin tissue remodelling as a new subtype of ESFA.

Ecthyma gangrenosum-like lesions: a sign of disseminated Fusarium infection in the neutropenic patient.

Fusarium is a saprophytic fungus of soil causing disease in plants and animals. In the immunocompetent patient, Fusarium is non-invasive, colonizing wounds, ulcers or nails. In the immunocompromised host, however, especially in those whose neutrophil and macrophage function is deficient, it can cause devastating systemic infections. Skin lesions are an early feature of the disseminated disease. Rapid diagnosis and treatment are mandatory in order to give the patient a better chance of survival, reported mortality rates being as high as 90%.

[Metastatic aspergillus panniculitis in blast transformation of a myelodysplastic syndrome and agranulocytosis]. / Metastatische Aspergilluspannikulitis bei blastischer Transformation eines myelodysplastischen Syndromes und Agranulozytose.

We present a 58-year-old patient with disseminated aspergillosis and metastatic panniculitis. He suffered from agranulocytosis and blastic transformation of a myelodysplastic syndrome. The skin lesion showed a single subcutaneous inflammatory nodule, which was clearly shown by histolog to be of embolic origin. This clinical presentation has not previously been described. Disseminated aspergillosis is found mainly in immunocompromised and neutropenic patients and the mortality is high. The present case demonstrates how histological examination together with skin biopsy culture can allow the diagnosis.

L-tryptophan-induced eosinophilia-myalgia syndrome associated with primary cutaneous malignant fibrous histiocytoma and extraabdominal desmoid tumor.

A 57-year-old woman with L-tryptophan-induced eosinophilia-myalgia syndrome, 23 months after the onset of symptoms, experienced development of a parasternal malignant fibrous histiocytoma on previously scleroderma-like skin areas and, almost concurrently, an extraabdominal desmoid tumor of the left arm muscle fascia. The malignant fibrous histiocytoma was treated by surgical excision without sign of recurrence or metastasis 19 months later. Radiation therapy was performed on the extraabdominal desmoid tumor. We suspect that these two connective tissue tumors in this patient were related to the exposure to contaminated L-tryptophan, which interfered with connective tissue metabolism.

Takayasu arteritis preceded by cardiac and cutaneous lesions. A case report.

A diagnosis of aortic insufficiency and mitral stenosis was made in a 24-year-old woman after an episode of heart failure following delivery. A double valvular replacement was performed 4 years later. At that time she suffered from recurrent episodes of erythema nodosum like lesions with an histological diagnosis of cutaneous polyarteritis nodosa. After another 4-year interval she presented with severe arterial hypertension and a biological inflammatory syndrome. An arteriography disclosed stenoses of the abdominal aorta, renal, and iliac arteries as well as occlusion of the right subclavian artery, diagnostic of Takayasu arteritis. We postulate that this woman presented a rare combination of cardiac and skin involvement of Takayasu's disease which preceded the classical arteritis of the aortic branches by 8 and 4 years, respectively.

Orbital and palpebral paraffinoma.

Paraffinoma is a well-recognized complication of paraffin injection. We describe a 44-year-old man who had an ethmoidectomy for chronic sinusitis. A communicating fracture of the ethmoid bone into the orbit occurred intraoperatively. The nasal cavity was subsequently packed with gauze containing a petrolatum-based antibiotic ointment. Bilateral, periocular swelling developed 1 week later. Optical and electron microscopic studies revealed a paraffinoma.