Clinical experience with the use of cyclosporin A in psoriasis. Results of a retrospective study.

The efficacy of cyclosporin A (Sandimmun Neoral) in severe psoriasis was analysed in 70 patients included in a retrospective descriptive study conducted at our Dermatology Department from January 1994 until December 2000. After 8 weeks of treatment, with an initial dose of 2.5-3 mg/kg/day, the reduction in the Psoriasis Area and Severity Index (PASI) score was>75% in 61% of patients. Most of the side effects observed turned out to be susceptible to being reversed over time or by dose reduction. The results of this study show that continuous and low-dose cyclosporin therapy is a safe and efficacious treatment for severe chronic psoriasis.

Concurrent localized scleroderma and discoid lupus erythematosus. Cutaneous 'mixed' or 'overlap' syndrome.

Four patients with concurrent, chronic, progessive, localized scleroderma and discoid lupus erythematosus were studied; the condition originated as linear scleroderma in three of them. Three of the four patients were young females at the onset of the first skin disease. Dermatopathologic study confirmed the scleroderma and lupus erythematosus (LE). Direct immunofluorescence showed a positive band test in three cases. Unusual serological results included a positive LE clot test in three cases, a positive extractable nuclear antigen test in one case, and a negative antinuclear antibody test on repeated occasions in all four cases. Rare cutaneous disease similar to systemic, "mixed," or "overlap" connective tissue disease exists and offers an opportunity to study unusual immunologic and pathological events in both scleroderma and LE.

Histologic, ultrastructural and histochemical studies of granuloma annulare.

We reviewed and reclassified the histology in 207 cases of granuloma annulare from our files. The most common pattern found was mononuclear infiltrative type (72%) in contrast to the palisading group (25%) and the epithelioid nodules (3%). Indeed, and entire spectrum from mononuclear infiltrates to the epithelioid sarcoidal types is seen. The electron microscopic findings include histiocytes with membrane activity, well-developed rough endoplasmic reticulum, large mitochondria, and numerous lysosomes. Pathcy damage of collagen and elastic fibers with the presence of vesicles, lysosomes, and cytoplasmic debris are demonstrated. The histochemical and ultrastructural studies localized acid phosphatase in the primary lysosomes and the routh endoplasmic membrane. The presence of fibrin deposition confirmed our previous immunofluorescence results.

[Parry-Romberg syndrome associated with homolateral segmental vitiligo]. / Syndrome de Parry-Romberg associé à un vitiligo segmentaire homolatéral.

We report the case of a 11 year-old girl with segmentary vitiligo in the left side of the neck and superior part of the hemithorax, associated to an homolateral Parry-Romberg syndrome. This association had never been reported. The pathogenic hypothesis are discussed. We think that this association can be explained by the autoimmune origin of these diseases.

[Multiple glomangioma]. / Glomangioma múltiple.

A case of multiple glomus tumour is described in a 33 year old man. The clinical and histopathological features are discussed, with the review of the literature. We discuss the types of glomus tumour and its differential features.

[Amicrobial pustulosis of the folds: report of a new case and review of literature]. / Pustulosis amicrobiana de las flexuras. Descripción de un nuevo caso y revisión de la literatura.

Amicrobial pustulosis of the folds is a rare disorder characterized by a recurrent sterile pustular rash mainly affecting the skinfolds, scalp, and periorificial regions such as around the external auditory meatus. Few cases have been reported in the literature, most of them occurring women and all of them associated with some immunological disorder, the most common being lupus erythematosus. We present a new case of amicrobial pustulosis of the folds in a woman; the only immunologic abnormality detected was an elevation of immunoglobulin E. We have also reviewed the 35 cases reported in the literature.

[Diagnosis and management of nail psoriasis]. / Aspectos diagnósticos y terapéuticos de la psoriasis ungueal.

Nail psoriasis has traditionally been considered as one of the most difficult forms of psoriasis to treat. The most recent studies have found a higher prevalence of fungal infections in psoriatic nails and therefore cultures before and during treatment are necessary. Over the last few years, novel psoriatic therapies have been developed, some of which are effective for both skin and nail lesions (cyclosporine and biologic agents). Of the topical agents, vitamin D and A derivatives as well as nail lacquer containing 8 % clobetasol propionate can help improve lesions of both the nail bed and matrix.

Hodgkin's disease presenting with superficial lymph nodes and tumors of the scalp.

Invasion of the skin by malignant cells in Hodgkin's disease is a rare occurrence and less common than skin involvement in non-Hodgkin's lymphoma. We describe a 36-year-old man with Hodgkin's disease who presented with superficial lymph nodes and tumors on the scalp as initial clinical findings. Biopsy of a tumor of the scalp showed a diffuse dermal infiltrate composed of polynuclear neutrophils and eosinophils, large lymphocytes, and Sternberg-Reed cells. Ultrastructural examination of the skin biopsy specimens demonstrated the characteristic morphology of the Sternberg-Reed cells.

Generalized cutaneous B-cell pseudolymphoma. Report of a case studied by immunohistochemistry.

A 77-year-old woman presenting with multiple reddish infiltrated papulonodular lesions on her back is reported. Histologic studies revealed nodular aggregates of lymphoid cells in a B-cell pattern. Immunohistochemical studies of several lesions showed the immunoarchitecture of germinal centers. Generalized lesions of cutaneous B-cell pseudolymphomas are uncommon. Our case is one of the few reported, and the first studied by immunohistochemistry.

Tubular apocrine adenoma.

Two cases of tubular apocrine adenoma were studied by light, histochemical, and electron microscopic methods. This benign apocrine hamartoma must be considered in the classification of appendage tumors, particularly those associated with organoid epithelial nevus, and may be confused with metastatic adenocarcinoma. The tumor is characterized by dermal and subcutaneous lobular masses of tubular structures. Connection to the epidermis by one or more ductlike structures is observed. The tubules contain typical apocrine epithelial cells, some with hyaline or clear cell differentiation. The tumor cells have apocrine differentiation by histochemistry and ultrastructure. The localization to the scalp and the nodular, lobulated appearance and the apocrine structures indicate that this tumor is similar to nevus syringocystadenoma papilliferum and must be diffentiated from it.

Granuloma annulare and sarcoidosis.

Granuloma annulare (GA) and sarcoidosis are two diseases of unknown cause which involve the skin and whose basic pathology is a mononuclear histiocytic cellular reaction. Biopsy plays the major role in the diagnosis of both diseases, and no other routine laboratory test for either disease is currently available. Sarcoidosis is generally considered to be an allergic or immune granuloma with inconstant defects in cell-mediated immunity (Broom & MacLaurin, 1973). There have been no immunological studies of GA. We recently studied 14 cases and found circulating lymphokines (macrophage migration inhibition factor) in 11 which correlated with circulating macrophage migration inhibition factor in sarcoidosis (9 of 10 cases) (Umbert, Belcher & Winkelmann, 1976). The co-existence of GA and sarcoidosis in 5 patients suggests to us that there are very similar immunological reactions as well as pathology in both diseases and that the elucidation of the pathogenesis of one disease should aid in understanding the other. To our knowledge, there are no prior reports of systemic sarcoidosis coexisting with or manifesting the histological picture of GA. In this report we have demonstrated by clinical and histological criteria that GA and sarcoidosis can co-exist.

Granuloma annulare: direct immunofluorescence study.

Direct immunofluorescence studies were carried out in eleven specimens of granuloma annulare. The results (in all cases) indicate the presence of focal deposition of fibrin, localized primarily in the intervascular portion of the dermis, corresponding to the granulomatous and necrobiotic areas. These findings implicate the cellular mechanism of delayed hypersensitivity with focal involvement of the clotting system in the development of granuloma annulare lesion.

Congenital erythrodermic psoriasis: case report and literature review.

A 4-year-old girl was seen in our department for erythroderma, palmoplantar hyperkeratosis, and scalp desquamation present since birth. The dermatosis had run an intermittent course, with exacerbations after infections and spontaneous remissions. A specimen from a skin biopsy performed at 1 year of age showed the characteristic features of psoriasis, findings that were confirmed in our biopsy specimen. Treatment with acitretin controlled the outbreaks. At 7 years of age she has developed, for the first time, plaque type psoriasis. Congenital erythroderma is an unusual form of psoriasis with a wide differential diagnosis.

Serum neopterin as an objective marker of psoriatic disease activity.

Neopterin is a non-specific marker of the activation of cell-mediated immunity. Several studies have demonstrated the crucial role of CD4+ T cells in the pathogenesis of psoriasis. We have measured serum and urine neopterin levels and urine neopterin/creatinine ratios by radioimmunoassay in 24 patients with plaque-type psoriasis before and after a course of topical treatment with triamcinolone acetonide 0.1% and coal tar 4%. Results were compared with a group of 20 healthy, non-psoriatic volunteers. Serum neopterin levels were significantly elevated in the psoriatic group compared with the control group (p=0.001) and were significantly reduced after treatment (p=0.01). There was a correlation between pretreatment serum neopterin levels and psoriasis area and severity scores (PASI) (r=0.37, p=0.03) and also for pretreatment neopterin/creatinine ratios and PASI scores (r=0.45, p=0.01). These findings indicate that serum neopterin concentrations reflect disease activity in psoriasis.

Systemic lupus erythematosus exacerbated by piroxicam.

A patient with Sjögren's syndrome and seronegative polyarthritis is reported. After piroxicam intake and sun exposure she developed subacute cutaneous lupus erythematosus lesions with Ro antibodies. Despite drug withdrawal, typical cutaneous lesions and serological markers of systemic lupus erythematosus (SLE) progressively appeared. The use of piroxicam and other nonsteroidal anti-inflammatory drugs with photosensitizing potential in patients with Sjögren's syndrome, sicca syndrome or a high suspicion of a collagen disorder should be avoided because these drugs may trigger a latent SLE.

Malignant proliferative angioendotheliomatosis or angiotropic lymphoma associated with a soft-tissue lymphoma.

We report a 63-year-old man with violaceous nummular patches on the trunk. Histopathologic studies were consistent with a diagnosis of malignant angioendotheliomatosis or angiotropic lymphoma. Immunohistochemical study of skin was positive for UCHL-1 antigen and leukocyte common antigen and negative for L-26, Ulex europaeus lectin I, vimentin, cytokeratin, and epithelial membrane antigen. Ultrastructural study ruled out an endothelial origin of the neoplastic cells. These data confirmed the diagnosis of malignant proliferative angioendotheliomatosis. Five years before, a soft tissue lymphoma had been excised. This is an unusual case of malignant angioendotheliomatosis for the following two reasons: (1) a previous association with a soft tissue lymphoma and (2) the rarely described T immunophenotype of neoplastic lymphoid cells.

Lymphokines (MIF) in the serum of patients with sarcoidosis and cutaneous granuloma annulare.

Serum MIF activity was studied in ten patients with sarcoidosis, fourteen with granuloma annulare, four with necrobiosis lipoidica, and nine with various dermatological diseases. Positive MIF activity was found in the sera of nine of the ten patients with sarcoidosis and eleven of the fourteen patients with granuloma annulare. The delayed hypersensitivity tests were negative in all nine of the patients with sarcoidosis who had serum MIF activity and were positive in only three patients with cutaneous sarcoid lesions. One of four patients with necrobiosis lipoidica demonstrated minimal serum MIF activity. Data on serum lymphokine activity in sarcoidosis and granuloma annulare suggest that these two diseases are related to delayed hypersensitivity mechanisms.

Macrophage inhibitor factor (MIF) in cutaneous lymphoproliferative diseases.

Macrophage migration inhibitor factor (MIF) activity in the sera of patients with mycosis fungoides, Sézary syndrome, and cutaneous lymphoma was observed in the sera of eight of the ten patients with stage II (infiltrative) mycosis fungoides, but in only one of the eight patients with stage I and in neither of the two patients with stage III mycosis fungoides. Two of the three patients with Sézary syndrome had MIF in the serum. No MIF was observed in cutaneous lymphoma. These data support the concept that Sézary syndrome and mycosis fungoides are T-cell diseases, and transitional, prelymphomatous diseases.

Pityriasis rubra pilaris as the initial manifestation of internal neoplasia.

Pityriasis rubra pilaris (PRP) is a rare papulosquamous disorder of unknown aetiology. There are only three cases reported occurring coincident with internal neoplasia. We report a case of this disorder presenting as the initial manifestation of a previously undiagnosed malignancy with metastases in the liver. The primary tumour was not detected. The patient had a complete remission of his PRP with topical steroids alone, but died of the malignancy.