An uncommon case of systemic Mucormycosis associated with spinal cord infarction in a recently diagnosed diabetic.

Mucormycosis is a relatively rare, opportunistic, invasive infection caused by various members of the Phycomycetes class [from Greek phyko- seaweed; having a plant body], an extensive taxonomy introduced in 1956 to enlarge the class of Zygomycetes. These filamentous fungi have a worldwide distribution and are capable of rapid growth and thermotolerance of human body temperature. Infection typically occurs in seriously compromised patients (i.e. diabetic ketoacidosis, hematologic malignancies, immunosuppressive disorders, end-stage renal disease, solid-organ or bone-marrow transplantation) and can be acute or fulminant, as well as indolent and chronic. In this paper we describe a case of cutaneous mucormycosis that occurred in a 54-year-old diabetic woman and evolved into a disseminated form, leading to an uncommon spinal cord infarction and consequent paraparesis. Our case suggests the importance of suspecting a mucormycosis infection in patients with decompensated diabetes mellitus, even without ketoacidosis.

Impetigo herpetiformis occurring during N-butyl-scopolammonium bromide therapy in pregnancy: case report.

Impetigo herpetiformis (IH) is a rare dermatosis arising during the third trimester of pregnancy which is generally considered as a form of pustular psoriasis of unknown aetiology. Clinically it is characterized by erythematous plaques surrounded by sterile pustules associated with fever, diarrhea, sweating and increasing risk of stillbirth for placental insufficiency. We describe a case of developed erythematous plaques surrounded by pustules localised initially to the trunk of a 35-year-old woman at the 34th week of gestation after 5 days of treatment with N-Butyl-Scopolammonium, and which later involved the upper and lower limbs. Skin histology confirmed the diagnosis of generalised pregnancy pustular psoriasis (impetigo herpetiformis). IH is reported to be associated with hypocalcemia, hypoparathyroidism, use of oral contraceptives and bacterial infections. This is the first report suggesting the potential role of drugs other than oral contraceptives in the pathogenetic mechanism of this disease. In this case an adverse cutaneous reaction to BB could be the cause of the development of Koebner isomorphism.

Penicillamine-related lichenoid dermatitis and utility of zinc acetate in a Wilson disease patient with hepatic presentation, anxiety and SPECT abnormalities.

Wilson's disease is an autosomal recessive disorder of hepatic copper metabolism with consequent copper accumulation and toxicity in many tissues and consequent hepatic, neurologic and psychiatric disorders. We report a case of Wilson's disease with chronic liver disease; moreover, in our patient, presenting also with high levels of state anxiety without depression, 99mTc-ECD-SPECT showed cortical hypoperfusion in frontal lobes, more marked on the left frontal lobe. During the follow-up of our patient, penicillamine was interrupted after the appearance of a lichenoid dermatitis, and zinc acetate permitted to continue the successful treatment of the patient without side-effects. In our case the therapy with zinc acetate represented an effective treatment for a Wilson's disease patient in which penicillamine-related side effects appeared. The safety of the zinc acetate allowed us to avoid other potentially toxic chelating drugs; this observation is in line with the growing evidence on the efficacy of the drug in the treatment of Wilson's disease. Since most of Wilson's disease penicillamine-treated patients do not seem to develop this skin lesion, it could be conceivable that a specific genetic factor is involved in drug response. Further studies are needed for a better clarification of Wilson's disease therapy, and in particular to differentiate specific therapies for different Wilson's disease phenotypes.

Lymphoepithelioma-like carcinoma of the skin.

The term lymphoepithelioma-like carcinoma identifies a group of nasopharingeal epithelial tumors characterized by aggregates of malignant undifferentiated cells surrounded by a dense reactive lymphoplasmacellular infiltrate. Primary cutaneous localization is rare, with approximately 30 cases reported in literature. We describe a case of primary lymphoepithelioma-like carcinoma of the skin in a 92-year-old woman. Immunohistochemical examination was positive for cytokeratine (KL1 and EMA) as regards epithelial cells, while the lymphocitic infiltrate was positive for LCA and CD3. In situ hybridization for Epstein Barr virus in tumor cells was negative. Electron microscopy showed rounded and occasionally spindle-shaped poorly-differentiated squamous epithelial cells, and a lymphoid stroma consisting mostly of normal-appearing small lymphocytes. Examination of the nasopharynx did not show any tumoral mass and after a 7 years follow-up the patient is free of local and distant recurrences. This tumor affects people aged over 50 years and is localized to the face, but scalp, shoulder and forearm may be involved. Research of Epstein-Barr virus is always negative in this tumor, unlike nasopharingeal epithelial carcinoma. The differential diagnosis of lymphoepithelioma-like carcinoma of the skin may present some difficulties and includes squamous cell carcinoma. Lymphoepithelioma-like carcinoma of the skin is a malignant neoplasm which tends to relapse locally and has a moderate tendency to metastatize.

Cutaneous manifestations in celiac disease.

Celiac disease (CD) is an autoimmune gluten-dependent enteropathy characterized by atrophy of intestinal villi that improves after gluten-free diet (GFD). CD is often associated with extra-intestinal manifestations; among them, several skin diseases are described in CD patients. The present review reports all CD-associated skin manifestations described in the literature and tries to analyze the possible mechanisms involved in this association. The opportunity to evaluate the possible presence of CD in patients affected by skin disorders is discussed.

Management and dermoscopy of fast-growing nevi in pregnancy: case report and literature review.

BACKGROUND: The relationship between pregnancy and a change in melanocytic nevi is still controversial. Moreover, management of the rapid evolution of a nevus in an unauspicious melanocytic lesion can be a clinical challenge in pregnancy. METHODS: This article examines a case of a fast-growing deep penetrating nevus in a pregnant woman and provides a literature review of articles relative to pregnancy and nevi change, the management of fast-growing pigmented lesions, and the role and usefulness of dermoscopy in these cases. RESULTS: Recent studies have documented that pregnancy is not associated with any significant change in the size of melanocytic nevi. The management of fast-growing melanocytic lesions during this period compulsorily leans toward excision. Dermoscopy can be useful, providing clinicohistopathologic correlations and a better assignment of the lesion. CONCLUSION: This case report and review provide important management considerations for nevi during pregnancy. Early intervention with aggressive treatment measures is the best management for fast-growing lesions, and epiluminescence dermoscopy can assist the management, although still remaining a second-level examination, useful for documentation and for a better classification of the lesion.

Expression of the high-affinity laminin receptor (67 kDa) in normal human skin and appendages.

The interaction of cells with extracellular matrix components plays a significant role in the regulation of cell biology. Laminin is a large glycoprotein involved in fundamental interactions between cells and the basement membrane. Several cell surface receptors are responsible for cell-matrix interactions. The 67 kDa high affinity laminin receptor, 67LR, is involved in the adhesion of normal cells to the laminin network and is also associated with the metastatic phenotype of some tumoral cells. We have investigated the expression of laminin and of the 67LR in normal human skin using immunoperoxidase staining. Twenty samples of skin were analyzed. Antibody against laminin reacted in a continuous linear band at the dermal-epidermal junction, as well as basement membranes of hair follicles, sebaceous and eccrine sweat glands, and dermal blood vessels. The epidermis and the follicular epithelium were negative for laminin. The 67LR seemed not to be expressed on the basal surface of basal keratinocytes. The major expression of this receptor may be detected in the upper half of the spinous layer and in the granular layer. The cells of the outer root sheath in hair follicle showed the same immunohistochemical pattern described for epidermis. In sebaceous glands and in eccrine sweat glands the secreting epithelium was positive. Endothelial cells of dermal blood vessels were routinely positive for 67LR. We observed that the expression of the 67LR in normal human skin is mostly located in epidermal areas in which the keratinizing process was particularly advanced.

A case of Sweet's syndrome associated with monoclonal immunoglobulin of IgG-lambada type and p-ANCA positivity.

We report a case of Sweet's syndrome associated with monoclonal gammopathy of uncertain significance (MGUS) and positivity for perinuclear antineutrophil cytoplasmic antibodies (p-ANCA). A 65-year-old man patient came to our hospital complaining of remittent-intermittent fever, weight loss, associated with papules affecting all his body and without mucosal or ocular involvement. Histology of biopsy specimens of papules allowed the diagnosis of Sweet's syndrome. This is the first case of this rare syndrome associated both with MGUS and p-ANCA positivity. The monoclonal immunoglobulin, possibly directed to neutrophils like an antibody, may cause both their fragmentation and release of antigens responsible of p-ANCA appearance.

Non-Herlitz junctional epidermolysis bullosa without hair involvement associated with BP180 deficiency.

Junctional epidermolysis bullosa (JEB) is a clinically and genetically heterogeneous recessively inherited blistering disease of the skin and mucous membranes due to impaired epithelial adhesion. In particular, defective expression of the 180-kD bullous pemphigoid antigen (BP180) has been correlated to a non-lethal (non-Herlitz) form of JEB, generalized atrophic benign epidermolysis bullosa (GABEB), characterized by widespread skin blistering healing with atrophy and by atrophic alopecia with onset in childhood. We report the case of a 33-year-old man suffering from a generalized blistering skin disorder since birth. He also presented nail dystrophy and tooth abnormalities. Mucosal involvement was limited to gingival erosion. Alopecia was absent and body, axillary and pubic hair were normal. Immunofluorescence analysis showed a markedly reduced expression of BP180, electron microscopy studies evidenced hypoplastic hemidesmosomes and Northern blot analysis confirmed a striking decrease in the amount of BP180 mRNA. The clinical features of our patient confirm that BP180 deficiency usually results in a non-Herlitz JEB form. However, the degree of skin, mucous membranes and hair involvement appears more variable and less typical than originally described for GABEB.

Follicular mucinosis successfully treated with isotretinoin.

We describe the case of a 33-year-old Caucasian male with follicular mucinosis successfully treated with isotretinoin. Follicular mucinosis is a primary idiopathic disease or a secondary, lymphoma-associated dermatosis. An effective standard therapy for this disease is unknown. In our case, isotretinoin led to a dramatic improvement of the skin lesions in about two weeks. To the best of our knowledge, the benefits of isotretinoin in the treatment of follicular mucinosis have never been reported previously. The efficacy of this drug could be mediated by a regulatory effect on the infiltrating cells and/or by a modulation of the target organ (skin) response to the infiltrating cells.

Multiple squamous cell carcinomas of the skin during long-term treatment with hydroxyurea.

Hydroxyurea is a chemotherapeutic agent used extensively for myeloproliferative disorders. Cutaneous side effects have been described during long-term hydroxyurea treatment. We described the occurrence of multiple squamous cell skin carcinomas in a patient treated with hydroxyurea for chronic myelogenous leukemia. The lesions were removed and the hematological therapy switched to busulfan. In a previously reported case, the development of cutaneous epithelial cancers required the discontinuation of hydroxyurea, in addition to the surgical excision of the neoplastic lesions. Since squamous cell carcinoma is a malignant cutaneous neoplasm that can metastatize, the surveillance of skin changes is advisable during hydroxyurea treatment.

Anaplastic progression of classic Kaposi's sarcoma.

We describe a case of classic Kaposi's sarcoma (KS) in an Italian HIV-negative patient, with bone involvement and progression to anaplastic histotype. At the age of 22, violaceous patches of KS appeared on his feet. At the age of 50, he noted the appearance of a violaceous firm nodule on his right wrist. The lesions grew rapidly and became ulcerated. Radiotherapy led to a complete remission of symptoms. At the age of 55, a subcutaneous nodule developed on the proximal third of the right forearm associated with a wide painful edema of the right forearm and the proximal third of the right arm. The nodule enlarged rapidly, and an X-ray of the right forearm revealed the presence of a large osteolytic area of the ulna. A biopsy specimen from the right ulna showed bone erosion by a mesenchymal neoplasia with a high degree of malignancy. The right arm was amputated, and histologic examination of the surgical material confirmed the diagnosis of undifferentiated spindle-cell malignant neoplasia strongly positive for factor-VII-related antigen and CD34 antigen. Three years after surgical treatment, no recurrences have been observed.

[Huangdi Neijing: a classic book of traditional Chinese medicine]. / Huangdi Neijing: un classico della medicina tradizionale cinese.

According to the data of the recent archaeological excavations, the history of traditional Chinese medicine begins in the neolithic period (10,000-4,000 years ago). The fundamental principles of the traditional Chinese medicine may be interpreted on the basis of two classic theories of Chinese thought: the Yin-Yang theory and the five phases theory. The former stated that our reality is the product of the continuous interaction and transformation of the Yin and Yang principles. The latter recognise the existence of five emblematic groups in which everything regarding humankind and nature may be classified. The five phases interact in two different ways: the cycle of production (Sheng) and the cycle of control (Ke). Theoretical basis of traditional Chinese medicine are described on the Huangdi Neijing (The Book of Yellow Emperor's: a classic of internal medicine) written during the period 475-225 b.C. as a dialogue between the mythical Emperor Huangdi and his physician Qibo. The book is divided into two parts: the Suwen (Questions about living matter) and Lingshu (The vital axe). Each part is composed of 81 sections. In this book are set correspondences between phases and organs, viscera, feelings, body fluids, flavours, foods, colours, and so on. We can also find the description of blood (Xue) and energy (Qi) circulation and the pathways of main, collateral and curious vessels. The origins of disease are also reported on the Huangdi Neijing. As regards the psychological point of view, special attention is dedicated to dreams and their possible causes and to the description of various kind of mental illness. The unknown Authors underline the five defects and the four mistakes of the physician, pointing out the importance to avoid them. Finally, it must be mentioned their special care to the principles of preventive medicine.

[Eosinophilic pustular folliculitis and Ofuji disease. A case report]. / Le follicoliti pustolose eosinofiliche e la malattia di Ofuji. Caso clinico.

We describe the case of a young man of Calabrian origin, who came to our observation for the appearance of erythematous pustular, intensely itching, lesions on the arms, trunk and, in a less extent, on the face. The blood count revealed a differential cell count of 16.8% eosinophils. Serum IgE levels were elevated (1000 IU/ml), and T cell subsets showed an increase in CD8+ and a decrease in CD4+ with an inversion of CD4+/CD8+ ratio (= 0.78). The result of the following investigations were either normal or negative: anti-(ds)DNA antibody, anti-nuclear antibody, anti-smooth muscle antibody, anti-striated muscle antibody, serological tests for viral, bacterial, fungal and parasitic diseases and cultural examination of the material from lesion. Histopathological examination of a biopsy specimen from the left arm showed the presence of abundant perivascular inflammatory infiltrate in the dermis and inflammatory infiltrate, with numerous eosinophils, around sebaceous glands. Taken together, all these data suggest the diagnosis of eosinophilic pustular folliculitis, a dermatosis of unknown etiology, with a histopathological picture identical to Ofuji's disease. Eosinophilic pustular folliculitis can be associated with HIV infection or haematological diseases (as non-Hodgkin lymphomas, myeloma, etc.); it was also reported in adult immunocompetent healthy individuals and in children. On the basis of our findings, we propose that this case should be classified as an idiopathic form, as we were not able to demonstrate any associated disease.

[Cutaneous tuberculosis: a case of difficult classification]. / Tubercolosi cutanea: un caso di difficile classificazione.

We describe a case of cutaneous tuberculosis in a 25-year-old Philippine man. Erythematous papulo-nodular lesions, measuring 0.5 to 1 cm, were present on lower limbs; a 2 cm nodule was located on the left flank. The examination of the skin biopsy showed the presence of a superficial and deep perivascular dermatitis with histiocytes, lymphocytes and plasma cells. Ziehl-Nielsen stain for mycobacterium were negative, while the cultural examination led to the isolation of M. tuberculosis. Direct smear and cultural examination of sputum for M. tuberculosis were negative; chest and skeletal roentgenograms, syphilis and HIV infection serology, haematological and hematochemical examinations and urinalysis were negative. Specific treatment with isoniazid, rifampin and ofloxacin led to a rapid remission of cutaneous lesions. This case was particularly difficult to classify, but the Authors think it would be considered a tuberculid.

[Skin manifestations in 140 kidney transplants]. / Manifestazioni cutanee in 140 trapiantati renali.

Cutaneous lesions can be a significant problem in kidney transplant recipients. The AA report a clinical spectrum of iatrogenic, infectious, preneoplastic and neoplastic skin diseases in 140 renal transplant recipients observed, from march 1988 to july 1991, at the Catholic University in Rome. Iatrogenic skin manifestations were the most common, followed by infections of the skin and preneoplastic and neoplastic cutaneous lesions.