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Queen Of Punt Syndrome

Clinical Genetics

At Cairo museum, on Queen Hatshepsut’s tomb in Deir el Bahari,it shows the chief Parihou with his wife Ati, Queen of Punt, (an area not still geographically established: Yemen, Somalia, Ethiopia, Eritrea, Sudan?) while they offer gifts to the Egyptian Queen Hatshepsut (1516-1481 BC).

A naval expedition to the mysterious land of Punt was undertaken in the summer of Hatshepsut’s eighth year as queen; she sent a fleet of five ships, headed by her Chancellor Senenmet. The Queen of Punt shows a rugged face, gluteal and femoral obesity, hyperlordosis and symmetrical deposits of fat on the trunk, limbs and thighs.

Endemic Leprosy In Europe - A Call For Information

Epidemiology

Leprosy, attributable to infection with Mycobacterium leprae, was once endemic over much of the world. Though now often considered a “tropical disease,” cases occurred north of the Arctic Circle just 100 years ago. The disease has gradually disappeared from higher latitudes in recent centuries.

Aristotle

We are what we repeatedly do. Excellence, therefore, is not an act but a habit.
Aristotle

The Medicine of the Templars

Science History

At Paris, 18 March of 1314, on the island of the Seine in front of the Garden real, Jacques de Molay, the last Great Master of the Templars, and Geoffroy de Charny, preceptor of Normandy, were burned as heretics.

Thus finishes the history of the Knights of the Temple after two centuries. The Templars would have been in possession of the most hidden secrets of alchemy. They were first to use the IPERICO on burns and hurts from cut, like antiseptic, astringent, healing, and in order to improve humor of the soldiers that remained immobilized in bed for months.

Medieval Medicine

Science History

Medieval medicine gave great importance to the planets as influences in disease. The influence of the stars began at birth and influenced complexion. The continuous flow of celestial forces could change the course of disease.

The position of the planets was important in choosing the moment in which to begin cures or carry out a bloodletting. In "Regimen against the Plague", Siegmund Albich (1347-1427) invites readers not to think about the plague because that was sure to cause its appearance.

During the Middle Ages, the prevailing popular attitude in medicine was dictated by S. Ambrogio (334-378) who declared " the garnishments of the medicine are contrary to celestial science, contemplation and prayers".

Hieronymus Fracastorius

Science History

Girolamo Fracastoro (also known as Hieronymus Fracastorius), was born in 1478 in Verona, at that time still part of the Republic of Venice, to a noble family. He studied at Padua University, where he graduated in 1502. At the same University, he was assigned the chair of Logic and Philosophy.

His teacher was the doctor-philosopher Pietro Pomponazzi and his study colleagues were Andrea Navagero, who became a noted historian, along with Pietro Bembo and Gaspare Contarini, both of whom became Cardinals.

Medicine was his passion but he was also a humanist and a scientist, he was interested in astronomy, mathematics, physics, botany, geology, geography, and even composition of verses. He was contemporary and friend to Nicolaus Copernico (Copernicus).

The History Of Ancient Medicine (Abstract)

Anthropology

From Gods To Avicenna

Originating as divine and supernatural, Greek medicine changed and moved toward analysis and logical thinking during the period 800 B.C. to 460 C.E.

Thales (636-546 B.C.), philosopher and scientist, undertakes examination about the laws of nature and physics. He supposed that water (moisture) was the first element from which the world was formed.

Empedocles (Agrigento c.495- c.435 B.C.) philosopher and physician, who lived in Sicily, wrote “On Nature” and “On Purification“. Its system was based on the interaction of the four elements (fire, air, earth and water), called by him “rhizomata” (roots) under the influence of love and hate (attraction and repulsion). He studied circulation of the blood and atmospheric pressure, foreshadowed view of evolution. He was the founder of Italian medicine.

Cyclosporine Therapy Of Atopic Dermatitis

Medicine

Reported: a 42 year old female patient with a serious atopic dermatitis, abruptly appearing since age 8.

During several hospitalizations, the patient underwent various therapies without reaching -she relates- any amelioration but on the other hand getting bad influences on the relationship, due to the deep hormonal diseases induced by the cortico therapy.

Kept under observation from october 1995, the patient was subjected to an increasing treatment with cyclosporine, starting with a 2,5 mg/Kg/die at the end of an 8 months timeframe archiving a total recovery of the cutaneous pathology and being enabled to a normal social life.

Lassueur Piccardi Graham Little Syndrome

Medicine

The first case of progressive scalp cicatricial alopecia and
follicular lichen planus (LP) on the trunk and extremities, was
described by Piccardi in 1914, to which he gave the name
"cheratosi spinulosa".

Graham-Little published a similar case in
the successive year of a 55 year old woman, referred by Lasseur of
Lausanne,Switzerland. LASSUEUR PICCARDI GRAHAM LITTLE Syndrome is a rare case where are associated "Cicatricial alopecia and lichen planus follicularis" come together small confluent patches of progressive scarring alopecia, appearance of the lichen planus follicularis with follicular keratosis and noncicatricial alopecia of the axilae and pubes.

ABCD's of Melanoma

Public Health

Malignant Melanoma is the most aggressive of malignant cutaneous tumours. Cases with lymphonode involvement, and distant metastases, carry a very poor prognosis, (50% and 20% respectively alive in 5 years), while those presenting without evident lymphonode involvement have a much better survival rate (60% alive in 5 years).

It is therefore extremely important to do early malignant melanoma diagnosis. There are several criteria that may lead to the diagnosis of a malignant melanoma. The most important one is the ABCD rule.

The ABCD diagnosis of Malignant Melanoma -

A = Asymmetry: Moles are commonly symmetrical and round. Very early melanomas, are asymmetrical assuming different forms. One half does not match the other half.

B = Border: Commonly moles, have even and smooth borders. Early melanomas, are usually uneven and notched borders.The edges are ragged, notched or blurred.

C = Color: Moles commonly are a single shade of brown, early malignant melanomas shows brown-tan or black color. Further, as melanomas progress, may see red, white, and blue color.

D = Diameter: Early malignant melanomas, differently from moles, tend to grow larger in diameter. First suspects in melanoma start fom, a 6 millimeters diameter on. Any sudden or continuing increase in size should be of special concern.

Local Control Of Malignant Melanoma

Research

The case of a 47 year-old woman presenting a wide abdominal zone with vitiligo like hypopigmentation is reported.

In 1977 she underwent, in the same site, melanoma (III Clarke's level) extirpation and local immunotherapy by DNCB ( dinitrochlorobenzene ).

Moshe Inbar et Al. ( Melanoma Research 1996/6/457-459 ) refers a case of melanoma, treated by DNCB in 1973, showing, the new skin, over the back, characterized by vitiligo like hypopigmentation and lack of hair.

HIV And Dermatology

Research

In people affected by acquired immuno-deficiency syndrome it may often be observed a rise of cutaneous emergencies, whose impact spans from 25% in asyntomatic subjects to 100% in the case of very evident AIDS.

The frequency and the atypic nature of these emergencies gives a highlighted role to the dermatologist, characterizing the early diagnosis of cutaneous pathologies as the qualifying moment in the analysis of AIDS affected patient.

As the starting moment in the HIV-positive subject examination, search for injuries referable to Kaposi's sarcoma, especially at the mucous level (pharinx wounds are evident in 10 to 50% of total cases).

Bioterrorism - Plague As A Biological Weapon

Public Health

Introduction: Bioterrorism is the utilization of microorganisms or toxins in order to produce a disease and/or death in human beings, animals or plants. Different from conventional weaponry, relatively economic means are used that allow the elimination of living beings without destroying the surrounding atmosphere.

The most probably method for spread of bioweapons is aerial transmission, continuing into water supplies and food. The CDC (US Centers for Disease Control and Prevention, Atlanta) has subdivided into three categories the aggressiveness of "Bioweapons".

Category A comprises:

History of Syphilis

Epidemiology

In the work "Tractado contra el mal serpentino" written in 1510 and published in 1539, Ruy Diaz de Isla refers to have cured, during the travel of return in Europe, many members of the shipment of Columbus, affections from certain luetic manifestations and thinks the new disease was imported from Hispaniola (Haiti).

Bartolomè de Las Casas had conceived the same opinion. In the "Historia de Las Indias" he wrote as between the Conquistadores the idea of the "bestiality" of the wild Americans was prevalent and the disease would have been known already previously in the New World.

Moreover the most modern historiography places the accent on the instrumentalization of this idea to the ends of the colonial enslavement. The aborigines: lustful, inferiors, "homuncoli" (De Oviedo), naturally needy of being converted and to receive, therefore, with the faith also the slavery.

Brodie's Disease-Madelungs Fetthals-Launois Bensaude's Lipomatosis-MSL

Medicine

Prof. Camillo O. DI CICCO, M.D.
XIth Congress of the European Academy of Dermatology and Venereology, Prague.

The first studies of the disease go back to 1846 by B.C. Brodie, which describes a picture of lipomatosis symmetrical disseminated with interest of the neck in the job " Clinical Lectures on Surgery, Delivered at St George's Hospital " Philadelphya, Lea and Blanchard (pub) 1846 Pp 201-210.

Subsequently such picture was described from Huguier (1855)), Founder (1863) and Vermeuil (1888).

In the same year Otto W. Madelung has accurately described the symptomatology in very 33 clinical cases[ Ueber den Fetthals: Diffuse Lipom des Halses: (von Langenbeck's) Archiv fur Klinische Surgeries, Berlin, 1888,37:106-130].

In the following years were published several jobs by Bucquoy, Dartignolles and Lèjars (1892), Koettnitz (1894), Payr (1895), Lowenthal(1896), Dalchè and Hayem (1897), Preyss (1898).

In 1898 with the detailed description of 75 clinical cases in "De adénolipomatose simmétrique" (Bull.Soc.Mèd.Hop.Paris,Mémoires,1898,1:298-318), P.E. Launois and R. Bensaude defined clearly such rare disease.
The clinical picture of the disease is characterized from the presence of symmetrical multiple masses of fat, painless, on the neck, upper trunk, arms and legs.

"Men that look no further than outside think health an appur

"Men that look no further than outside think health an appurtenance unto life and quarrel with our condition of being sick. But I who have looked at the innermost parts of man and known what tender filaments that fabric hangs on oft wonder that we are not always so. And considering the thousand doors that lead to death do thank our God that I can die but once".

Launois-Bensaude's Lipomatosis - Mitochondrial DNA

Medicine

Even though Brodie is said to have first described diffuse symmetrical lipomatosis with predilection for the neck already in the 1846 (Clinical Lectures on Surgery, Delivered at St. George's Hospital Philadelphia: Lea and Blanchard pub. Pp 201-201. Brodie, B.C.) still unknown is today the pathogenesis of Launois Bensaude' Lipomatosis.

Klopstock et Al. are found in muscle biopsy specimens ragged and red fibers, pathologic subsarcolemmal accumulations of mitochondria, suggesting a mitochondrial abnormality. Biochemical analysis of respiratory chains shows a decreased of cytochrome c oxidase activity.

Multiple deletions of mitochondrial DNA are found in one case (Southern Blot analysis).Gamez J. et Al. describe the 8344 mutation in the tRNA (Lys) gene of mitochondrial (mtDNA ) in another case.

The distribution of the mutation was unusual because the proportion of mutated genomes was higher in blood and lipomas than in muscle tissue.

The distribution of the mutation was unusual because the proportion of mutated genomes was higher in blood and lipomas than in muscle tissue.

Therefore, using lymphocytes of patients a important decreasing in oxygen consumption of intact lymphocytes as a decreased activity of components of the respiratory chains were detected. ( Becker-Wegerich et Al.).

Consequently, the mutations and deletions of mitochondrial DNA, can be hypothetical "Primum Movens" of Launois Bensaude's Lipomatosis.

Prof. Camillo O. DI CICCO, M.D.
14th Congress of the European Academy of Dermatology and Venereology, London, UK.

Kindler Syndrome

Clinical Genetics

A case is reported of a 28 year old man referring the appearance of swollen blisters due to insignificant trauma since birth. Later he noticed progressive changes of the skin as teleangectasies, atrophic spots, sensivity to sun, dystrohic fingermails and webbing between fingers.

The patient was hospitalized several times for an appropriate diagnosis and asked for the permission to undergo an operation of plastic surgery in Paris to correct webbing of the hands.
The rare association of two congenital diseases, epidermolysis bullosa dystrophica and poikiloderma, leads to the diagnosis of a Kindler syndrome.

The Kindler Syndrome described by Theresa Kindler in 1954 is probably a variant of hereditary acrokeratotic Poikiloderma in whitch Poikiloderma is preceded by a tendency to blistering following traumatic blisters.

There are not many ultrastructural studies on this syndrome. The ultrastructural level of blister formation has not been well characterized. The cutis of the hand back of our patient has been examined at the electronic microscope and it shows a thinned epidermis with a normal keratinization and a compact corneous ortokeratosic layer. A dermo epidermic flaking is noticed in several points and at different levels.

Since the first description in 1954 approximately 70 cases have been reported worldwide.

KINDLER SYNDROME (Abstract)
Prof. Camillo O. DI CICCO, M.D.

Presented to VIth Congress of the European Society for Pediatric Dermatology.

Incontinentia Pigmenti

Clinical Genetics

Incontinentia pigmenti is a rare genodermatosis also called Bloch-Sulzberger syndrome or Bloch-Siemens, that shows early at birth or in the neonatal period.In its classical form,the cutaneous symptomatology develops through three steps.

1st step, with evidence of injuries, of erythematic-vescicular-blistered kind and one wave after another, linearly positioned and involving upper body and limbs;haematic hypereosinophilia is also present.

2nd step, that pops up between the second and the sixty life-week with papulo-lichenoid injuries,hyperkeratosic and warty, looking as elongatad striae in the distal limbs section ( knee, foot-and hand back ).

3rd step, when at third-sixth month of life dark pigmented spots appear at the upper body level, positioned like a vortex, a whirl or spurts.

Xeroderma Pigmentosum

Clinical Genetics

The first descriptions of the disease are found again in the Textbook of Dermatology, published in 1874, "On disease of the skin, including exanthemata" London - New Sydenham Society, Hebra F.-Kaposi M.

The term "Xeroderma Pigmentosum" was coined from the hungarian dermatologist Moritz Kaposi wanting in such a way to indicate a characterized disease picture from pigmented and dry skin.
Hereditary disease, trasmitted with recessive autosomical modality, the XP is characterized from extreme photosensivity that causes strict and premature damages to level of the cutis and of the eyes. Its incidence is of 1:250000 in Europe and USA, while in Japan the relationship is of 1:40000.

In the child affected by XP also short exposure to the sun's ultraviolet rays determines severe cutaneous sunburn with slow resolution, therefore it is from avoiding sources of ultraviolet cancellations categorically, considering moreover that the effects of the exposures are cumulative in the time.

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