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Pituitary Diseases

  1. Panhypopituitarism
    1. The skin is dry, smooth, soft and pale.
    2. The face may be puffy, facial fold is decreased. Fine wrinkles are commonly found around the eyes and mouth making the patient look older.
    3. The scalp hair is dry, fine and generally thin.
    4. Characteristically, there is uniform loss of body hair. Axillary hair is affected first and public hair loss takes longer to develop. Loss of beard hair may appear, too.
    5. Finally, the nails are fragile, thin and opaque.
  2. Acromegaly
    1. On the face, the acromegalic appearance is very typical and presents as frontal bossing, widening of the nose, coarsening of the skin, and enlargement of the lips.
    2. The facial, neck and scalp creases are accentuated. Infrequently, overgrowth of the dermis results in ridging of the skin of the scalp (cutis verticis gyrata).
    3. Soft tissue swelling of the hands and feet are very common.
    4. The sebaceous glands and sweat glands are enlarged.
    5. Sessile or pedunculated fibromas are found in 20 to 30 percent of patients. Acanthosis nigrican is found in about 10 percent of cases.
  3. The Porphyrias
    Porphyrias result from either acquired or inherited enzymatic abnormalities in haem synthesis.
    There are 2 types: the erythropoietic porphyria and the hepatic porphyria.
    1. The erythropoietic porphyria (EP)
      It includes the congenital erythropoietic porphyria and erythropoietic protoporphyria.
    2. Congenital Erythropoietic porphyria (Gunther's disease)
      1. This is an extremely rare autosomal recessive disorder and is due to a decreased activity of uroporphyrinogen cosynthetase.
      2. Photosensitivity is quite significant and presents as blisters, vesicles and bullae in sun-exposed areas. These skin lesions heal slowly with scarring, atrophy and mutilating deformities. Hyperpigmentation and hypertrichosis are common.
      3. Bone abnormalities such as resorption of distal phalanges and sclerodacyl may occur.
      4. Haemolytic anaemia and splenomegaly may be present.
      5. Erythrodontia (red-stained teeth) in both deciduous and permanent teeth is pathognomonic of erythropoietic porphyria.
      6. The urine also fluoresces reddish.
      7. The diagnosis is made from the early onset of severe cutaneous photosensitivity associated with red fluorescent urine and erythrodontia.
      8. The treatment is usually preventive. Avoidance of sunlight must be emphasized.
      9. Splenectomy may be useful in dealing with intractable hemolytic anaemia.
  4. Erythropoietic Protoporphyria (EPP)
    1. An autosomal dominant disorder which is characterized by cutaneous photosensitivity, cholelithiasis and potentially severe liver disease.
    2. The acute episodes of photosensitivity include itching, burning or pruritis in sun-exposed areas and occur within minutes after sun-exposure.
    3. These are followed by erythema, oedema, urticarial lesions and rarely purpura a few hours later.
    4. Chronically damaged skin may heal with superficial pitted and linear scars especially on the cheeks and nose.
    5. Pseudorhagades, thickened and leathery skin over the knuckles and fingers together with the wrinkles give an appearance of premature aging.
    6. The diagnosis is made by detecting elevated levels of free protoporphyria in the red blood cell and/or faeces. Photoprotection is essential.
    7. Beta-carotene has been found helpful in preventing or minimizing the symptoms of cutaneous photosensitivity reactions. In severe end-stage liver failure, liver transplantation will be the last resort.
  5. The Hepatic Porphyria
    The hepatic porphyria includes porphyria cutanea tarda, acute intermittent porphyria, variegate porphyria and hereditary corproporphyria.
    1. Porphyria Cutanea Tarda (PCT) – Most common porphyria
      1. PCT is due to the decreased activity in uroporphyrinogen decarboxylase and is divided into two categories.
        1. Type I (symptomatic, acquired) PCT is characterized by the deficiency of the enzyme in the liver only.
        2. Type II (hereditary)
      2. PCT is inherited in an autosomal dominant fashion and the uroporphyrinogen decarboxylase is decreased approximately 50 percent in all tissues.
      3. Vesicles and bullae appear on sun-exposed areas or in areas subjected to repeated trauma.
      4. Increased skin fragility is common and lesions usually heal with milia formation.
      5. Hypertrichosis (non-virilizing) is a useful diagnostic sign. Hyperpigmentation, sclerodermoid plaques and scarring alopecia are not uncommon.
      6. Ethyl alcohol, estrogen hormones, hexachlorobenzene, chlorinated phenols and iron have been associated with the exacerbation of PCT.
      7. Patients with PCT excrete increased amounts of porphyrins in the urine, which give rise to pink-red fluorescence under wood's lamp examination.
      8. In equivocal situation, quantitative 24 hour urine uroporphyrin and coproporphyrin determinations and stool protoporphyrin and coproporphyrin determinations should be performed.
      9. Drugs that may have triggered the disease must be strictly avoided.
      10. Phlebotomy is a safe, effective and relatively simple method of therapy for PCT.
      11. In some patients who are not suitable for phlebotomy, may respond to antimalarials. e.g. chloroquine and hydroxychloroquine.
  6. Variegate Porphyria (VP)
    1. VP is an autosomal dominant disease and is quite common among the white.
    2. The clinical manifestations of VP include those of AIP and PCT, either or both of which may occur in the same individual
    3. The skin manifestations do not correlate with the acute attacks in most patients.
    4. The diagnosis is made as shown by the elevated urinary ALA and PBG levels during acute attacks of VP but characteristically fall to normal levels between attacks.
    5. Precipitating factors and drugs should be avoided. Photoprotection for photosensitivity is a must.
  7. Hereditary Coproporphyria (HCP)
    1. HCP is an autosomal dominant disorder and is due to deficiency of coproporphogen oxidase activity in red blood cells and lecuocytes.
    2. It is an extremely rare disease.
    3. The acute attacks are similar to those of AIP but are milder than that of AIP.
    4. Cutaneous photosensitivity occurs in 20 percent of cases.
    5. HCP is characterized by increased excretion of coproporphyrin III in urine and faeces.
    6. Avoidance of drugs, glucose loading and hematin infusions may be helpful in dealing patients with HCP.


Pruritus Associated With Systemic Disease

Systemic Disease

Postulated Cause


Secondary hyperparathyroidism, high skin calcium concentration.

Obstructive biliary disease

High concentrations of bile salts in skin

  Primary biliary cirrhosis


  Cholestatic hepatitis secondary to drugs (chlorpropamide)


  Intrahepatic cholestasis of pregnancy


  Extrahepatic biliary obstruction


Hematologic and myeloproliferative disorders


  Lymphoma including Hodgkin's disease


  Mycosis fungoides


  Polycythemia vera


  Iron-deficiency anemia


Endocrine disorders










Visceral malignancies


  Breast, stomach, lung


Psychiatric disorders




  Delusions of parasitosis


Neurologic disorders


  Multiple sclerosis (paroxysmal itching)


  1. Itch and pain are carried on unmyelinated C fibers found in the upper portion of the dermis of the skin, mucous membranes, and cornea.
  2. The afferent C fibers enter the dorsal horn of the spinal cord, synapse, cross the midline, and ascend the spinothalamic tracts to the thalamus. Then the impulse proceeds to the sensory area of the postcentral gyrus of the cortex.
  3. Cutting the spinothalamic tract, as in an anterolateral hemichordotomy, abolishes pain and itch
  4. Generalized itching in the absence of primary skin disease (pruritus) may be an important sign of internal disease

Management of pruritus associated with chronic renal failure

i. Ultraviolet B (UVB) phototherapy twice weekly

ii. Oral activated charcoal 6g daily

iii. Cholestyramine 5 g twice daily

iv. Naltrexone 50 mg daily

v. Ondansetron 4 mg twice daily

vi. Topical capsaicin (if localized)

vii. Encourage renal transplantation program enrollment


Management of pruritus associated with intrahepatic cholestasis

i. Cholestyramine 4 g once to three times daily

ii. Rifampin 300 – 450 mg daily

iii. Ultraviolet B (UVB) phototherapy twice weekly

iv. Naltrexone 50 mg daily

v. Ondansetron 8 mg twice daily

vi. Encourage liver transplantation program enrollment


Management of pruritus associated with polycythemia vera

i. Phlebotomy or chemotherapy to reduce red cell mass

ii. Cyproheptadine 4 mg three times daily

iii. Cimetidine 300 three times daily

iv. Pizotifen 0.5 mg three times daily

v. Cholestyramine 4 mg three daily

  1. Paraneoplastic Dermatoses
    1. Acanthosis palmaris, Acanthosis nigricans
    2. Acquired ichthyosis, Amyloidosis
    3. Bazex' syndrome, Carcinoid
    4. Clubbing, Coagulopathies
    5. Cryoglobulinaemia, Cronkhite-Canada syndrome
    6. Cushing's, Dermatomyositis
    7. Erythema gyratum repens. Erythroderma
    8. Hyertrichosis lanuginosa, Leser-Trelat sign
    9. Pemphigus, Sweets syndrome
    10. Necrolytic migratory erythema
  2. Important Topic
    1. Acanthosis nigricans can occur in obesity, hyperinsulinaemia, Cushing's syndrome and adenocarcinoma of the stomach. Several features help to distinguish the benign type from the malignant form. In the malignant form, there are mucosal involvement, pachydermatoglyphy and the disease tends to be more extensive. Besides gut tumours, others known neoplastic associations include lung, breast, uterus, ovary and prostate.
    2. Leser-Trelat sign is the sudden appearance of multiple seborrhoeic keratoses in association with an underlying malignancy. Leser-Trelat and acanthosis nigricans can occur together and they may share similiar aetiology.
    3. Cushing's syndrome can occur either due to excessive ACTH production by a pituitary tumour or as a result of ectopic ACTH production. Small cell carcinoma of the bronchus, pancreatic tumour and carcinoid tumour can all produce ectopic ACTH.
      Common features seen in ectopic ACTH production includes marked muscle wasting, severe hyperpigmentation and hypokalaemia.
    4. Carcinoid tumours are neoplasm that derived from amine precursor uptake and decarboxylation (APUD) cells. These cells produce a wide range of chemical mediators that include serotonin, histamine and substance P. Gut and lung are the main site of their origin. The main symptoms are flushing, diarrhoea, abdominal pain and wheezing.
    5. Dermatomyositis is most commonly associated with carcinoma of the breast and bronchus in the western world. Interesting in our local environment, nasopharyngeal carcinoma is more commonly associated with dermatomyositis.
    6. Paraneoplastic pemphigus is an recently discovered phenomenon characterised by the development of erythema multiforme like erosion and extensive mucosal involvement in patients with an underlying neoplasm. Lymphoreticular malignancy were the most commonly associated neoplasm.
  3. Environmntal Carcinogens
    1. Nicotine stain is undoubtedly the most commonly seen cutaneous sign of carcinogen exposure.
    2. Arsenic exposure can increase the risk of internal malignancy such as lung, bladder, kidney and prostate. Signs of previous arsenic exposure include hyperkeratotic lesions in palms and soles, diffuse truncal hyperpigmentation and multiple squamous cell carcinomas.
  4. Lipoid Proteinosis
    1. Lipoid proteinosis is a rare disorder characterized by infiltrate of hyaline material into the skin, oral cavity and internal organs
    2. Autosomal recessive disorder
    3. The earliest manifestation is hoarseness that develops in infancy, which can progress to complete aphonia without breathing difficulty. Examination of larynx frequently reveals infiltration, thickening and nodularity of cords. The mucosa of lip, pharynx and tongue may also develop firm yellow nodules giving if a cobblestone appearance. The tongue and firm on palpation with decreased range of movements. Ankyloglossia can occur due to involvement of the lingual frenulum
    4. Deposition of hyaline material along the eyelid margins results in typical beaded papules – ‘moniliform blepharosis’. This eyelid is the single most typical clinical feature of this disease. In later stages, there may be fetal loss of eyelashes. Further infiltration of eyelids leads to its malfunction and can cause corneal ulcer
    5. Neurological abnormalities include calcification in the hippocampus and falxcerebri and temporal lobe epilepsy
    6. The nature of hyaline material and underlying metabolic defect is unknown. It may represent an underlying lysosomal storage disorder with single or multiple enzyme defects, others have postulated it to be a disorder of collagen metabolism or collagen synthesis
    7. There is presently no effective therapy for lipoid proteinosis. Hoarseness may be relieved temporarily by surgical removal of vocal cord infiltrates. Facial lesions may be treated by dermabrasion, chemical skin peeling and blepharoplasty. Treatment by oral dimethyl sulphoxide has been claimed to the successful
  5. Pellagra
    1. Pellagra is a nutritional disorder that occurs due to niacin deficiency. The term pellagra is derived from the Italian words ‘pelle agra’ meaning rough skin
    2. It is due to poor nutrition and intake of certain cereals such as a  maize and jowar (Indian millet)
    3. Pellagra is a clinical syndrome characterized by
      1. symmetrical photosensitive skin eruption
      2. gastrointestinal manifestations
      3. neurological and psychiatric disturbances. There well known group of symptoms are traditionally remembered as pellagra’s four D’s – dermatitis – diarrhea – dementia and when untreated, death – is very seldom seen. But most of the manifestations are borderline and/or less typical in nature.
    4. The initial manifestation is an erythematous, photosensitive pruritic rash that occurs on the dorsa of hands
    5. The usual sites affected are the face, neck and dorsal surfaces of hands, arms and feet
    6. The dorsa of the hands are the most frequent site from where it may extend upward to form the ‘glove’ or ‘gauntlet’ of pellagra. The dermatosis is strikingly symmetrical and clearly demarcated from the normal skin
    7. The feet is commonly involved and it may also affect the front and back of the leg to form a boot
    8. In the face, and erythematous rash extending from the nose to the cheeks, chins, lips, may resemble lupus erythematosus “Butterfly rash”. Rarely eyelids and ears may be affected. Facial rash usually occurs concurrently with lesions elsewhere.
    9. This eruption forms a broadband or collar around the neck, known as Casal’s ‘necklace’. In many instance, the necklace has an anterior continuation, also known as ‘Cravat’
  6. Aribofalvinosis (Oro-Oculo-Genital Syndrome)
    1. This syndrome occurs due to vitamin B2 (Riboflavin) deficiency and is characterized by mucocutaneous lesions of angular stomatitis, cheilosis, glossitis, seborrhoeic dermatitis (especially around the nose), scrotal and vulvar dermatitis and increased corneal vascularity
    2. It usually responds prompty and dramatically to riboflavin supplementation with 5 to 10 mg of riboflavin daily
  7. Acrodermatitis Enteropathica
    1. Acrodermatitis eneteropathica, an autosomal recessive disorder which appears to be due to defective absorption of zinc from the gastrointestinal tract
    2. It may be a presenting sign in cystic fibrosis or AIDS
    3. It manifests insidiously between the ages three weeks and 18 months (often when the baby is switched from breast milk to cow’s milk) with periorificial (mouth, nose, eyes, ears, and perineum) and acral (extensor surfaces of the major joints, fingers, and toes) dermatitis, alopecia and diarrhoea
    4. The primary cutaneous eruption is vesicobullous, which is symmetrical and grouped. The lesions soon evolve into erosive vesicobullous eruption or psoriasiform patches
    5. At the same time or shortly afterward, loss of hair and gastrointestinal disturbances manifested chiefly by diarrhoea, occur
    6. Mental depression, listlessness, loss of appetite, perleche and blepharitis may occur during exacerbations
    7. Laboratory verification of deficient plasma or serum zinc levels may be undertaken where facilities exist, otherwise, all these cases respond to zinc sulphate/gluconate.

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