Periarticular lesions in the skin
Causes of different nail colours
The onset of the diseases of pregnancy
Questions will be asked on the following:
Ochronosis
Mafucci's Syndrome
Goltz Syndrome
Bullous Pemphigoid
Cheilitis Granulomatosis
Proteus syndrome
Oral hairy leukoplakia
Plasma cell vulvitis
Linear Atrophy, Telangiectases, and Soft Nodules Along the Lines of Blaschko With Skeletal Abnormalities
Periarticular lesions in the skin Heberden's nodes, Gottron's papules, Gout, GA, Rheumatoid nodules, Myxoid cyst, Ganglion, Giant cell tumor of tendon sheath, Multicentric reticulohistiocytosis, Erythema elevatum diutinum, Pachydermodactyly
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| This is Goltz syndrome Seen in females Linear telangiectasia |
Causes of Different Nail Colours
Dark nails can occur in acanthosis nigricans, Addison's Disease, Peutz Jegher's Syndrome, Vitamin B12 deficiency and drugs such as phenolthalein, anti malarials and gold.
Melanonychia
Chemotherapeutic agents (doxorubicin, daunorubicin, 5-fluorouracil)
Psoralens
Zidovudine
Discoloration (non-melanin) Minocycline
Antimalarials
Gold
Nevi, Melanoma, Racial and Laugier Hunziger syndrome
Blue nails can occur with the anti malarial drugs Minocycline, also hemochromatosis, ochronosis and exposure to silver nitrate and also in Wilson's Disease.
Drug-induced nail changes usually involve several or all nails. The pathogenesis is most often toxic in nature . Cancer chemotherapeutic agents, due to their cytotoxic properties, are the most frequent cause of nail changes. These include Beau's lines, onychomadesis, fragility, pigmentation, onycholysis, paronychia and vascular problems including subungal hemorrhages, hematomas and ischemia.
Oral retinoids are frequently responsible for nail abnormalities, especially nail fragility, paronychia and pyogenic granulomas. Antiretroviral drugs often cause nail pigmentation, paronychia and pyogenic granulomas. Although uncommon, photo-onycholysis is a characteristic side effect of tetracyclines and psoralens.
The Diseases of Pregnancy
Pruritic urticarial papules and plaques in pregnancy typically begins late in the third trimester, with lesions beginning around the umbilicus and the stretch marks.
Atlas images of PUPPS
Herpes gestationis or bullous pemphigoid of pregnancy starts in the late 2nd trimester.
Impetigo herpetiformis or pustular psoriasis of pregnancy is usually in the 3rd trimester and may have a high maternal mortality rate.
Prurigo gestationis. There is an early form that affects the upper trunk and the proximal limbs and a late form that affects the lower abdomen and may be similar to pupps.
Papular dermatitis of pregnancy presents as papules with crusts which are very itchy and it may occur at any time during pregnancy. Usually seen in pregnant patients with an atopic diathesis. Better described as atopic eruption of pregnancy.
Video of Zinc Def to Oral Hairy Leukoplakia
Blue nails can occur with the anti malarial drugs Minocycline, also hemochromatosis, ochronosis and exposure to silver nitrate and also in Wilson's Disease.
Drug-induced nail changes usually involve several or all nails. The pathogenesis is most often toxic in nature . Cancer chemotherapeutic agents, due to their cytotoxic properties, are the most frequent cause of nail changes. These include Beau's lines, onychomadesis, fragility, pigmentation, onycholysis, paronychia and vascular problems including subungal hemorrhages, hematomas and ischemia.
Oral retinoids are frequently responsible for nail abnormalities, especially nail fragility, paronychia and pyogenic granulomas. Antiretroviral drugs often cause nail pigmentation, paronychia and pyogenic granulomas. Although uncommon, photo-onycholysis is a characteristic side effect of tetracyclines and psoralens.
The Diseases of Pregnancy
Pruritic urticarial papules and plaques in pregnancy typically begins late in the third trimester, with lesions beginning around the umbilicus and the stretch marks.
Atlas images of PUPPS
Herpes gestationis or bullous pemphigoid of pregnancy starts in the late 2nd trimester.
Impetigo herpetiformis or pustular psoriasis of pregnancy is usually in the 3rd trimester and may have a high maternal mortality rate.
Prurigo gestationis. There is an early form that affects the upper trunk and the proximal limbs and a late form that affects the lower abdomen and may be similar to pupps.
Papular dermatitis of pregnancy presents as papules with crusts which are very itchy and it may occur at any time during pregnancy. Usually seen in pregnant patients with an atopic diathesis. Better described as atopic eruption of pregnancy.
Video of Zinc Def to Oral Hairy Leukoplakia
Zinc deficiency
may be genetic or acquired. The genetic form is a rare autosomal recessive disorder known as Acrodermatitis enteropathica, caused by a specific defect in the intestinal absorption of zinc.
The acquired form is caused by deficient intake of zinc, a high fiber content of food (phytate) that interferes with zinc absorption, malabsorption syndromes (including cystic fibrosis) and is associated with chronic renal failure, malignancy, drugs, alcoholism, HIV infection, pregnancy, and total parenteral nutrition; the latter has decreased in frequency since its recognition. Zinc deficiency may be associated with PEM. Acquired zinc deficiency is also endemic in some Middle Eastern countries .
In the genetic form, clinical signs of zinc deficiency usually occur 1 to 2 weeks after weaning. In the acquired form clinical signs may be acute or chronic depending on the associated cause. Dermatitis, diarrhea, and alopecia characterize the classic presentation of zinc deficiency. Dermatitis has a periorificial and acral distribution , and it may be erythematous, vesiculobullous with exudation and crusting, or lichenified with psoriasiform plaques in chronic cases. Secondary superinfection with Candida species and staphylococci is very common. Nail changes include a pustular paronychia with subsequent nail dystrophy. Hair is fine and sparse, and its growth ceases eventually leading to total alopecia. Photophobia, blepharitis, conjunctivitis, stomatitis, and cheilitis are frequent findings.
Diarrhea with steatorrhea may precede or develop subsequent to the cutaneous findings. Other clinical signs and symptoms include failure to thrive, apathy, irritability, ataxia, immune abnormalities, poor wound healing and, in adolescents, short stature and delayed puberty (endemic form).
These are some pictures of Acrodermatitis enteropathica
Proteus Syndrome
This is a hamartomatous disorder showing hypertrophy of limbs, hands and feet with the skin findings of hyperpigmentation, port wine stains and cerebriform thickening of the soles of the feet. They also may have giant lipomas, macrodactyly and macroglossia. Image
Oral hairy leukoplakia
This distinctive condition was first described in HIV cases but was subsequently seen in patients with leukaemic disorders and in transplant patients. It is seen mainly on the sides of the tongue but can occur elsewhere in the mouth. It is a manifestation of EBV infection in an immunocompromised host.
In HIV cases the lesions resolve when HAART is instituted. Otherwise Famvir can be used.
Video of Ochronosis to Inherited Amyloidosis
Ochronosis
Pigmentation of cartilage and ultimately the dermis giving a brownish pigmentation of the skin. Homogentisic acid is an excess in the blood giving alcaptonuria.
eMedicine on Ochronosis GSA Images Ochronosis
At what age do the clinical features of ochronosis become visible?
What effect does renal disease have on this?
The cutaneous manifestations of ochronosis are generally not detected until the individual reaches the third or fourth decade of life. Renal disease, inhibiting the excretion of homogentisic acid, however, may result in an accelerated development of the cutaneous features. Although generally insidious, homogentisic acid pigmentation may occur around sebaceous and sweat gland orifices. A bluish-black discoloration of skin over cartilage surfaces and tendons may become prominent with time. For example, sebaceous gland secretion of homogentisic acid may produce dark cerumen or axillary staining. The cartilage of the ear and nose may show a bluish-gray tinge. Calcification may occur. The deposition of the homogentisic acid may produce a degeneration of the tympanic membrane and ossicles, resulting in tinnitus and deafness. The cartilage deposition of homogentisic acid, with time, becomes more and more apparent. The areas of hyperpigmentation are characteristically the tip of the nose and the cartilage of the ear. The sclera may demonstrate a grayish-black pigmentation anterior to the horizontal recti muscle insertion. With time, pigment deposits may be seen adjacent to the limbus. occurring in third or fourth decade Staining of cerumen or axillary region Pigmentation on tip of nose and ears.
See image below in context
Mafucci's Syndrome
These occur as hemangiomas with dyschondroplasia and fragility of bones. They are susceptible to chondrosarcomas.
Inherited forms of Amyloidosis
Muckle Wells Syndrome - urticaria, deafness and amyloid.
Familial Mediterranean fever presents with erysipelas of the legs and also there is amyloid with upper and lower limb neuropathy. What is the nature of the amyloid fibril in these conditions?
The amyloid fibril in secondary amyloidosis, occurring with chronic infections (eg, tuberculosis, osteomyelitis), familial Mediterranean fever, and a syndrome characterized by urticaria, deafness, and nephropathy (Muckle-Wells syndrome) is a proteolytic cleavage product of the acute phase reactant protein SAA.
Secondary Amyloidosis
Secondary cutaneous amyloidosis refers to secondary, often clinically inapparent, deposits that are seen within skin tumors including dermatofibromas, intradermal melanocytic nevi, seborrheic keratoses, pilomatricomas, sweat gland tumors, basal cell carcinomas, Bowen's disease, porokeratosis and trichoepitheliomas. It has also been reported following PUVA therapy.
The image below is of lichen amyloidosis
The following are comments I made after a slide test I was given by Ken Paver on some of the ones I got wrong
PCT - watch this usually back of hand, nail involvement, scars, may look lichenoid.
Molluscum contagiosum - watch for the delling.
Syphilitic gumma - dusky lesion on leg, difficult to evaluate.
Dermatitis Artefacta - this is a case of mutilation on the penis. If an unusual picture, especially ulceration in an unusual area, think of artefacta.
Ichthyosis Linearis Circumflexa - watch for the double edge scale. This can also be found in some lesions of discoid eczema.
Erythema Nodosum Leprosum - this can look like a thrombophlebitis or even a deep fungal infection.
Pemphigus erythematosus -may look like mycosis fungoides when viewed on the back but watch for the crusting as well.
Ochronosis on the eye can look very much like a melanoma.
Arsenical keratoses - watch these , punctate, dell like depression when they are picked off. They may resemble verrucae on the hands and feet.
Pigmentation of cartilage and ultimately the dermis giving a brownish pigmentation of the skin. Homogentisic acid is an excess in the blood giving alcaptonuria.
eMedicine on Ochronosis GSA Images Ochronosis
At what age do the clinical features of ochronosis become visible?
What effect does renal disease have on this?
The cutaneous manifestations of ochronosis are generally not detected until the individual reaches the third or fourth decade of life. Renal disease, inhibiting the excretion of homogentisic acid, however, may result in an accelerated development of the cutaneous features. Although generally insidious, homogentisic acid pigmentation may occur around sebaceous and sweat gland orifices. A bluish-black discoloration of skin over cartilage surfaces and tendons may become prominent with time. For example, sebaceous gland secretion of homogentisic acid may produce dark cerumen or axillary staining. The cartilage of the ear and nose may show a bluish-gray tinge. Calcification may occur. The deposition of the homogentisic acid may produce a degeneration of the tympanic membrane and ossicles, resulting in tinnitus and deafness. The cartilage deposition of homogentisic acid, with time, becomes more and more apparent. The areas of hyperpigmentation are characteristically the tip of the nose and the cartilage of the ear. The sclera may demonstrate a grayish-black pigmentation anterior to the horizontal recti muscle insertion. With time, pigment deposits may be seen adjacent to the limbus. occurring in third or fourth decade Staining of cerumen or axillary region Pigmentation on tip of nose and ears.
See image below in context
Mafucci's Syndrome
These occur as hemangiomas with dyschondroplasia and fragility of bones. They are susceptible to chondrosarcomas.
Inherited forms of Amyloidosis
Muckle Wells Syndrome - urticaria, deafness and amyloid.
Familial Mediterranean fever presents with erysipelas of the legs and also there is amyloid with upper and lower limb neuropathy. What is the nature of the amyloid fibril in these conditions?
The amyloid fibril in secondary amyloidosis, occurring with chronic infections (eg, tuberculosis, osteomyelitis), familial Mediterranean fever, and a syndrome characterized by urticaria, deafness, and nephropathy (Muckle-Wells syndrome) is a proteolytic cleavage product of the acute phase reactant protein SAA.
Secondary Amyloidosis
Secondary cutaneous amyloidosis refers to secondary, often clinically inapparent, deposits that are seen within skin tumors including dermatofibromas, intradermal melanocytic nevi, seborrheic keratoses, pilomatricomas, sweat gland tumors, basal cell carcinomas, Bowen's disease, porokeratosis and trichoepitheliomas. It has also been reported following PUVA therapy.
The image below is of lichen amyloidosis
The following are comments I made after a slide test I was given by Ken Paver on some of the ones I got wrong
PCT - watch this usually back of hand, nail involvement, scars, may look lichenoid.
Molluscum contagiosum - watch for the delling.
Syphilitic gumma - dusky lesion on leg, difficult to evaluate.
Dermatitis Artefacta - this is a case of mutilation on the penis. If an unusual picture, especially ulceration in an unusual area, think of artefacta.
Ichthyosis Linearis Circumflexa - watch for the double edge scale. This can also be found in some lesions of discoid eczema.
Erythema Nodosum Leprosum - this can look like a thrombophlebitis or even a deep fungal infection.
Pemphigus erythematosus -may look like mycosis fungoides when viewed on the back but watch for the crusting as well.
Ochronosis on the eye can look very much like a melanoma.
Arsenical keratoses - watch these , punctate, dell like depression when they are picked off. They may resemble verrucae on the hands and feet.
Lymphangioma circumscriptum - look for the frogspawn. Small vesicles on a normal base, possibly in unusual areas such as around the umbilicus.
Watch erythrasma. I always think it is tinea but the edge is different, it is more diffuse and you dont get the central clearing you get with a tinea cruris.
Whenever you think something is pemphigus foliaceous think of Hailey Hailey.
Chronic mucocutaneous candidiasis. The nails are the giveaway. Severe swelling and thickening and scaling.
Extra genital lichen sclerosis et atrophicus often starts around the umbilicus.
The image below shows the nail involvement in chronic mucocutaneous candidiasis
Question 1. What diseases are made better by raising the arms?
Question 2. What conditions have horizontal folds on the face?
Watch erythrasma. I always think it is tinea but the edge is different, it is more diffuse and you dont get the central clearing you get with a tinea cruris.
Whenever you think something is pemphigus foliaceous think of Hailey Hailey.
Chronic mucocutaneous candidiasis. The nails are the giveaway. Severe swelling and thickening and scaling.
Extra genital lichen sclerosis et atrophicus often starts around the umbilicus.
The image below shows the nail involvement in chronic mucocutaneous candidiasis
Question 1. What diseases are made better by raising the arms?
Question 2. What conditions have horizontal folds on the face?
Answer 1 . Fabry's disease, Thoracic outlet compression syndrome
Answer 2.: Mycosis fungoides, leprosy, lichen myxedema, Sezary's Syndrome, amyloidosis, chronic leukaemia, actinic reticuloid and multicentric reticulo histiocytosis and tuberculosis.
Note that these all have vertical folds.
Answer 2. Cutis verticis gyrata, acanthosis nigricans, primary pachyderm periostosis, acromegaly, Rosenthal-Kloepfer Syndrome and Leri's Syndrome.
Answer 2.: Mycosis fungoides, leprosy, lichen myxedema, Sezary's Syndrome, amyloidosis, chronic leukaemia, actinic reticuloid and multicentric reticulo histiocytosis and tuberculosis.
Note that these all have vertical folds.
Answer 2. Cutis verticis gyrata, acanthosis nigricans, primary pachyderm periostosis, acromegaly, Rosenthal-Kloepfer Syndrome and Leri's Syndrome.
