Search eMedicine Topics covered in this tutorial
Causes of localised and generalised hyperpigmentation.
A newborn with hard firm skin
Conditions with a scaly serpiginous edge
Ichthyosis and mental retardation
Nodules in vets and farm workers.
Questions will be asked on the following topics:
Acrodermatitis enteropathica
Monilethrix and
Giant Condyloma of Buschke
Generalised and Localised Hyperpigmentation
The mnemonic to use for this is DAMN and ANNE.
For generalised hyperpigmentation it is DAMN with D for drugs, such as Bleomycin, Gold, Arsenic and Cyclophosphamide. A for autoimmune - for example lupus dermatomyositis and scleroderma, M for metabolic - particularly hemochromatosis, renal failure, hepatic failure, malabsorption, PCT and amyloid, N for neoplastic - particularly lymphoma, melanoma and carcinoma and E for endocrine - particularly Addison's Disease, acromegaly, thyrotoxicosis and phaeochromocytoma.
Localised hyperpigmentation use the mnemonic ANNE. The autoimmune being the same as generalised, neoplastic also, another N for nutritional - for example pellagra and malabsorption and E for endocrine as in generalised.
Sometimes it is easier to consider what causes skin hyperpigmentation and work back from there. eg Melanin in melanomas and post inflammatory hyperpigmentation, iron in blood in the pigmented purpuric dermatoses, drugs and chemicals as in argyria and excess stimulation of melanocytes from ACTH and other hormones in the endocrine disorders.
eMedicine-Incontinentia pigmenti
eMedicine-Dermatopathia Pigmentosa Reticularis
eMedicine-Pellagra
Cutaneous reactions to cytotoxic drugs Acral erythema and peeling is one of the commonest cutaneous reactions occurring with cytotoxic drugs. See link for some of the newer biologics.
The image below is a plant contact dermatitis Lime Phytophoto dermatitis
Newborn with Hard, Firm Skin
The three things to consider are neonatal cold injury, sclerema of the newborn and subcutaneous fat necrosis of the newborn.
Neonatal cold injury
Babies are usually healthy. It occurs within the first 24 hours. The skin is red or purple with pitting oedematous extremities. The rest of the body is white and cold. There can be a 25% mortality mainly from pulmonary haemorrhage.
Sclerema of the Newborn
It is usually a premature baby who is ill. It occurs on the buttocks and trunk but spreading then peripherally. Compare this with neonatal cold injury. There is no pitting. The skin has a yellow white colour. The palms and soles and the genitalia are usually spared. The onset is early. There is a 50% mortality.eMedicine-Sclerema neonatorum
Subcutaneous Fat Necrosis of the Newborn
Again the baby is usually healthy. The onset is in the first 2 to 20 days with variable size and number of violaceous nodules. Some babies have hypercalcemia and the complications of this. Histology will often show granulomatous inflammation in the fat whereas the histology of sclerema of the newborn shows very little. Generally this condition will clear up on its own. Some nodules may rupture and scar.
Localised hyperpigmentation use the mnemonic ANNE. The autoimmune being the same as generalised, neoplastic also, another N for nutritional - for example pellagra and malabsorption and E for endocrine as in generalised.
Sometimes it is easier to consider what causes skin hyperpigmentation and work back from there. eg Melanin in melanomas and post inflammatory hyperpigmentation, iron in blood in the pigmented purpuric dermatoses, drugs and chemicals as in argyria and excess stimulation of melanocytes from ACTH and other hormones in the endocrine disorders.
eMedicine-Incontinentia pigmenti
eMedicine-Dermatopathia Pigmentosa Reticularis
eMedicine-Pellagra
Cutaneous reactions to cytotoxic drugs Acral erythema and peeling is one of the commonest cutaneous reactions occurring with cytotoxic drugs. See link for some of the newer biologics.
The image below is a plant contact dermatitis Lime Phytophoto dermatitis
Newborn with Hard, Firm Skin
The three things to consider are neonatal cold injury, sclerema of the newborn and subcutaneous fat necrosis of the newborn.
Neonatal cold injury
Babies are usually healthy. It occurs within the first 24 hours. The skin is red or purple with pitting oedematous extremities. The rest of the body is white and cold. There can be a 25% mortality mainly from pulmonary haemorrhage.
Sclerema of the Newborn
It is usually a premature baby who is ill. It occurs on the buttocks and trunk but spreading then peripherally. Compare this with neonatal cold injury. There is no pitting. The skin has a yellow white colour. The palms and soles and the genitalia are usually spared. The onset is early. There is a 50% mortality.eMedicine-Sclerema neonatorum
Subcutaneous Fat Necrosis of the Newborn
Again the baby is usually healthy. The onset is in the first 2 to 20 days with variable size and number of violaceous nodules. Some babies have hypercalcemia and the complications of this. Histology will often show granulomatous inflammation in the fat whereas the histology of sclerema of the newborn shows very little. Generally this condition will clear up on its own. Some nodules may rupture and scar.
eMedicine-Subcutaneous Fat necrosis of the Newborn
An infant with firm fixed plaques
The image below is sclerema neonatorum
Conditions with a scaly serpiginous edge
Necrolytic migratory erethyma
Subcorneal pustular dermatosis
Hailey Hailey Disease, and
eMedicine-Acrodermatitis enteropathica
Periorificial dermatitis and irritability in an infant
The image below is acrodermatitis enteropathica
Glomus Tumours
The solitary glomus tumour is a small blue red papular nodule that may be soft or firm but is usually painful. Temperature changes can cause pain to occur. It is commonly seen in the nail beds, however it has also been reported on the neck, face, penis and scrotum.
In contrast, multiple Glomus tumours are usually asymptomatic but occasionally are painful and they are typically found in children. They can be inherited in an autosomal dominant manner. The Kasabach-Merritt Syndrome (consumption coagulopathy) can be seen rarely in patients with generalised multiple Glomus tumours. Histologically there are fewer glomus cells in multiple Glomus tumours and they are not encapsulated.
eMedicine-Glomus tumour
Diffuse and progressive nodular plaques
Monilethrix
In monilethrix the lanugo hair at birth may be normal but is soon replaced by dry brittle lustreless hair which breaks easily. If the hair shafts are examined they show nodes 1mm apart with intervening tapered non-medullated constrictions. A string of beads appear and this is what gives the condition its name. Monile is the Latin for necklace and thrix is the Greek for hair. People with this condition seldom need a haircut because the hair breaks. There is often associated follicular hyperkeratosis and keratosis pilaris of the scalp and arms and cheeks can also be seen. The condition is inherited as an autosomal dominant.
Pseudo monilethrix
Pseudo monilethrix shows nodes as well, but these are actually indentations in the shaft of the hair. These indentations occur at irregular intervals. It is thought to be due to inadvertent mechanical pressure. A hair mount showed the irregular nodes in pseudo monilethrix.
eMedicine-Monilethrix
Unraveling the genetics of hair and nail genodermatoses
eMedicine-Giant condyloma of Buschke
An ulcerating verrucous plaque on the foot
The image below is Giant condyloma of Buschke
Question 1. What pigmentation pattern may occur with Doxorubicin?
Question 2. What 2 important cutaneous reactions can occur with Hydroxyurea?
Question 3. What type of pigmentation can occur with Bleomycin?
Question 4. What are the histology findings in sclerema neonatorum?
Question 5. Is Necrolytic migratory erythema always seen with a glucagonoma?
Question 6. In Netherton's syndrome the hair and skin abnormalities may not develope until after the first year of life.What hairs may give you the diagnosis first?
Question 7. Can acrodermatitis enteropathica be seen in full term breast fed babies?
Question 8. Multiple glomangiomas occur at an earlier age than solitary lesions T or F?
Answer 1. melanotic macules may appear on the trunk or extremities.
Answer 2. Leg ulcers and lichenoid poikilodermatous reactions.Also dermatomyositis like on the dorsum of the hands.
Answer 3. Flagellate pigmentation.
Answer 4. Oedema, a thickening of the subcutaneous fibrous septa, and a radial array of fine needlelike clefts in the fat cells. The fat cells often are enlarged. In contrast to subcutaneous fat necrosis of the newborn, no fat necrosis or inflammation exists.
Answer 5. No. Cases have been seen in the abscence of a glucagonoma but in association with chronic liver disease, chronic pancreatitis, traumatic necrotizing pancreatitis, celiac disease, and jejunal adenocarcinoma.
Answer 6. The eyebrow hairs.Reference
Answer 7. Yes There may be low zinc levels in some mother's breast milk.
Answer 8. True Multiple glomangiomas present at an earlier age than do solitary lesions, with one third presenting before the age of 20 years. Solitary tumors occur equally in both sexes, whereas multiple tumors are more frequent in males.
An infant with firm fixed plaques
The image below is sclerema neonatorum
Conditions with a scaly serpiginous edge
Necrolytic migratory erethyma
Subcorneal pustular dermatosis
Hailey Hailey Disease, and
Pemphigus Foliaceus Image
Larva migrans gives a very typical serpiginous rash but it is not scaly Image
Recurrent annular erythematous scaly patches
The image below is necrolytic migratory erythema
Nodules in Vets and Farm Workers
Orf. Orf is a red papule initially but it is umbilicated later. It may be target like and pseudo bullous.
Recurrent annular erythematous scaly patches
The image below is necrolytic migratory erythema
Nodules in Vets and Farm Workers
Orf. Orf is a red papule initially but it is umbilicated later. It may be target like and pseudo bullous.
Milker's nodules. This condition goes through the same developmental stages as Orf before resolving over a five to six week period. It can be difficult to tell the two apart clinically, and
Cowpox presents as vesicles and umbilicated pustules. Person to person contact is possible and it does give protection against smallpox. Image
Consider also deep fungal infections and atypical mycobacterial infections.
Cutaneous manifestations of biological warfare agents
The image below is of multiple orf
Ichthyosis with Mental Retardation
Netherton's Syndrome. This gives an annular rash called ichthyosis linearis circumflexa. This causes a double edged scale. It also gives bamboo hairs , a hair shaft abnormality, called Trichorrhexis Invaginata.
eMedicine-Netherton's syndrome Image
Down's Syndrome with X linked ichthyosis and ichthyosis vulgaris.
Sjogren-Larssen. This gives an ichthyosiform erythroderma.eMedicine-Sjogren-Larsson syndrome
Rudd's Syndrome which also gives an ichthyosiform erythroderma and
Conradi's Syndrome which gives a whorled ichthyosis but has an ichthyosiform erythroderma at birth. Look also for stippling of the bony epiphyses.
Abnormal hair growth in a child with atopy Neonatal and infantile erythrodermas
The image below is of ichthyosis linearis circumflexa
Consider also deep fungal infections and atypical mycobacterial infections.
Cutaneous manifestations of biological warfare agents
The image below is of multiple orf
Ichthyosis with Mental Retardation
Netherton's Syndrome. This gives an annular rash called ichthyosis linearis circumflexa. This causes a double edged scale. It also gives bamboo hairs , a hair shaft abnormality, called Trichorrhexis Invaginata.
eMedicine-Netherton's syndrome Image
Down's Syndrome with X linked ichthyosis and ichthyosis vulgaris.
Sjogren-Larssen. This gives an ichthyosiform erythroderma.eMedicine-Sjogren-Larsson syndrome
Rudd's Syndrome which also gives an ichthyosiform erythroderma and
Conradi's Syndrome which gives a whorled ichthyosis but has an ichthyosiform erythroderma at birth. Look also for stippling of the bony epiphyses.
Abnormal hair growth in a child with atopy Neonatal and infantile erythrodermas
The image below is of ichthyosis linearis circumflexa
Acrodermatitis Enteropathica
This is a vesiculo bullous rash that may present just with crusting or a spreading scaly rash around the perineum and also around the mouth and cheeks. It usually occurs in premature babies or in other children at around weaning time. Paronychia may occur as well as loss of scalp hair, eyebrows and eyelashes. Secondary infection with staph aureus and candida is not uncommon. Acrodermatitis enteropathica can be an autosomal recessive disorder but obviously there is the acquired type as well. Clinically they appear the same. The differential diagnosis includes atopic dermatitis, psoriasis, seborrhoeic dermatitis, Kwashiorkor, cystic fibrosis, Glucagonoma Syndrome, essential fatty acid deficiencies, mucocutaneous candidiasis, histiocytosis X, neonatal lupus erythematosus, epidermolysis bullosa, biotin and multiple carboxylase deficiencies and other conditions causing acquired zinc deficiencies. This is an interesting differential diagnosis list and is well worth going through these conditions thinking about how their presentation would differ from that of acrodermatitis enteropathica.
This is a vesiculo bullous rash that may present just with crusting or a spreading scaly rash around the perineum and also around the mouth and cheeks. It usually occurs in premature babies or in other children at around weaning time. Paronychia may occur as well as loss of scalp hair, eyebrows and eyelashes. Secondary infection with staph aureus and candida is not uncommon. Acrodermatitis enteropathica can be an autosomal recessive disorder but obviously there is the acquired type as well. Clinically they appear the same. The differential diagnosis includes atopic dermatitis, psoriasis, seborrhoeic dermatitis, Kwashiorkor, cystic fibrosis, Glucagonoma Syndrome, essential fatty acid deficiencies, mucocutaneous candidiasis, histiocytosis X, neonatal lupus erythematosus, epidermolysis bullosa, biotin and multiple carboxylase deficiencies and other conditions causing acquired zinc deficiencies. This is an interesting differential diagnosis list and is well worth going through these conditions thinking about how their presentation would differ from that of acrodermatitis enteropathica.
eMedicine-Acrodermatitis enteropathica
Periorificial dermatitis and irritability in an infant
The image below is acrodermatitis enteropathica
Glomus Tumours
The solitary glomus tumour is a small blue red papular nodule that may be soft or firm but is usually painful. Temperature changes can cause pain to occur. It is commonly seen in the nail beds, however it has also been reported on the neck, face, penis and scrotum.
In contrast, multiple Glomus tumours are usually asymptomatic but occasionally are painful and they are typically found in children. They can be inherited in an autosomal dominant manner. The Kasabach-Merritt Syndrome (consumption coagulopathy) can be seen rarely in patients with generalised multiple Glomus tumours. Histologically there are fewer glomus cells in multiple Glomus tumours and they are not encapsulated.
eMedicine-Glomus tumour
Diffuse and progressive nodular plaques
Monilethrix
In monilethrix the lanugo hair at birth may be normal but is soon replaced by dry brittle lustreless hair which breaks easily. If the hair shafts are examined they show nodes 1mm apart with intervening tapered non-medullated constrictions. A string of beads appear and this is what gives the condition its name. Monile is the Latin for necklace and thrix is the Greek for hair. People with this condition seldom need a haircut because the hair breaks. There is often associated follicular hyperkeratosis and keratosis pilaris of the scalp and arms and cheeks can also be seen. The condition is inherited as an autosomal dominant.
Pseudo monilethrix
Pseudo monilethrix shows nodes as well, but these are actually indentations in the shaft of the hair. These indentations occur at irregular intervals. It is thought to be due to inadvertent mechanical pressure. A hair mount showed the irregular nodes in pseudo monilethrix.
eMedicine-Monilethrix
Unraveling the genetics of hair and nail genodermatoses
eMedicine-Giant condyloma of Buschke
An ulcerating verrucous plaque on the foot
The image below is Giant condyloma of Buschke
Question 1. What pigmentation pattern may occur with Doxorubicin?
Question 2. What 2 important cutaneous reactions can occur with Hydroxyurea?
Question 3. What type of pigmentation can occur with Bleomycin?
Question 4. What are the histology findings in sclerema neonatorum?
Question 5. Is Necrolytic migratory erythema always seen with a glucagonoma?
Question 6. In Netherton's syndrome the hair and skin abnormalities may not develope until after the first year of life.What hairs may give you the diagnosis first?
Question 7. Can acrodermatitis enteropathica be seen in full term breast fed babies?
Question 8. Multiple glomangiomas occur at an earlier age than solitary lesions T or F?
Answer 1. melanotic macules may appear on the trunk or extremities.
Answer 2. Leg ulcers and lichenoid poikilodermatous reactions.Also dermatomyositis like on the dorsum of the hands.
Answer 3. Flagellate pigmentation.
Answer 4. Oedema, a thickening of the subcutaneous fibrous septa, and a radial array of fine needlelike clefts in the fat cells. The fat cells often are enlarged. In contrast to subcutaneous fat necrosis of the newborn, no fat necrosis or inflammation exists.
Answer 5. No. Cases have been seen in the abscence of a glucagonoma but in association with chronic liver disease, chronic pancreatitis, traumatic necrotizing pancreatitis, celiac disease, and jejunal adenocarcinoma.
Answer 6. The eyebrow hairs.Reference
Answer 7. Yes There may be low zinc levels in some mother's breast milk.
Answer 8. True Multiple glomangiomas present at an earlier age than do solitary lesions, with one third presenting before the age of 20 years. Solitary tumors occur equally in both sexes, whereas multiple tumors are more frequent in males.
