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Follicular skin diseases

Hair problems case challenge 3

A 25-year-old woman is referred to the Skin Clinic because she has lost a substantial amount of hair from the vertex of her scalp. She has had a facial rash for several years, but recently it has become more prominent.

Which of the following descriptions of hair loss are most suitable? # Localised non-scarring alopecia #! Erythematous scaly plaques #! Localised cicatricial alopecia # Folliculitis Explanation: There is an ill-defined area of balding. The central area is hairless and shiny, and although this may not be clearly seen on the photograph, this is due to scarring. There are erythematous plaques on the periphery of the bald area.
Select the most appropriate description(s) for the rash on her face: # Sunburn #! Well demarcated erythematous plaques #! Scarring # Seborrhoeic distribution Explanation: She has well demarcated erythematous slightly scaly plaques in a photosensitive distribution. The loss of normal pigmentation is due to scarring.
Select the appropriate description of her fingernails: * Heberden’s nodes *! Cuticular telangiectasia * Onycholysis * Paronychia Explanation: The picture shows cuticular telangiectasia and slight swelling of the proximal nail folds without loss of cuticular adherence. The nail plates appear normal and there is no interphalangeal swelling (Heberden’s nodes, seen in osteoarthritis). Select the correct diagnosis: * Seborrhoeic dermatitis * Systemic lupus erythematosus * Subacute cutaneous lupus erythematosus *! Discoid lupus erythematosus (DLE) Explanation: The patient has discoid lupus erythematosus (DLE), the most common form of cutaneous lupus erythematosus. The cuticular telangiectasia may indicate the presence of systemic lupus (SLE), but may be seen in the absence of other features of internal disease. At which time of year do most patients with DLE present? *! October to December * January to March * April to June * July to September Explanation: Most patients with DLE present for medical consultation during the spring months as ultraviolet radiation levels rise. This precipitates onset or reactivation of disease.

Refer to your textbook or the emedicine dermatology page on DLE.

Which of the following antibodies are present in more than 5% of patients with this diagnosis? #! Antinuclear antibodies (ANA) # Anti-Ro (SS-A) antibodies # Anti-double-stranded DNA # Anti-mitochondrial antibodies Explanation: On serologic testing about 20% patients with DLE have a positive antinuclear antibody. Anti-Ro (SS-A) autoantibodies are present in approximately 1-3% of patients and anti-double-stranded DNA antibodies usually reflect SLE, and they may occur in some patients (<5%). Which of the following commonly accompany DLE without indicating the patient has SLE? * Renal disease * Malignant degeneration *! Arthralgia * Positive lupus band test Explanation: Renal disease indicates SLE. Malignant degeneration is rare, but may complicated severe scarring cutaneous disease. Arthralgia is not uncommon in those with severe DLE and does not necessarily indicate systemic disease. Direct immunofluorescence of lesional skin biopsy is positive in 90% of those with DLE, but a positive lupus band test (4 immunoreactants in the basement membrane zone of non-exposed non-lesional skin) suggests SLE. Which of the following possible treatments are recommended in this patient? # Cyclosporin #! Hydroxychloroquine #! Ultrapotent topical steroid cream #! Sun protection including sunscreen Explanation: First line treatment of DLE is sun protective measures, cosmetic camouflage and potent or ultrapotent topical steroids. Systemic treatment is necessary for patients with severe disease (for example hair loss, scarring, extensive rash). This may include antimalarials (usually hydroxychloroquine), systemic steroids, retinoids and immunosuppressive agents. Rarely, thalidomide is used; it can be extremely effective. She tells you that she may be pregnant. Which of the following possible drugs are Category X (contraindicated in pregnancy) or Category D (unsafe in pregnancy)? # Hydroxychloroquine #! Thalidomide # Oral prednisone #! Acitretin Explanation: Both thalidomide and acitretin are completely contraindicated in pregnancy (Category X) because they are known teratogens. Oral prednisone is permitted if necessary for severe inflammatory skin diseases. Antimalarials are of uncertain safety – they’ve been taken by many pregnant women without adverse effect. However the drug is deposited in fetal pigmented tissue. The risk of a flare-up of disease if the drug is discontinued may be more worrying than the risks of the drug.

Treatment with antimalarials is effective in most patients although short courses of systemic steroids are often required in severe cases to deal with flare-ups. The scalp is often more resistant to the treatment than facial skin. If the perifollicular erythematous scaly plaques respond, partial or complete regrowth of hair may occur. However if there is scarring, these sites remain bald.