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-Group of diseases in which hair follicles become blocked with keratin (scale) and then rupture, resulting
in inflammatory skin disease.
Hidradenitis suppurativa (acne inversa)\Acne conglobata (severe nodulocystic acne)
Dissecting cellulitis (perifolliculitis capitis abscedens et suffodiens)
A fourth condition, pilonidal sinus or pilonidal disease, makes up the follicular occlusion tetrad.
Hidradenitis suppurativa
Hidradenitis suppurativa is increasing referred to by the name acne inversa. It is a chronic inflammatory
skin condition in which there are painful boil-like lumps and draining sinus tracts (canals) that leave
unsightly scars. It affects the armpits, groin, genitals, buttocks and skin under the breasts.
Hidradenitis suppurativa is three times as common in women as in men; it most affects them between
puberty and menopause. Follicles are more likely to block up under the influence of hormones, especially
the male type (androgens). Levels of androgens may be normal or raised in women with hidradenitis
suppurativa. Hidradenitis suppurativa is much more common in smokers than non-smokers, and is also
more common in the obese compared to those of normal weight.
Treatment involves:
Various antiseptics and antibiotics
Retinoids such as acitretin and isotretinoin
Antiandrogens
Systemic corticosteroids
Surgery.
Acne conglobata
Acne conglobata is an uncommon and unpleasant form of nodulocystic acne in which there are
interconnecting abscesses and sinuses, which result in unsightly hypertrophic (thick) and atrophic
(thin) acne scars. There are groups of large macrocomedones and cysts that are filled with smelly pus.
Treatment involves:
Prolonged courses of tetracycline antibiotics
Oral isotretinoin
Sometimes, systemic corticosteroids.
Dissecting cellulitis
Dissecting cellulitis or folliculitis is also known as perifolliculitis capitis abscedens et suffodiens. It is a
rare and severe form of scalp folliculitis.
Large nodules and cysts accompany smaller follicular papules and pustules, from which purulent material
can be expressed. Temporary hair loss over the lesions eventually results in permanent scarring and bald
patches.
This condition is very resistant to treatment. The severity may be reduced with:
Oral isotretinoin
Antibiotics
Dapsone
Injected intralesional or systemic steroids.
si/sx: single glistening orange-red plaque. Patients may have no symptoms but they may
complain of tenderness and/or slight itchiness.
Ddx: pre-cancerous condition - Erythroplasia of Queyrat
Tx: topical steroid, topical abx, tacrolimus ointment or pimecrolimus cream, Imiquimod cream,
laser, circumcision
Bullous Pemphigoid
chronic, inflammatory, subepidermal, blistering disease
IgG immunoglobulins (antibodies) and activated T lymphocytes attack components of the
basement membrane
o associated with the hemidesmosomes: structures that ensure the epidermal keratinocyte
cells stick to the dermis to make a waterproof seal
If untreated, it can persist for months or years, with periods of spontaneous remissions and
exacerbations.
Si/ sx: In the most common presentation: crops of tense, fluid-filled, itchy bulla arise on any part
of the skin surface, with a predilection for the flexural areas of the skin. Other forms: vegetative,
acral, nodular, urticarial
Dx: Histology- split between the epidermis and the dermis. Direct immunofluorescence:
antibodies along the basement membrane that lies between the epidermis and dermis. Indirect
immunofluorescence test on blood serum: circulating pemphigoid antibodies.
Tx: Anti-inflammatory agents (eg, corticosteroids, tetracyclines, dapsone) and
immunosuppressants (eg, azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide)
Most patients affected with bullous pemphigoid require therapy for 6-60 months
Hutchinsons Sign
Melanonychia Striata
Accutane
** Because of isotretinoin's teratogenicity and to minimize fetal exposure, isotretinoin is approved for
marketing only under a special restricted distribution program approved by the Food and Drug
Administration. This program is called iPLEDGE.
The iPLEDGE program requires the following:
All female patients of childbearing potential must select and commit to the use of two forms of
birth control for at least one month prior to starting isotretinointherapy, during therapy, and for one
month after therapy. The forms of contraception that meet these requirements are specified by the
iPLEDGE program.
All female patients who can become pregnant must have two negative urine or blood pregnancy
tests (with a sensitivity of at least 25 mIU/mL) before receiving the initial prescription. The second
pregnancy test must be conducted in a CLIA-certified laboratory. For each month of therapy and one
month after completing therapy, female patients must return to their clinicians for evaluation,
counseling, education, and a pregnancy test conducted by a CLIA-certified laboratory.
For female patients who can become pregnant, prescribing clinicians must, on a monthly basis,
document in the iPLEDGE system the results of the pregnancy test as well as report the two forms
of birth control being used by the patient. The clinician must also confirm that the patient has
received counseling and education. For all other patients, prescribers must document that the patient
was counseled on the iPLEDGE program requirements, which include knowledge of the birth
defects that may result from the use of isotretinoin by females of child-bearing potential.
As part of this system, clinicians must certify expertise in the diagnosis and treatment of acne, and
knowledge of the risk and severity of birth defects withisotretinoin
MOA: 1) shrinkage of sebaceous glands 2) decrease of sebum secretioninhibition of the sebumdependent bacterium Propionibacterium acnes 3) inhibits comedogenesis by fostering keratinocyte
differentiation and by normalizing desquamation.
Dosing
Acne, severe recalcitrant nodular (acne with many (greater than 5 mm) inflammatory nodules): Oral:
0.5-1 mg/kg/day in 2 divided doses for 15-20 weeks; may discontinue earlier if the total cyst count
decreases by 70%. Adults with very severe disease/scarring or primarily involves the trunk may
require dosage adjustment up to 2 mg/kg/day. A second course of therapy may be initiated after a
period of 2 months off therapy. A dose of 0.5 mg/kg/day may be used to minimize initial flaring
(Strauss, 2007).
Acne, moderate (unlabeled use): Oral: 20 mg/day (~0.3-0.4 mg/kg/day) for 6 months (Amichai, 2006)
Administration: Absorption of isotretinoin is improved when taken with food (especially high-fat
meals); thus, administration during meals is recommended.
Use
Treatment of severe recalcitrant nodular acne unresponsive to conventional therapy
Use - Unlabeled
Management of moderate degrees of treatment-resistant acne, management of acne that produces physical
or psychological scarring; treatment of cutaneous T-cell lymphomas (mycosis fungoides and Szary
syndrome); prevention of squamous cell skin cancers (in high-risk patients); treatment of high-risk
neuroblastoma in children
.
side effects
Myalgias
Decreased night vision, corneal opacities, hepatotoxicity, and bone marrow suppression may occur
infrequently.
Hypertriglyceridemia occurs in up to 45 percent of patients on isotretinoin therapy and elevations
of total cholesterol and low-density lipoprotein are seen in approximately 30 percent
idiopathic intracranial hypertension (pseudotumor cerebri). avoid tetracycline
Diffuse skeletal hyperostosis and calcifications in ligaments and tendons may rarely occur,
especially among patients being treated with high doses for longer than usual periods.
Monitoring Parameters
CBC with differential and platelet count, baseline sedimentation rate, glucose, CPK; signs of depression,
mood alteration, psychosis, aggression, severe skin reactions
Pregnancy test (for all female patients of childbearing potential): Two negative tests prior to beginning
therapy (the second performed at least 19 days after the first test and performed during the first 5 days of
the menstrual period immediately preceding the start of therapy); monthly tests to rule out pregnancy
prior to refilling prescription.
Lipids: Prior to treatment and at weekly or biweekly intervals until response to treatment is established.
Liver function tests: Prior to treatment and at weekly or biweekly intervals until response to treatment is
established
Rosacea
Tinea Versicolor
Acne Keloidalis Nuchea
Telogen Effluvium
Prurigo Nodularis
Folliculitis Decalvans
Hypertrophic Scar/Keloid
DPNs
Pityriasis Rosea