14 - 80 - ACNE VARIANTS AND ACNEIFORM ERUPTIONS Flashcards
Infantile acne presents at 3 to 6 months of age classically with open and closed comedones over the cheeks and chin. (Fig. 80-2). Papules, pustules, and nodules can also present on the face. Pitted scarring may occur even with relatively mild disease. Infantile acne is caused in part by the transient elevation of dehydroepiandrosterone (DHEA) produced by the immature adrenal gland. 4 Additionally, during the first 6 to 12 months of life, boys may also have an increased level of luteinizing hormone (LH) that stimulates testosterone production. Around 1 year of age, these hormone levels begin to stabilize until they surge again during adrenarche. As a result, infantile acne usually resolves around 1 to 2 years of age. Treatment generally consists of topical retinoids and benzoyl peroxide. Oral therapy with erythromycin, azithromycin, trimethoprim, or isotretinoin can be used in severe or refractory cases.
most severe form of acne and may occur with or without systemic symptoms
Acne fulminans (also known as acute febrile ulcerative acne)
The sudden appearance of inflammatory, tender, oozing, friable plaques with hemorrhagic crusts characterizes this extreme presentation. The lesions predominate on the chest and back (Fig. 80-3) and rapidly become ulcerative and heal with scarring. The disease is reported to occur primarily in teenage boys. Systemic symptoms are often present. The patients are febrile; have a leukocytosis of 10,000 to 30,000/mm 3 white blood cells; and usually have polyarthralgia, myalgia, hepatosplenomegaly, and anemia. Bone pain is common, especially at the clavicle and sternum. Radiologic examination may demonstrate lytic bone lesions. Occasionally, there is accompanying erythema nodosum. Although this disease is often classified with acne conglobata, there are basic differences. The onset of acne fulminans is more explosive; nodules and polymorphous comedones are less common; the face is not involved as frequently, and the neck is usually spared; ulcerative and crusted lesions are unique; and systemic symptoms are more common.
Systemic glucocorticoid therapy, along with intralesional glucocorticoids, is first-line treatment for acne fulminans. Systemic glucocorticoids (prednisone 0.5–1.0 mg/kg/day) are started before isotretinoin for 2 to 4 weeks, depending on the severity of systemic symptoms, and continued during the first few weeks to months of isotretinoin therapy. The initial dosing of isotretinoin should be quite low (0.1 mg/kg/day) during the initial weeks of therapy until the inflammation is controlled. 6 The daily dose of glucocorticoids should be slowly decreased as tolerated over weeks to months. Alternately, 0.5 mg/kg/day of isotretinoin started immediately with 10 mg of prednisolone three times daily has been recommended. 7 Dapsone in conjunction with isotretinoin has been reportedly beneficial in the treatment of acne fulminans associated with erythema nodosum. 8 Cyclosporine, anakinra, and tumor necrosis factor (TNF) inhibitors have also been used in difficult cases of acne fulminans.9-11
What is SAPHO SYNDROME
SAPHO syndrome is manifested by synovitis, acne, pustulosis, hyperostosis, and osteitis. It is predominantly associated with hyperostosis of the anterior chest, palmoplantar pustulosis, hidradenitis suppurativa, and acne fulminans. Its cause is unknown. Reported successful treatments for SAPHO syndrome include nonsteroidal antiinflammatory drugs, sulfasalazine, infliximab, adalimumab, methotrexate. 12 Paradoxically, worsening of SAPHO skin manifestations can be seen with anti-TNF agents. 13,14 The bisphosphonates are beneficial for treating the associated bone pain.
What are PAPA, PASH, AND PAPSH SYNDROME
PAPA (pyogenic arthritis, pyoderma gangrenosum and acne), PASH (pyoderma gangrenosum, acne and hidradenitis suppurativa) and PAPASH (pyogenic arthritis, acne, pyoderma gangrenosum and hidradenitis suppurativa) are a group of systemic autoinflammatory disorders resulting from dysregulation of the innate immune system and over production of interleukin (IL)-1. 16 Patients with these disorders have a variable combination of sterile neutrophilic skin lesions, including acne, pyogenic granuloma, and hidradenitis suppurativa, and pyogenic arthritis. They may also give a history of inflammatory bowel disease and pancytopenia after administration of sulfacontaining medications. Mutations in the protein serinethreonine phosphatase interacting protein (PSTPIP1) results in an increase in IL-1β production. 17 There have been reports of successful treatment with cyclosporine, dapsone, infliximab, and anakinra.18,19
an autosomal dominant disorder marked by synostoses of the cranium, vertebral bodies and hands and feet
Apert syndrome, also known as acrocephalosyndactyly
It is caused by a mutation in the gene encoding FGFR-2. These patients have a diffuse acneiform eruption that often involves the arms, buttocks, and thighs. It is typically very resistant to treatment, but excellent responses to isotretinoin have been reported. 55 Patients with Apert syndrome may also present with severe seborrhea, nail dystrophy, and cutaneous and ocular hypopigmentation.
The facial Afro-Caribbean eruption (FACE) is another variant of periorificial dermatitis characterized by uniform, granulomatous sarcoidal papules distributed over the typical periorificial areas. 60 (Fig. 80-6) Distinguishing features include involvement of the upper eyelids and helices of the ears. 61 Histology is similar to granulomatous perioral dermatitis.
neonatal acne occurs around what age?
It resolves spontaneously within how many months?
2 weeks of age and resolve spontaneously within 3 months
Infantile acne is caused in part by the transient elevation of what hormone ?
**dehydroepiandrosterone (DHEA) **produced by the immature adrenal gland
Infantile acne presents at what month?
3 to 6 months
Mid-childhood acne is defined as appearing between what ages?
1 and 7 years of age
any child with acne in mid-childhood should be evaluated for other signs of hyperandrogenism (pubic or axillary hair, testicular enlargement or breast development)
mixture of comedones, papules, pustules, nodules, abscesses, and scars arising in a more generalized pattern over the back, buttocks, chest, and, to a lesser extent, on the abdomen, shoulders, neck, face, upper arms, and thighs
Acne conglobata
Draining lesions discharge a foul-smelling serous, purulent, or mucoid material.
Subcutaneous dissection with the formation of multichanneled sinus tracts is common
management of acne conglobata
- The use of isotretinoin is highly effective in these patients.
- However, because severe flares may occur when isotretinoin is started, the initial dose should be 0.5 mg/kg/day or less, and systemic glucocorticoids are often required either before initiating isotretinoin therapy or as concomitant therapy.
- Systemic tetracyclines, intralesional glucocorticoids, systemic glucocorticoids, surgical debridement, surgical incision, and surgical excision may also be required to effectively control acne conglobata
most severe form of acne and may occur with or without systemic symptoms
Acne fulminans (also known as acute febrile ulcerative acne)
The sudden appearance of inflammatory, tender, oozing, friable plaques with hemorrhagic crusts characterizes this extreme presentation.
Acne fulminans (also known as acute febrile ulcerative acne)
- The lesions predominate on the chest and back and rapidly become ulcerative and heal with scarring.
- The disease is reported to occur primarily in teenage boys
symptoms associated with acne fulminans
- The patients are febrile;
- have a leukocytosis of 10,000 to 30,000/mm3 white blood cells; and
- usually have polyarthralgia, myalgia, hepatosplenomegaly, and anemia.
- Bone pain is common, especially at the clavicle and sternum.
- Radiologic examination may demonstrate **lytic bone lesions. **
- Occasionally, there is accompanying erythema nodosum.
difference of acne fulminans and acne conglobata
- The onset of acne fulminans is more explosive;
- nodules and polymorphous comedones are less common;
- the face is not involved as frequently, and
- the neck is usually spared;
- **ulcerative and crusted **lesions are unique; and
- systemic symptoms are more common
first-line treatment for acne fulminans
Systemic glucocorticoid therapy, along with intralesional glucocorticoids
- Systemic glucocorticoids **(prednisone 0.5–1.0 mg/kg/day) **are started before isotretinoin for 2 to 4 weeks, depending on the severity of systemic symptoms, and continued during the first few weeks to months of isotretinoin therapy
- The i**nitial dosing of isotretinoin **should be quite low **(0.1 mg/kg/day) **during the **initial weeks of therapy **until the inflammation is controlled
- The daily dose of glucocorticoids should be** slowly decreased as tolerated over weeks to months. **
- Alternately, 0.5 mg/kg/day of isotretinoin started immediately with 10 mg of prednisolone three times daily has been recommended
- Cyclosporine, anakinra, and tumor necrosis factor (TNF) inhibitors have also been used in difficult cases of acne fulminans
has been reportedly beneficial in the treatment of acne fulminans associated with erythema nodosum
Dapsone in conjunction with isotretinoin
An explosive flare of tender, oozing, friable plaques with hemorrhagic crusts occurs with or without associated systemic symptoms after initiation of isotretinoin therapy
ISOTRETINOIN-INDUCED ACNE FULMINANS
- The flaring typically arises within the first month of treatment but may occur later.
- Patients with severe inflammatory acne in particular are at risk
how can you prevent flaring of acne when initiating isotretinoin?
- Lower starting doses of **isotretinoin (0.3 to 0.5 mg/kg/day) **and the concomitant addition of systemic corticosteroids may prevent flaring.
- If isotretinoin-induced acne fulminans does occur, the isotretinoin dose should be lowered or discontinued and prednisone therapy immediately started, following the guidelines for the management of acne fulminans above.
SAPHO syndrome
synovitis, acne, pustulosis, hyperostosis, and osteitis
It is predominantly associated with hyperostosis of the anterior chest, palmoplantar pustulosis, hidradenitis suppurativa, and acne fulminans.
Reported successful treatments for SAPHO syndrome
nonsteroidal antiinflammatory drugs, sulfasalazine, infliximab, adalimumab, methotrexate
Paradoxically, worsening of SAPHO skin manifestations can be seen with what agents
anti-TNF agents
beneficial for treating the associated bone pain in SAPHO syndrome
bisphosphonates
PAPA syndrome
pyogenic arthritis, pyoderma gangrenosum and acne
PASH syndrome
pyoderma gangrenosum, acne and hidradenitis suppurativa
PAPASH syndrome
pyogenic arthritis, acne, pyoderma gangrenosum and hidradenitis suppurativa
PAPA, PASH and PAPASH syndrome are a group of systemic autoinflammatory disorders resulting from dysregulation of the innate immune system and over production of what IL?
IL-1
what mutation results in an increase in IL-1β production?
protein serinethreonine phosphatase interacting protein (PSTPIP1)
Often quite mild acne is systematically and neurotically excoriated, leaving crusted erosions that may scar.
Acne excoriée (or acne excoriée des jeunes filles)
They are categorized under obsessive-compulsive disorders and as such should be comanaged with psychiatry to address both the acne and the underlying psychogenic excoriation.
Acneiform eruptions have been observed after repetitive physical trauma to the skin such as pressure, friction, or rubbing
ACNE MECHANICA
- Two types of reactions result, either a flare in acne with comedones and inflammatory papules (“acne mechanica”) or follicular inflammatory lesions (“folliculitis mechanica”)
- can occur from clothing (belts and straps), sports equipment (football helmets and shoulder pads), and with crutches and prosthetics
- classic example of acne mechanica is **“fiddler’s neck,” **produced where the violin pad repetitively rubs against the player’s lateral neck
- Alterations in the barrier function, microbiome, and activation of innate immune system, as well as keratinocyte disruption and increase in IL-1a are purposed to play a role
rare and disfiguring variant of acne vulgaris is acne with solid facial edema
Morbihan disease
- There is a** woody edema** of the mid third face with accompanying erythema and acne
- Treatment with low dose isotretinoin (0.2–0.5 mg/kg/day) alone or in combination with oral glucocorticoids, ketotifen (1–2 mg/day), or clofazimine for 4 to 5 months has been reported to be beneficial
clinical markers of PCOS
Hyperandrogenism, acne, insulin resistance, and acanthosis nigricans
- PCOS should be suspected in women with any combination of oligomenorrhea, clinical or biochemical hyperandrogenism, or polycystic ovaries on ultrasound scan
- Women with PCOS are at increased risk of infertility, impaired glucose tolerance, type 2 diabetes, dyslipidemia, endometrial cancer, and cardiovascular disease
Premenarchal women with acne and hirsutism should be screened for PCOS with what lab test?
serum free testosterone level
treatment of PCOS
- Combined oral contraceptives with spironolactone can be helpful in controlling acne and hirsutism
- The addition of metformin can also beneficial
- Additional treatment consists of weight management, lipid control, regulation of insulin resistance, and fertility assistance
Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder caused by varying defects in what enzyme?
21β-hydroxylase
what lab test can be requested to investigate for presence of Congenital adrenal hyperplasia (CAH)?
dehydroepiandrosterone sulfate (DHEAS) in the range of 4000 to 8000 ng/mL
manifestations of CAH
- Neonates are screened at birth for the classic type and typically present with ambiguous genitalia and salt wasting
- The nonclassic type is not identified at birth and can present throughout childhood and adolescence
- Patients with this type of CAH have normal cortisol levels but increased androgens
- Female patients present with findings similar to PCOS, including precocious puberty, irregular menses, polycystic ovaries, hirsutism, and acne.
- Findings of CAH in males are often subtle and acne may be the only sign.
- CAH should be considered in patients who do not respond to treatment
- Treatment of patients with CAH consists of** low-dose replacement of glucocorticoids, as well as oral contraceptives, spironolactone, or flutamide in females.**
as early as how many weeks can steroid folliculitis occur after initiation of systemic steroids ?
2 weeks
Similar lesions may follow the prolonged application of topical glucocorticoids to the face or with inhaled steroids for asthma
differentiate lesions of steroid folliculitis from acne vulgaris
- The lesions, which are usually all in the same stage of development, consist of small pustules and red papules
- In contrast to acne vulgaris, they appear mainly on the trunk, shoulders, and upper arms, with lesser involvement of the face.
- comedones, cysts, and scarring are unusual
- Treatment consists primarily of **stopping any corticosteroid use. **
- Typical acne treatments such as topical retinoids and antibiotics may also be helpful
other drugs that can cause a monomorphic, diffuse papular eruption that mimics steroid folliculitis (Drug-induced acneiform eruptions)
BEG PHIL Halo
- BRAF inhibitors
- Epidermal growth factor receptor inhibitors (cetuximab, panitumumab, necitumumab, pertuzumab, and small-molecule tyrosine kinase inhibitors gefitinib, erlotinib, afatinib, osimertinib, and lapatinib)
- Glucocorticoids
- Phenytoin
- High-dose vitamin B complex
- Isoniazid
- Lithium
- Halogenated compounds (containing iodides/ bromides - often found in cold and asthma remedies, sedatives, radiopaque contrast material, kelp, and other vitamin and mineral combinations)
frequent side effect of the EGFR inhibitors
perifollicular, papulopustular eruption distributed on the face and upper torso
- For mild disease, t**opical hydrocortisone 2.5% cream or clindamycin 1% lotion **is suggested.
- For more severe disease, **higher strength topical steroids, doxycycline or minocycline, or low-dose isotretinoin **is recommended
term that is used to describe occupational acne caused from chlorinated hydrocarbons
Chloracne
- Chloracne classically affects the malar, retroauricular, and mandibular regions of the head and neck, as well as the axillae and scrotum
- Most chloracne lesions clear up within 2 years, providing exposure to the chemical has stopped.
- Treatment with topical or systemic retinoids or oral antibiotics may be beneficial
most common cause of industrial acne
Cutting oils
may occur in patients with preexisting acne vulgaris treated with long-term oral antibiotics, especially the tetracyclines
Gram-negative folliculitis
- Gram-negative folliculitis may appear as either papulopustules concentrated around the nose or as deep-seated nodules.
- Culture of these lesions may reveal **Enterobacter, Klebsiella, or Escherichia **spp. in the papulopustules or Proteus spp. in the nodules.
Patients usually give a history of initial success with oral tetracyclines followed by a worsening of their acne
Gram-negative folliculitis
- An appropriate antimicrobial agent with adequate gram-negative coverage should be used.
- In recalcitrant cases, gram-negative folliculitis improves with oral isotretinoin for 4 to 5 months.
- Gram-negative bacteria require a moist environment for survival, and the drying action of isotretinoin will kill the bacteria.
this type of acneiform reaction may produce a yellow, atrophic plaque studded with large open comedones known as Favre-Racouchot syndrome
Radiation Acne
- The lesions are usually symmetrically distributed on the temporal and periorbital areas.
- The exact pathogenesis of Favre-Racouchot syndrome is unknown, but it is suggested that extensive UV exposure as well as exposure to harsh climates and smoking may be risk factors.
- It can be treated with oral or topical retinoids as well as comedone extraction or CO 2laser
In extreme heat, a severe acneiform folliculitis may develop. This is known as
TROPICAL ACNE
- occurs mainly on the trunk and buttocks
- It has many deep, large, inflammatory nodules with multiple draining areas, resembling acne conglobata
This monomorphous eruption consists of multiple, uniform, red, papular lesions seen after sun exposure.
ACNE AESTIVALIS
- It was initially called Mallorca acne
- The eruption is caused by the effects of UV radiation, primarily UVA
- Like polymorphous light eruption, patients with acne aestivalis will flare on reexposure to UV light.
Preadolescent patients develop c**omedones, milia, and acneiform red papules **within the nasal crease
PSEUDOACNE OF THE NASAL CREASE
- It can be familial or associated with the “allergic salute.”
autosomal dominant disorder marked by synostoses of the cranium, vertebral bodies and hands and feet
Apert syndrome, also known as acrocephalosyndactyly,
Apert syndrome, also known as acrocephalosyndactyly is caused by what mutation?
- These patients have a diffuse acneiform eruption that often involves the arms, buttocks, and thighs.
- may also present with severe seborrhea, nail dystrophy, and cutaneous and ocular hypopigmentation
mutation in the gene encoding** FGFR-2**
Small inflammatory papules and pustules group around the mouth in a typically well demarcated pattern, with sparing of the lip margin
PERIORAL DERMATITIS/ PERIORIFICIAL DERMATITIS
- acneiform eruption that occurs in a bimodal distribution **young children and young adults. **
- there is female predominance, mostly in adolescents and adults
- If the nose, eyes, or groin region is involved, it is called periforificial dermatitis
- The neck, trunk, and extremities are rarely involved but have been reported in children with the disorder.
- Usually perioral dermatitis is asymptomatic with occasional itchy or burning noted.
larger, granulomatous inflammatory papules coalesce into a well delineated plaque around the mouth with more likely involvement of the periocular, perinasal, and groin areas
periorificial granulomatous dermatitis
known triggers for periorificial dermatitis
- topical or inhaled corticosteroid use, particularly fluorinated steroids and fluoride in dentifrices
- Additionally, demodex may play a role in some cases of periorificial dermatitis
treatment of periorificial dermatitis
- **Topical corticosteroid **use should be avoided because it tends to perpetuate the eruption.
- In mild cases resulting from corticosteroid use, discontinuation of steroid application can alone be effective.
- Alternate therapies include topical antimicrobial and antiinflammatory agents, including metronidazole cream or gel, clindamycin gel or lotion, erythromycin gel, azelaic acid cream, and sodium sulfacetamide lotion.
- Topical pimecrolimus cream or tacrolimus ointment has also been used in to treat periorificial dermatitis and to diminish flares from topical steroid withdrawal.
- In children,** oral erythromycin or azithromycin** may be added for refractory or severe cases, but tetracyclines are commonly used in adolescents and adults.
- Recently, the antiparasitic agents praziquantel and ivermectin (both topical and oral) have been successfully used.
