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3.12 Figurate Erythemas
Box 3.7 Differential Diagnosis of Flushing
Common causes
• Benign cutaneous ushing
• Emotion
• Exercise
• Temperature
• Food or beverage
• Rosacea
• Climacteric ushing
• Fever
• Alcohol
Uncommon, serious causes
• Carcinoid tumors/syndrome, gastroenteropancreatic neuroendocrine tumors
• Pheochromocytoma
• Mastocytosis
• Medullary thyroid carcinoma
• Pancreatic cell tumor (VIP tumor)
• Renal cell carcinoma
• Anaphylaxis/hypersensitivity
Other causes
• Fish ingestion
• Histamine (scombroid): poor refrigeration → bacterial overgrowth and
conversion of histidine to histamine
• Ciguatera sh poisoning from ciguatoxin (made by algae Gambierdiscus
toxicus)
• Psychiatric or anxiety disorders
• Hyperthyroidism
VIP, vasoactive intestinal peptide; CCBs, calcium channel blockers; NSAIDs, nonsteroidal anti-inammatory drugs; POEMS, polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes
Modied from Izikson L, English JC 3rd, Zirwas MJ. The ushing patient: differential diagnosis, workup, and treatment. J Am Acad Dermatol. 2006;55(2):
193–208; Data from Sadeghian A, Rouhana H, Oswald-Stumf B, Boh E. Etiologies and management of cutaneous ushing: malignant causes. J Am Acad
• Idiopathic ushing
• Neurologic
• Parkinson’s
• Migraines
• Multiple sclerosis
• Harlequin syndrome
• Trigeminal nerve damage
• Horner syndrome
• Riley-Day syndrome
• Frey syndrome
• Autonomic epilepsy
• Autonomic hyperreexia
• Paroxysmal extreme pain disorder
• Orthostatic hypotension
• Erythromelalgia
• Streeten syndrome
• Medications (e.g., CCBs, vancomycin, metronidazole, cisplatin, cyclosporine,
tamoxifen, disulram, glucocorticoids, morphine, opiates, NSAIDs, pilocarpine, serotonin agonists, niacin, nitroglycerine, sildenal, prostaglandins)
• Infusion reactions (e.g., vancomycin, ciprooxacin, amphotericin B, blood)
Very rare causes
Sarcoid, mitral stenosis, dumping syndrome, male androgen deciency,
arsenic intoxication, POEMS syndrome, basophilic granulocytic leukemia,
bronchogenic carcinoma, malignant histiocytoma, malignant neuroblastoma,
malignant ganglioneuroma, peri-aortic surgery, Leigh syndrome,
homocystinuria, superior vena cava obstruction, Rovsing syndrome,
postherpetic gustatory ushing and sweating
Table 3.27 Common Malignancies Associated with Flushing: Clinical Findings and Laboratory Markers
Mastocytosis Carcinoid Syndrome Pheochromocytoma Central Nervous System Other
Clinical ndings
Flushing X X X X
Blood pressure May be low Hypertension May be low or high VIPoma is
Oropharyngeal edema/congestion X Medullary thyroid
Dysphagia Rare Esophageal
Cardiac disease X X
Pulmonary X X Bronchogenic
Gastrointestinal X X X X VIPoma
Laboratory markers
Serotonin/5-hydroxyindoleacetic
acid
Total tryptase X Hypersensitivity/
Histamine X
Metanephrines X
Vanillylmandelic acid X
Norepinephrine X
Vasoactive intestinal peptide VIPoma
Creatinine X (obtained to ensure
VIPoma, Vasointestinal polypeptide-secreting tumor.
From Sadeghian A, Rouhana H, Oswald-Stumpf B, Boh E. Etiologies and management of cutaneous ushing: malignant causes. J Am Acad Dermatol.
2017;77(3):405–414.
carcinoid
X (24-hour urine for
2 consecutive days)
adequate urine
collection)
associated with
hypotension
carcinoma
carcinoma
anaphylaxis
175
CHAPTER 3 • General Dermatology
3.13 FOLLICULAR AND ECCRINE/ APOCRINE DISORDERS
Acne vulgaris
Epidemiology
• Peaks in adolescence; affects 85% between 11 and 30 yo
• .35% of women and 20% of men report acne in their 30s
Pathogenesis
• Disease of pilosebaceous unit with multifactorial
pathogenesis: Cutibacterium acnes, sebum overproduction,
abnormal keratinization, and/or inammation
• Abnormal follicular keratinization (IL-1α trigger?) and
microcomedone formation → comedo rupture → release
of keratin and sebum → inammatory papule/pustule
→ nodule/cyst
• Hormones
■
Androgens (especially dihydrotestosterone [DHT] and
testosterone) → ↑ growth of sebaceous glands/↑
sebum production
Androgen receptors are found on the basal layer of
sebaceous glands and outer root sheath of hair follicle
↑ Androgen levels present during rst 6 months
(↑ luteinizing hormone [LH]) and at adrenarche
(↑ DHEAS)
• Cutibacterium acnes
■
Gram-positive anaerobic rod; ribotypes 4 and 5 more
prominent in acne
■
Produces lipases that break down triglycerides in sebum
into FFAs, which are comedogenic and proinammatory
■
Activates TLR-2 on macrophages, which induces
proinammatory cytokines (IL-1a, IL-8, IL-12, MMPs,
TNF-a) and attracts neutrophils
■
Activates NLRP3 (part of inammasome) of
neutrophils/monocytes → ↑ IL-1b
■
Stronger peripheral macrophage response to P. acnes
noted in patients with acne (i.e., tretinoin n
CD2091 macrophages which are good good at
killing P. acnes)
■
Produces coproporphyrin III, which uoresces under
Wood’s lamp
• Inammation: neutrophil predominant → pustule;
lymphocytes 1 foreign body-type GCs 1 neutrophils →
nodules/papules/cysts; scars more common in
inammation that is delayed and specic
• Dietary
■
Unclear, but skim milk, whey protein, vitamin B12,
and high glycemic load may contribute to acne
Clinical features
• Most common sites (sebaceous areas): face, neck, behind
ears, upper trunk, and upper arms
• Frequently start as open and closed comedones
• May develop more inammatory lesions including
papules, pustules, nodules, and cysts; nodulocystic lesions
can coalesce into plaques and sinus tracts
• As lesions resolve may leave postinammatory
hyperpigmentation/erythema or scars (icepick, rolling,
boxcar, anetoderma-like, hypertrophic, and keloidal)
• Women may are week prior to menstruation
Histopathology
• Follicle lled with laminated keratin and debris,
6 suppurative inammation
Treatment/clinical course
• See Table 3.28 for treatment approach to acne
• Topicals/devices: retinoids (downregulate TLR-2
expression), benzoyl peroxide, azelaic acid, clindamycin,
dapsone, erythromycin, sodium sulfacetamide/sulfur,
clascosterone, salicylic acid, chemical peels, light/laser
therapy (blue light safe in pregnancy)
• Orals: oral antibiotics (tetracyclines, penicillins/
cephalosporins, sulfonamides, and azithromycin/
erythromycin), hormonal agents (spironolactone, OCPs
[5 are FDA-approved for acne]), isotretinoin, metformin
Table 3.28 Treatment Approach for Acne
Mild Moderate Severe
1st Line
Treatment
Alternative
Treatment
The double asterisks (**) indicate that the drug may be prescribed as a xed combination product or as separate component.
From Zaenglein AL, Pathy AL, Schlosser BJ, Alikhan A, Baldwin HE, Berson DS, et al. Guidelines of care for the management of acne vulgaris. J Am Acad
Dermatol. 2016;74(5):945-973.e33.
Benzoyl peroxide (BP)
or topical retinoid
-or-
Topical combination therapy**
BP 1 antibiotic
or retinoid 1 BP
or retinoid 1 BP 1 antibiotic
Add topical retinoid or BP (if not
on already)
-or-
Consider alternate retinoid
-or-
Consider topical dapsone
Topical combination therapy**
BP 1 antibiotic
or retinoid 1 BP
or retinoid 1 BP 1 antibiotic
-or-
Oral antibiotic 1 topical retinoid 1 BP
-or-
Oral antibiotic 1 topical retinoid 1 BP 1 topical
antibiotic
Consider alternate combination therapy
-or-
Consider change in oral antibiotic
-or-
Add combined oral contraceptive or oral
spironolactone (females)
-or-
Consider oral isotretinoin
Oral antibiotic
1
Topical combination therapy**
BP 1 antibiotic
or retinoid 1 BP
or retinoid 1 BP 1 antibiotic
-or-
Oral isotretinoin
Consider change in oral antibiotic
-or-
Add combined oral contraceptive or oral
spironolactone (females)
-or-
Consider oral isotretinoin
176
3.13 Follicular and Eccrine/Apocrine Disorders
(especially polycystic ovarian syndrome [PCOS]
patients), prednisone
• Complementary and alternative therapies: pantothenic
acid, zinc, L-carnitine, probiotics, omega 3, borage seed
oil, pumpkin seed oil; myoinositol and diindolmethane
for women
• Intralesional CS for inamed papulonodules
• Multiple modalities for scarring, including laser resurfacing,
chemical peels, subcision, dermabrasion, llers
Acne variants
Acne fulminans
• Males 13–16 years
• May occur after isotretinoin initiation or dose increase
• Most severe cystic acne, w/ systemic manifestations
■
Acute suppurative nodules and plaques
■
Lesions are friable w/ hemorrhagic crust; can ulcerate
and form black eschar
■
Often scars
■
Chest, shoulders, and back; rarely on face
■
Fever, ↑ WBC, and ↑ ESR
■
Sterile osteolytic bone lesions (sternum, clavicle, and
long bones), arthralgias, myalgias, and
hepatosplenomegaly can also be seen
• Treat with oral CS, followed by oral isotretinoin when
acute inammation has subsided
■
If a patient on isotretinoin develops acne fulminans →
↓ isotretinoin dose immediately
• Polymorphisms in TLR-4 may be protective against acne
fulminans
• Unusual distribution pattern based on external provoking
factor
Acne excoriée (de jeunes lles)
• Young women; may be a/w underlying depression or
anxiety disorder, obsessive compulsive disorder (OCD),
body dysmorphic disorder, eating disorder,
trichotillomania, or borderline personality disorder
• Self-mutilation of imagined acneiform lesions or mild
acne lesions → excoriated crusted erosions
• Treatment: antidepressants, behavioral modication, and
psychotherapy
Neonatal acne (Neonatal cephalic pustulosis)
Epidemiology
• Appears within rst few weeks, resolves by 3 months
• 20% of healthy newborns
• M . F
Pathogenesis
• KOH may demonstrate Malassezia
• Other cases likely related to stimulation of sebaceous glands
by maternal androgens or transient androgen production
Clinical features
• Cheeks and nasal bridge are common sites, but lesions
may occur anywhere on the face/head/neck
• Inammatory papules and pustules more common than
comedones
Acne conglobata
• M . F
• Severe eruptive nodulocystic acne, without systemic
manifestations (vs. acne fulminans)
■
Cysts, nodules, and large abscesses with sinus formation
■
Suppuration is characteristic (lesions contain thick,
yellow, blood-tinged uid)
■
Secondary comedones can be white, rm, cyst-like or
polyporous (clusters of blackheads)
■
Often scars
■
Usually on trunk (especially the back); less severe on face
• Treat with isotretinoin; may need to pretreat with
prednisone to avoid acne fulminans
Solid facial edema in acne
• Swelling in midline face and cheeks
• Woody non-pitting, non-scaling induration (peau d’orange
appearance)
• Acne predates edema by 2–5 years
• ToC 5 isotretinoin 1/– ketotifen (antihistamine) or
prednisone
Acne mechanica
• Repeated pressure/friction → obstruction of pilosebaceous
unit (helmets, backpacks, collars, ddler’s neck)
Treatment/clinical course
• Usually regresses over few months
• If mild, cleanse with gentle soap and water
• Topical imidazole, topical retinoid, topical antibiotic or
benzoyl peroxide
• Oral antibiotics (erythromycin) or isotretinoin if severe
Differential diagnosis
• Some neonates with trisomy 21 may develop a leukemoid
reaction, which manifests as severe pustular eruption on
the face, mimicking neonatal acne
Infantile acne
Epidemiology
• Appears around 3–6 months; usually resolves by
2–3 years
• M . F
Pathogenesis
• Hormonal imbalance (hyperandrogenism)
Clinical features
• More severe and persistent compared with neonatal acne
• Comedones (primarily) and inammatory lesions
including occasionally deep cysts
• Can result in scarring
• Usually limited to face
177
CHAPTER 3 • General Dermatology
Treatment/clinical course
• Therapy is often necessary due to risk of scarring
• Topical retinoids, topical antibiotics, or in moderate-
severe cases azithromycin or isotretinoin
• If acne arises between ages 1 and 7 (mid-childhood
acne) and signs of pubertal development are noted
(pubarche, thelarche, etc), an endocrinology evaluation
should be considered along with the following
laboratory analysis: DHEAS, androstenedione, 17-OHprogesterone, and bone age
• May predict propensity toward future acne
Transverse nasal crease
• Arise during early childhood
• Horizontal anatomical demarcation line at border of
middle and lower third of the nose at junction of
triangular and alar cartilage (Fig. 3.79)
• May contain milia/cysts/comedones
Acne in setting of endocrinologic abnormality
Epidemiology/pathogenesis
• Hyperandrogenism due to:
■
PCOS: suspect in females with hirsutism or
irregular menses; most common endocrinopathy a/w
acne
■
HAIR-AN syndrome (discussed below)
■
Congenital adrenal hyperplasia (CAH; children with acne)
■
Androgen-secreting tumors and Cushing’s syndrome
Clinical features
• Signs: irregular menstrual cycles, androgenetic alopecia
(AGA), hirsutism, deep voice, clitoromegaly
■
Distribution depends on underlying cause of androgen
excess
• Adult women may have acne along jawline or lower face
(adult female acne; hormonal pathogenesis)
Workup
• Initial workup: total/free testosterone, DHEAS, LH,
and follicle-stimulating hormone (FSH); can also
consider sex hormone-binding globulin [SHBG],
17-hydroxyprogesterone, prolactin, morning cortisol,
and adrenocorticotropic hormone [ACTH]
stimulation test
■
↑ Total testosterone 5 ovary producing androgen
Testosterone 100 to 200 ng/dL 1/– LH/FSH ratio .
2 to 3: PCOS
Testosterone . 200 ng/dL: ovarian tumors
■
↑ DHEAS or ↑ 17-hydroxyprogesterone 5 adrenal
gland producing androgen
DHEAS 4000 to 8000 ng/mL or
17-hydroxyprogesterone . 3 ng/mL 5 CAH (defects
in 21-hydroxylase or 11-hydroxylase)
DHEAS . 8000 ng/mL: adrenal tumors
Treatment/clinical course
• Treat underlying abnormality
• OCPs or spironolactone
Additional information
• XYY genotype may have more severe acne
Acne cosmetica
• Frequent/heavy use of cosmetics containing lanolin,
petrolatum, vegetable oils, butyl stearate, isopropyl
myristate, sodium lauryl sulfate, lauryl alcohol, or oleic
acid → small closed comedones, small papules and
pustules
Pomade acne
• More common in African Americans using greasy/oily
grooming substances on the scalp
• Closely set, monomorphic, small closed comedones on
forehead and temples
Chloracne
• Type of occupational acne caused by exposure to
chlorinated aromatic hydrocarbons (found in electrical
conductors, insulators, and insecticides/fungicides/
herbicides)
■
Agent orange was contaminated with
2,3,7,8-tetrachlorodibenzo-p-dioxin (dioxin), a
chlorinated hydrocarbon
• Acne develops after several weeks of exposure
• May have recurrent outbreaks for many years after
exposure
• Preferred sites are face, neck (including retroauricular),
axilla, scrotum, and penis
• Treatment difcult; ToC is isotretinoin (high dose
followed by low-dose maintenance), topical retinoids,
surgical intervention for large lesions
Fig. 3.79 Transverse nasal crease. (Courtesy of Christopher Sayed, MD.)
178
Radiation acne
• Ionizing rays induce epithelial metaplasia in follicles →
hyperkeratotic plugs
• Comedo-like papules in areas of previous radiation
exposure
• Appears as the acute phase of radiation dermatitis
resolves
3.13 Follicular and Eccrine/Apocrine Disorders
Acneiform eruptions
Drug-induced acne
Epidemiology/pathogenesis
• A/w multiple meds:
■
Anabolic steroids
■
Androgens (testosterone)
■
CS
■
Halogens (iodides and bromides)
■
INH
■
OCPs (containing androgen-like progestins)
■
Lithium
■
Phenytoin
■
Upadacitinib
■
Vitamins B2, B6, and B12
■
Epidermal growth factor receptor (EGFR) inhibitors
(monoclonal antibodies: cetuximab and panitumumab;
tyrosine kinase inhibitors: getinib, erlotinib, and
lapatinib)
■
Other chemotherapy a/w acneiform eruptions:
mTOR inhibitors (sirolimus and tacrolimus)
Multikinase inhibitors (sunitinib and sorafenib)
MEK inhibitors (trametinib)
• Mnemonic: “SHIELD yourself from acne with vitamin T:”
Steroids (anabolic, CS), Halogens, Isoniazid, EGFR
inhibitors, Lithium, Dilantin (phenytoin), Vitamin B2,
B6, B12, Testosterone
Clinical features
• Abrupt-onset monomorphous inammatory papules or
pustules (classic in CS-induced acne)
• Usually lacks comedones
• Trunk . face
• In setting of EGFR inhibitors, occurs in usual acne-
prone areas and sun-exposed areas; starts 1 to 3 weeks
after beginning treatment; severity of reaction positively
correlates with clinical response to medication; occurs in
. 80% of patients
Treatment/clinical course
• Discontinue offending medication if possible
• For EGFR inhibitors: prophylaxis with doxycycline or
minocycline started on same day as EGFR inhibitor
therapy; DO NOT use irritating agents like topical
retinoids or benzoyl peroxide
Acne-associated syndromes
SAPHO (Chronic recurrent multifocal osteomyelitis)
Chest wall and mandible are most common areas of
musculoskeletal pain
Of note, “bull’s head” sign may be seen on X-ray
• Acne varies from mild to acne congolobata/fulminans;
hidradenitis suppurativa and dissecting cellulitis can also
be seen
• Pustulosis includes palmoplantar pustulosis and pustular
psoriasis (psoriasis can also be seen)
• Associated with IBD
• Treatment: bisphosphonates, TNF-a inhibitors, MTX,
NSAIDs, CS, colchicine, anakinra
PAPA
• Pyogenic Arthritis (sterile), Pyoderma gangrenosum, Acne
conglobata
• Autosomal dominant mutation in CD2-binding protein 1
(CD2BP1; aka PSTPIP1)
■
Part of autoinammatory disease group as CD2BP1
interacts with pyrin (mutation → unopposed
inammation)
• Treatment: systemic or local CS, dapsone, iniximab,
anakinra
• Also know PASH (Pyoderma gangrenosum, Acne,
Suppurative Hidradenitis) and PAPASH (Pyogenic Arthritis,
Pyoderma gangrenosum, Acne, Suppurative Hidradenitis)
HAIR-AN
• Hyper Androgenism, Insulin Resistance, Acanthosis Nigricans
• Can be considered a unique subtype of PCOS in women
with high risk of diabetes and cardiovascular disease
• Treatment: anti-androgens (e.g., spironolactone), OCPs,
and insulin-sensitizing medications (e.g. metformin)
Apert syndrome (Acrocephalosyndactyly)
• Autosomal dominant mutation in broblast growth
factor receptor 2 (FGFR2); ↑ FGFR2 signaling → follicular
hyperkeratosis and sebaceous gland hypertrophy; FGFR2
mutations also found in some nevus comedonicus cases
• Synostoses of bones of hands/feet, vertebral bodies,
and cranium
• Diffuse distribution of moderate to severe acne,
especially on extensor arms, buttocks, and thighs
• Nail dystrophy and cutaneous/ocular hypopigmentation
• Treatment: isotretinoin
Rosacea
Epidemiology
• Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis
• Affects children and young adults (usually appears in
third decade); more common in Japan
■
Inammatory disorder of unclear etiology
■
Characterized by RF(–) osteoarthropathy with various
skin manifestations
Bone disease precedes skin disease in the majority
Sternoclavicular area is the most common site of
inammation
• Peaks at 30–40 yo; F . M; usually skin types I and II
Pathogenesis
• Chronic vascular inammatory disorder
• Multifactorial: vascular hyperreactivity, dysregulated
innate immunity, chronic solar damage, sensitivity to heat,
hyperirritable skin, and possible association with Demodex
• Kallikrein 5, TPRV, cathelicidin LL-37, and TLR2 are
upregulated
179
CHAPTER 3 • General Dermatology
Clinical features
• Usually limited to central face
• Depends on subtypes (see Rosacea subtypes section)
Histopathology
• Perivascular and perifollicular lymphohistiocytic inltrate,
vascular ectasia, mild edema, and sebaceous hyperplasia
Treatment/clinical course
• Avoid triggers (sunlight, heat/cold, stress, strong
emotions, alcohol, hot beverages, spicy foods, chemical
irritation) and use sunscreen
• Topicals: metronidazole, sodium sulfacetamide/sulfur,
azelaic acid, benzoyl peroxide, clindamycin, ivermectin,
and green-tinted makeup
• Topical brimonidine (a2-adrenergic agonist) and
oxymetazoline (a1 . a2-adrenergic agonist) reduce
background erythema and ushing
• Systemic: tetracyclines (consider subantimicrobial
modied release dosage doxycycline—fewer AEs),
amoxicillin, and isotretinoin if severe
• Others: intense pulsed light (IPL) and pulsed dye laser
(PDL); rhinophyma: CO2 laser and electrosurgery
Additional information
• Haber’s syndrome: genodermatosis w/ rosacea-like
eruption and verrucous lesions
Rosacea subtypes
Rosacea variants
Solid facial edema in rosacea (Morbihan disease and rosacea lymphedema)
• Unknown etiology; possibly due to chronic inammation
→ obstruction of lymph vessels or brosis
■
There is a similar condition in acne
• Hard non-pitting swelling of forehead, glabella, nose,
and cheeks
• May be more pronounced during early morning hours
• ↓ Vision if eyelids involved
• Spontaneous resolution DOES NOT occur
• ToC 5 isotretinoin 6 ketotifen (antihistamine)
■
Other options: systemic steroids, antibiotics, and
lymphatic drainage/compression therapy
Pyoderma faciale (Rosacea fulminans)
• Females in their 20s to 30s
• Rapid onset of intensely inamed coalescent uctuant
nodules and cysts on background of dark red to cyanotic
erythema; can have draining sinuses with purulent drainage
• Centrofacial region, no involvement elsewhere and no
comedones (vs. acne fulminans)
• Most develop scarring
• Can have low-grade fever, myalgias, ↑ WBC, and ↑ ESR
• Treatment (same as acne fulminans): prednisone (with
slow taper) and isotretinoin
Erythematotelangiectatic (Vascular)
• Central face with recurrent blush that eventually becomes
permanent ushing
• Burning, stinging sensation; easily irritated with roughness
and scaling
• Can have associated edema
• 1/– Telangiectasias
Papulopustular (Inammatory)
• Similar to acne vulgaris, but lesions may have deeper red
color and no comedones
• Persistent central facial erythema with transient papules/
pustules
Phymatous
• Thickening of skin due to overgrowth of sebaceous glands
• Most common on nose (rhinophyma); can also involve
chin (gnathophyma), forehead (metophyma), earlobes
(otophyma), and eyelids (blepharophyma)
Ocular
• 50% of rosacea patients affected
• Many complaints including dryness, foreign body
sensation, photosensitivity, burning/stinging, blepharitis,
recurrent chalazion, conjunctivitis, keratitis, iritis, and
scleritis
• Treatment: doxycycline/minocycline
Granulomatous rosacea
• Middle-aged women
• Discrete yellow/brown-red rm papules or nodules on
background of diffusely reddened thickened skin on
buttery region; can be distributed around periphery of
face and perioral areas
• Histology: non-caseating epithelioid granulomas
(resembles sarcoidosis)
• Treatments: tetracyclines and isotretinoin
Lupus miliaris disseminatus faciei
• Young adults; more common in Asians (especially Japanese)
• Smooth, rm, yellow-brown to red 1 to 3 mm
monomorphous papules with apple jelly color on diascopy
• Present in typical buttery distribution, but also seen
laterally (below mandible), perioricially, and involving
the eyelid skin (characteristic site)
• Heals with scarring
• Histology often shows caseating granulomas
• Treatment is difcult—can try isotretinoin and
tetracyclines
Perioral/perioricial dermatitis
Epidemiology
• Young women in their 20s to 30s
• Children can also be affected (of note, perialar intertrigo
may be an overlap of seborrheic dermatitis and perioral
dermatitis in kids)
180
3.13 Follicular and Eccrine/Apocrine Disorders
Pathogenesis
• Inammatory condition of unknown cause, perhaps
related to rosacea
• Most commonly attributed to use of topical uorinated
CS or facial cosmetics
Clinical features
• Clusters of small, pink discrete scaly papules/pustules in
perioral region with clear zone around the vermilion border
■
Can also involve opening of nares, nasolabial folds,
and cheeks
• Burning sensation, minimal itching
Treatment/clinical course
• Self-limited, but resolution can take months to years
• Avoid cosmetics, topical steroids, and other irritating topicals
• Tetracyclines (or azithromycin/erythromycin in pediatrics)
3 6 to 8 weeks with gradual tapering to avoid rapid
rebounding, oral/topical ivermectin, TCIs, topical
antibacterials, topical metronidazole
Variants
• Periorbital/periocular dermatitis
• Perioricial dermatitis is a combination of perioral and
periorbital/periocular dermatitis
Folliculitis
Supercial folliculitis
• Culture of pustule usually 5 normal ora
■
When culture is positive, S. aureus is most common
infectious etiology
• Perifollicular pustules often with erythematous base in areas
with terminal hairs (scalp, beard, trunk, buttocks, and thighs)
• Treatment depends on culture results; if culture-negative:
topical benzoyl peroxide, topical antibiotics, tetracyclines
Gram-negative folliculitis
• Occurs in acne patients receiving prolonged antibiotic
treatment (esp. tetracyclines) More common in adult men
• Anterior nares become colonized with gram-negative
organisms (Proteus, Enterobacter, Escherichia coli, or Klebsiella)
• Numerous pruritic pustules on an erythematous base;
short-lived, but new lesions continue to appear
• Central region of face, lesions fan out from nose/mouth
to involve perinasal/beard region
• ToC 5 isotretinoin; topical gentamicin or oral
ciprooxacin can be considered
Hot tub folliculitis
• A gram-negative folliculitis due to Pseudomonas aeruginosa
• Use of hot tub 12 to 48 hours prior to onset
• Edematous pink to red perifollicular papules and pustules
on the trunk
• Self-resolves
Eosinophilic folliculitis
• Three forms:
■
Eosinophilic pustular folliculitis (Ofuji’s disease)
30 yo; M . F; more common in Japanese
Recurrent explosive crops of intensely pruritic
grouped follicular papules and pustules
♦ Can also have erythematous patches and plaques
with superimposed coalescent pustules
♦ Central clearing and centrifugal extension leads to
gurate/serpiginous lesions
♦ Most common on face, back, and extensor arms
♦ Peripheral eosinophilia
Spontaneous resolution followed by relapses every
3–4 weeks
Symptomatic treatment of pruritus and oral
indomethacin may help
■
Immunosuppression-associated esosinophilic
folliculitis (AIDS-associated eosinophilic folliculitis)
See Chapter 5, Infectious Dermatology
■
Neonatal eosinophilic pustular folliculitis
Early in infancy
Pruritic perifollicular pustules and vesicles on
erythematous base usually on scalp
♦ Secondary crusting is common
Self-limited, cyclical course for few months to years
Disseminate and recurrent infundibulofolliculitis
• Adults with darkly pigmented skin
• Hundreds of monotonous 1 to 2 mm pruritic esh-colored
follicular papules (similar to goose bumps in appearance)
on trunk . neck and upper extremities lasting months-years
• Histology: edema and sparse lymphocytes and neutrophils
inltrating the follicular infundibulum
• Treatments: topical CS, lactic acid creams, urea creams
Viral-associated trichodysplasia
• Occurs in the context of immunosuppression
• Trichodysplasia spinulosa-associated polyomavirus →
pink spiny papules on central face
• Histology: proliferation of inner root sheath cells with
increased trichohyalin granules
• Treatment: topical cidovir, tazarotene gel, oral
valganciclovir
Pseudofolliculitis barbae
• Most common in African American curly-haired men
who shave their beard
■
Keratin H6hf (Keratin 75) mutations have been
implicated
• Tightly curled hairs curve back into skin after being
shaved → inammatory reaction with papules/pustules
• Hyperpigmentation, hypertrophic scars, and keloids are
possible
• Treatment: stop shaving; laser hair removal, chemical
depilatories, oral antibiotics and topical CS for antiinammatory effects, tretinoin can be used to “toughen”
the skin
■
If patient must shave, instruct to gently dislodge
ingrown hairs and shave in direction of hair growth
181
CHAPTER 3 • General Dermatology
Acne keloidalis nuchae
• Males; African American . Latinos . Asians . Caucasians
• Dome-shaped, pruritic, follicular papules on posterior
neck and occipital scalp
• Develop into keloidal papules which coalesce into large
plaques in band-like distribution near posterior hairline;
cicatricial alopecia in areas of involvement (Fig. 3.80)
• Keloidal collagen only present in severe disease; early
disease has scar-like collagen
• Treatment: ↓ mechanical irritation to affected areas;
tretinoin gel plus potent topical CS; intralesional CS; oral/
topical antibiotics if inamed; surgical excision; CO
laser
2
Follicular occlusion tetrad (acne conglobata, hidradenitis suppurativa, dissecting cellulitis of the scalp, and pilonidal cyst)
Hidradenitis suppurativa (acne inversa)
Epidemiology
• 0.3%–1% prevalence
• Starts after puberty
• F . M, but on average more severe in males
• More common in African Americans and those of lower
socioeconomic status
• 35% of patients have rst-degree relative affected
Pathogenesis
• Immune dysregulation (both innate and adaptive
immunity), follicular occlusion
• 1%–2% of patients have familial mutations seen in
gamma-secretase complex (nicastrin, presenilin 1,
presenilin enhancer 2) leading to atypical disease with
diffuse involvement and many cysts
• Associations: obesity, smoking, diabetes, metabolic
syndrome, cardiovascular disease, PCOS, lymphoma,
anxiety, and depression
Clinical features
• Criteria of (1) nodules and abscesses with (2) predilection
for intertriginous locations (axilla, inguinal, anogenital,
and inframammary) (3) with recurrence
• Hurley stage I: recurrent nodules and abscesses without
sinuses or signicant scarring
• Hurley stage II: stage I with the addition of limited sinuses
and scarring
• Hurley stage III: widespread or interconnected sinuses
and/or scarring
• Sinus tracts and double or multi-headed comedones are
classic features
• Chronic thick viscous suppurative drainage; frequently
malodorous; 1/– secondary infection
• Anemia, secondary amyloidosis, lymphedema, stula
formation, and SCC due to chronic scarring
• Cultures most commonly grow skin ora (i.e.,
Staphylococcus epidermidis)
Histopathology
• Suppurative folliculitis with abscess formation, follicular
plugging, granulation tissue, and inammation that can
involve apocrine glands; late stages can have brosis
Treatment/clinical course
• In early stage disease: ↓ weight, ↓ friction/moisture,
oral antibiotics (clindamycin and rifampin, tetracyclines,
R-O-M therapy), topical clindamycin, laser-based
follicular ablation (i.e., Nd:YAG), PDT, and
intralesional CS
• In severe disease: high-quality data for adalimumab (FDA
approved for weekly 40 mg dosing or 80 mg every other
week) and iniximab; lower-quality studies of anakinra,
ustekinumab, apremilast, IL-17 inhibitors (though
secukinumab has phase 3 data) and IL-23 inhibitors;
ertapenem in extreme ares
• In female patients, spironolactone and OCPs can be
considered
• For sinuses, surgical excision, marsupialization,
deroong, and CO2 laser with secondary intention
healing, may also be required
• Variable success: isotretinoin/acitretin, nasteride, MTX,
metformin, colchicine
Fig. 3.80 Acne keloidalis nuchae. Crusted papules and pustules and keloidal
plaques studded with papules on the occipital scalp. (Courtesy of Christopher
Sayed, MD.)
182
Pilonidal cyst
• M . F, 20–40 yo
• Associated with curly hair, obesity, poor hygiene, and
prolonged sitting
• Painful draining sinus in sacrococcygeal region
■
Can be lled with nests of hair
• Treatment: surgical excision; if inamed, oral
antibiotics; moderate success with laser-based follicular
ablation
Dissecting cellulitis of the scalp and acne conglobata (discussed in alopecia and acne sections)
• Discussed in Alopecia and Acne sections of Chapter 3
3.13 Follicular and Eccrine/Apocrine Disorders
Other diseases of eccrine and apocrine sweat glands
Hyperhidrosis
Pathogenesis
• Sweating is a reex controlled through the sympathetic
nervous system; nerves are anatomically sympathetic but
functionally cholinergic
• Primary localized hyperhidrosis is most common type
• 60%–80% have family history with possible autosomal
dominant inheritance pattern for some
• Secondary hyperhidrosis is due to an underlying
condition. There are many causes that can be classied
based on the source of neural impulse:
■
Cortical (emotional)
↑ Neural impulses from the cerebral cortex due to
emotion or sensory stimuli
■
Hypothalamic (thermoregulatory)
Due to ↑ body temperature or direct hypothalamic
stimuli
■
Medullary (gustatory)
Physiologic medullary hyperhidrosis: afferent
impulses from taste receptors stimulate sweating
(spicy foods, alcohol, and citrus fruits)
Pathologic medullary hyperhidrosis:
auriculotemporal or Frey’s syndrome
■
Spinal (cord transection)
Spinal disorders may result in lack of thermal
sweating below the injury, hyperhidrosis at the
injured level, or other unusual patterns of sweating
■
Axon reex (local inammatory)
Direct stimulation of a sympathetic axon can cause
sweating (electrical, physical, or drug-induced)
Mediators from inammatory skin disease (psoriasis
and dermatitis) can elicit localized hyperhidrosis
Clinical features
• Shiny wet skin surfaces or excessive sweat stains on
clothing
• Primary localized hyperhidrosis: palmoplantar . axillary
. forehead
• Secondary hyperhidrosis: can be localized or generalized
• Starch-iodine technique can aid in determining the most
active areas (highlighted by reaction changing to blue/
black color)
Treatment/clinical course
• Topical antiperspirants (aluminum chloride),
iontophoresis, botulinum toxin, systemic anticholinergics
(glycopyrrolate, propantheline, and oxybutynin), topical
glycopyrrolate/glycopyrronium, a-adrenergic blockers
(clonidine), thoracic sympathectomy (risk of compensatory
hyperhidrosis), microwave ablation (miraDry®),
liposuction, and behavioral modication/psychotherapy
Hypohidrosis and anhidrosis
Epidemiology/pathogenesis
• Central or neuropathic diseases (e.g., brain tumors
and spinal cord injuries) or medications (e.g.,
anticholinergics and a-adrenergic blockers) that
disrupt neural impulses
• Congenital alterations of sweat glands (e.g., ectodermal
dysplasias)
• Acquired destruction/atrophy of sweat glands (e.g., burns,
scleroderma, morphea, GVHD)
• Sweat gland obstruction (e.g., miliaria, ichthyoses,
psoriasis, and eczematous dermatoses)
Clinical features
• Skin may appear unremarkable, but there is ↓ or absence
of sweating and resulting hyperthermia
• Attempt to induce sweating (exercising in hot room or
using electric blanket) followed by starch-iodide
technique to demonstrate ↓ or absent sweating
Treatment/clinical course
• Discontinue offending medication(s)
• Avoid hyperpyrexia and maintain a cool environment
• Gentle exfoliation if obstructed sweat ducts
Miliaria
Epidemiology
• Most common in neonates who have not fully developed
eccrine ducts
• Adults in hot humid climates
Pathogenesis
• Excessive sweating causes maceration of the stratum
corneum → eccrine duct obstruction → sweat retention
within the duct
Clinical features
• Table 3.29
Bromhidrosis
• Apocrine bromhidrosis: bacterial degradation of apocrine
sweat yields ammonia and short chain fatty acids;
exaggeration of typical axillary body odor
Table 3.29 Three Types of Miliaria
Type Location of Obstruction Cutaneous Lesions Patient Population Most Common Location
Crystallina Stratum corneum Non-pruritic, clear, fragile, 1 mm
vesicles
Rubra Mid-epidermis Pruritic, erythematous, 1–3 mm
papules; may have pustules
Profunda Dermal–epidermal junction Non-pruritic, white, 1–3 mm papules Adults in hot climates; often with
Neonates , 2 weeks of age
Children and adults in hot climates
Neonates 1–3 weeks of age
Children and adults in hot climates
multiple bouts of miliaria rubra
Face and trunk
Neck and upper trunk
Trunk and proximal
extremities
183
CHAPTER 3 • General Dermatology
• Eccrine bromhidrosis: three types
■
Keratogenic: bacterial degradation of stratum corneum
macerated by excess eccrine sweat
■
Metabolic: abnormal secretion of amino acids or
breakdown products as seen in heritable metabolic
disorders (e.g., phenylketonuria has mousy odor and
maple syrup urine disease has sweet odor)
■
Exogenous: odorogenic compounds such as garlic,
asparagus, curry, DMSO, penicillins
Chromhidrosis
Pathogenesis
• Apocrine chromhidrosis: adrenergic stimuli causes
myoepithelial contractions
• Eccrine chromhidrosis: contamination of colorless eccrine
sweat by a chromogen
Clinical features
• Colored sweat
• Apocrine chromhidrosis:
■
Usually on face and axilla
■
Yellow 5 lipofuscin (if lipofuscin is more oxidized or
concentrated, color can be blue, green, black)
• Eccrine chromhidrosis:
■
Blue or blue/green 5 copper
■
Brown 5 dihydroxyacetone-containing self-tanning
products
■
Red 5 clofazimine and rifampin
■
Brown 5 ochronosis
• Pseudochromhidrosis: sweat stained after excretion on
the skin
■
Corynebacterium (brown), Serratia marcescens (pink),
copper salts (blue)
Fox-fordyce disease (Apocrine miliaria)
• Second degree: two forms
■
Supercial: painful vesicles due to edema of supercial
dermis and epidermis; non-scarring; may take 3 weeks
to heal
■
Deep: pale, anesthetic skin; results in scarring (due to
reticular dermis/appendageal injury)
• Third and fourth degree: third (full-thickness skin
destroyed → ulcer → scar) and fourth (loss of skin and
subcutaneous fat 1/– underlying structures)
■
Grafting usually needed to help with function/contractures
■
May need to excise non-healing tissue
■
Silver-impregnated dressings may help to ↓ infection risk
Silver sulfadiazine absorption can → leukopenia
and argyria (mafenide acetate is alternative)
■
Diligent wound care and monitoring for infection are key
■
More than two thirds of BSA 5 poor prognosis/↑
mortality (women, infants, toddlers)
■
For larger surface areas, will need IV uid resuscitation
Erythema ab igne
• Thick reticulated erythema and/or pigmentation from
chronic, non-burning, heat/infrared radiation exposure to
a particular anatomic site (Fig. 3.81)
• F . M
• Classic sites and causes: shins (space heaters), lower back
(heating pad), and anterior thighs (laptop)
• Possible ↑ SCC risk
Cold injuries
• Acrocyanosis
■
Blue discoloration of hands and/or feet 1/–
hyperhidrosis; ↑ with colder temperatures
Young women mainly
• Plugging of apocrine sweat glands in adolescent/young
adult females
• Extremely pruritic, skin-colored or yellow dome-shaped
follicular papules in apocrine areas (axilla . periareolar
and anogenital) → ↓ hair density
• Treatment is difcult—options: tretinoin, topical CS, TCIs,
topical antibiotics, microwave ablation, and surgical
excision; pregnancy can lead to improvement
3.14 DRUG REACTIONS
See Pharmacology Section
3.15 PHOTODERMATOSES AND OTHER PHYSICAL DERMATOSES
Temperature-related dermatoses
Thermal burns
• Arise due to excess heat on skin
• First degree: erythema 1 epidermal peeling (e.g., ordinary
sunburn)
184
Fig. 3.81 Erythemat ab igne. Site of chronic heating pad use for hip pain demonstrating reticulated hyperpigmentation and erythema as well as scar at areas
of previous ulceration. (Courtesy of Christopher Sayed, MD.)