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Dissecting cellulitis
introduction
The term "cicatricial alopecia" refers to a diverse group
of rare disorders that destroy the hair follicle, replace it with
scar tissue, and cause permanent hair loss. In some cases, hair
loss is gradual, without symptoms, and is unnoticed for long periods.
In other cases, hair loss is associated with severe itching, burning
and pain and is rapidly progressive. Cicatricial alopecias are classified
as primary or secondary. In primary cicatricial alopecia the hair
follicle is the main target of the destructive inflammatory process.
In secondary cicatricial alopecia, destruction of the hair follicle
is incidental to a non-follicle directed process.
Primary cicatricial alopecias are further classified by the type
of inflammatory cells that destroy the hair follicle during the
progress of disease. The inflammation may predominantly involve
lymphocytes or neutrophils, and primary cicatrical alopecias are
hence classified as lymphocytic or neutrophilic. Dissecting cellulitis
comes under the category of neutrophilic alopecias.
Dissecting cellulitis (also called perifolliculitis capitis abscedens
et suffodiens) manifests with perifollicular pustules, nodules,
abscesses and sinuses that evolve into scarring alopecia. As the
condition worsens, there is progressive hair loss on the scalp.
The hair loss seen in dissecting cellulitis predominantly occurs
in African American men especially in the age-group 20 to 40 years.
Occasionally, the condition affects other ethnicities as well as
women. Dissecting folliculitis, dissecting perifolliculitis, perifolliculitis
capitis and Hoffman disease are all synonyms for the same condition.
The presence of hair follicles is evidently essential for disease
progression, because if all follicles growth activity is inhibited,
as seen with X- ray exposure epilation, the disease appears to rapidly
subside.
Dissecting cellulitis may occur alone or in conjunction with the
other members of the follicular occlusion triad, namely acne conglobata
and hidradenitis suppuritiva. The physiopathology is believed to
involve follicular blockage in all these conditions. As material
accumulates in the follicle, the follicle dilates and then bursts.
Keratin and bacteria from the ruptured follicles can set off a neutrophilic
and granulomatous response. It is very likely that there is a primary
inflammatory process with secondary bacterial infection. There are
also suggestions that the disorder may be an abnormal host response
to bacterial antigens. As in the case with the scarring alopecia
folliculitis decalvans, S. Aureus is the most commonly isolated
organism from dissecting cellulites pustules.
Dissecting cellulitis
clinical features
Clinical evaluation of the scalp is crucial to the diagnosis of
the particular type of scarring alopecia. However, a typical case
of dissecting cellulitis is relatively easy to diagnose, unlike
some other forms of scarring alopecia. The initial lesion is a follicular
pustule often found on the vertex. This then transforms into a painful,
bulbous, firm or fluctuant nodule (aggregation of cells). Nodules
are said to be fluctuant when a wavelike motion is felt on palpating
the nodule.
As the disease progresses, most of the scalp is covered by boggy
or fluctuant nodules, especially the crown and the vertex. Pressure
on the nodule often releases pus or serosanguineous material (consisting
of serum and blood) directly from the lesion itself, or from an
adjacent or interconnected nodule. A non-scarring alopecia initially
develops over the nodules.
Advanced stage of the disease results in cicatricial alopecia marked
by depressed, hypertrophic or keloidal scars. Hypertrophic scars
occur when the fibrous tissue that replaces normal tissue is destroyed
by injury or disease. Keloids are red, raised formations of fibrous
scar tissue caused by excessive tissue repair in response to trauma
or incision.
Dissecting cellulitis can wax and wane in severity for many years,
but eventually culminates in dense dermal fibrosis, sinus tract
formation, hypertrophic scarring (as described) and permanent hair
loss. Although spontaneous remission can occur, chronic relapse
is equally possible. Cervical or occipital lymphadenopathy (chronic,
abnormal enlargement of the lymph nodes) may be present.
One third of the reported cases of dissecting cellulitis show the
coexistence of other diseases called acne conglobata (a severe form
of acne) and hidradenitis suppuritiva (a condition where the apocrine
sweat glands become blocked and inflamed, particularly under the
arms and around the groin). In such cases, asymmetric peripheral
and axial joint involvement is not unusual. Active skin disease
usually precedes onset of arthritis and mirrors acute worsening
of all the symptoms.
Dissecting cellulitis
differential diagnosis
The distinctive clinical appearance of the disease and the fact
that dissecting cellulites (Perifolliculitis capitis abscedens et
suffodiens) occurs predominantly in adult black men makes definitive
diagnosis rather simple, as compared to other forms of scarring
alopecia. A patient with typical scalp lesions associated with acne
conglobata or hidradenitis suppuritiva can quite easily be diagnosed
as having dissecting cellulitis.
Often dissecting cellulitis occurs without the other members of
the occlusion triad, and might then be confused with a highly inflammatory
case of central centrifugal scarring alopecia (CCSA). Occasionally,
a patient may have features of both CCSA and dissecting cellulites
together, and it is unclear whether the patient has two different
diseases concurrently or whether there is an unusual presentation
of one or the other.
Dissecting cellulitis differs from Pseudopelade of Brocq by its
lack of skin atrophy and "foot prints in the snow" alopecia
morphology.
Some authors have observed inflammatory tinea capitis in children
and adolescents that closely resembles dissecting cellulitis. Tinea
capitis is a disease caused by superficial fungal infection of the
skin of the scalp, eyebrows, and eyelashes, with a tendency to attack
hair shafts and follicles. There is also a report of fatal folliculotropic
mycosis fungoides (MF) with follicular mucinosis and large-cell
transformation mimicking dissecting cellulitis.
Dissecting cellulitis
pathology
A scalp biopsy is a valuable tool in the diagnosis of dissecting
cellulitis and helps to differentiate the disease from conditions
that mimic the same clinical symptoms. The histological findings
are dependant on the stage of the disease.
In early disease, there is acne-like swelling of the follicular
infundibulum with perifollicular, mixed, neutrophilic and lympho-plasmacytic
inflammation. The inflammatory process tends to involve the lower
portion of the dermis and the subcutaneous junction, and so the
lower portion of the terminal scalp follicles is most affected.
Inflammatory cells invade the follicular epithelium, which eventually
leads to follicular destruction. Some follicles which are affected
by acute inflammation are forced into the catagen /telogen phase
of the hair cycle. To elucidate, the catagen phase of the hair growth
cycle is the transitional or regressive phase, when the hair stops
growing. The telogen is the final resting stage, or ‘off’ phase
of the hair growth cycle. This forcing of the hair follicles in
a resting state results in hair shaft shedding without follicular
destruction. The fact that there is no follicular destruction probably
explains the fact that significant hair re-growth occurs when prompt
and effective treatment is initiated in the early stages of the
disease. If fluctuant nodules are sampled, large perifollicular
and deep dermal abscesses composed of neutrophils and numerous plasma
cells are found. Intact and seemingly normal follicles may be acutely
inflamed as well.
With time, the chronic abscesses become lined with squamous or
scaly epithelium, and true sinus tracts – a characteristic
of dissecting cellulitis form. End stage disease is characterized
by complete destruction of the follicles. The inflammation subsides
and the scalp area is replaced by fibrosis of the dermis and subcutis,
surrounding sinus tracts.
Dissecting cellulitis
treatment
Dissecting cellulitis, or Perifolliculitis capitis abscedens et
suffodiens, responds unpredictably to treatment. For this reason
it is important that the condition be evaluated and diagnosed by
a dermatologist skilled in scalp and hair disorders. A thorough
evaluation with respect to all diagnostic parameters is important
before coming to a conclusive diagnosis and commencing therapy.
In general, early intervention with treatment is best. If treatment
is started early enough it is possible to reverse the disease before
hair follicles become permanently damaged or destroyed.
Isotretinoin and oral zinc have shown results as single agent therapies.
Oral isotretinoin is regarded as first line therapy and, in a small
number of cases, remission of disease has been reported. With this
line of treatment, flattening of nodules takes time, and a slow
response should not be a cause for premature discontinuation of
treatment. Dosing below the optimal levels, as well as inadequate
treatment duration, can reduce the efficacy of the therapy.
Antibiotics and oral, topical or intralesional corticosteroids
may be considered. Oral and topical antibiotics (such as tetracyclines
or anti-staphylococcal agents), antibacterial soaps and intralesional
triamcinolone acetonide are variably effective when used as single
agents or in combination with other therapies. Often, combination
therapy makes the treatment sufficiently effective. A combination
of incision and drainage, corticosteroids, antibiotics and isotretinoin
usually produces good results.
Other treatments that have been used successfully include X-ray
epilation, in which there is removal of the entire hair, including
the part below the skin. Although it is extreme and there are risks
with X-ray exposure.
Present day medical experts are in favor of incision and drainage
of painful nodules or excision laser surgery. Surgical excision
of lesions can be considered in severe or intractable cases. Laser
epilation has been advocated as a therapeutic option in the case
of severe disease that is past the stage of responding to treatment.
Treatment should never be discontinued immediately; instead, drug
doses should be gradually tapered. All forms of treatment have undesirable
side effects, and the therapist should closely monitor the well
being of the patient and the progress of treatment throughout the
course of therapy.
In conclusion, it can be said that dissecting cellulitis or perifolliculitis
capitis abscedens et suffodiens is a rare, chronic, progressive,
suppurative disease of the scalp and of unknown pathogenesis. The
disease manifests as painful nodules, purulent drainage, burrowing
interconnecting abscesses, and cicatricial alopecia. The etiology
is obscure, although it is probably related to follicular blockage,
secondary infection, and deep inflammation.
Dissecting cellulitis
references
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