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Erosive pustular dermatosis
introduction
Scarring alopecia, a diverse group of skin disorders associated
with different clinical presentations, etiology and pathogenesis
(origin and development of disease), is classified into primary
and secondary scarring alopecias. What all these anomalies have
in common is irreversible hair loss, which occurs either insidiously
or rapidly, depending on the specific condition.
With respect to the classification system for primary scarring
alopecia, several methods have been adopted based on different criteria,
to provide a diagnostic framework for dermatologists and clinicians
to identify and treat the disease. Perhaps the most accepted classification
of these disorders is the classification as defined by the North
American Hair Research Society (NAHRS), a categorization that is
based on the principal inflammatory cell type observed in scalp
biopsies taken from active lesions.
Primary alopecias are classified by NAHRS into scarring (cicatricial)
or non-scarring variants. The most basic pathogenic mechanism of
scarring alopecias is believed to be hair follicle stem cell failure.
Follicular stem cells may be destroyed by inflammatory processes
of various immune cell types, predominantly lymphocytic and neutrophilic.
Scarring variants are consequently classified histopathologically
(the study of microscopic changes in diseased tissues) into neutrophilic,
lymphocytic, and mixed types of alopecia, based on the type of inflammatory
infiltrate observed around affected hair follicles.
Erosive pustular dermatosis is an idiopathic (of unknown cause),
chronic relapsing, amicrobial (bacteria is not involved), pustular
(a visible collection of pus within or beneath the epidermis)
skin disease of the scalp, classified by the North American
Hair Research
Society as a mixed alopecia. Tiny pustules (small inflamed skin
swellings that are filled with pus) form on the scalp, forehead
or temples of the affected persons. The pustules are usually sterile
(uninfected) but they can become secondarily colonized by bacteria
such as Staphylococcus aureus after the condition has developed.
This particular disorder primarily occurs in older Caucasian women.
Therefore, erosive pustular dermatosis manifests as a pus-filled,
necrotic (necrosis is the death of cells or tissues) folliculitis
(inflammation of a follicle or follicles) of the infundibulum
(the upper part of a hair follicle) with crusting of the skin.
Later
features are similar to those of folliculitis decalvans with eventual
scarring and this can progress to extensive balding. Folliculitis
decalvans has been described as a cicatrical alopecia characterized
by erythematous scalp with pustules around the hair follicles.
A variant of erosive pustular dermatosis with chronic swelling
and
erosions without scale in the scalp has been reported in younger
patients of African ethnicity, but there is no clear indication
if the two conditions are related.
The cause of erosive pustular dermatosis of the scalp is unknown.
The predominance of disease in the elderly has led some to the
opinion that chronic damage to the scalp by UV sun rays may
be a predisposing
factor. The condition may also possibly be triggered by a minor
injury to the affected skin. Specific precipitants documented
by researchers in the field include minor lacerations (tearing),
contusions
(a bruise, an injury of a part without a break in the skin), accidental
scalping, sunburn, varicella zoster (a virus), skin grafting,
radiation, synthetic hair fiber implantation, cryotherapy (the
therapeutic
use of cold to reduce discomfort in, for example, laser hair removal),
topical fluorouracil (an agent used in the treatment of cancers)
and topical tretinoin (a form of retinoic acid). Prompt diagnosis
and aggressive therapeutic intervention is required to prevent
a damaging situation.
Immunologic studies (a subfield of biology that deals with the
study of antigens and the immune process and how humans and higher
animals fight off disease) have suggested that in people affected
by erosive pustular dermatosis, there may be a defective immunologic
response to infectious organisms such as Staphylococcus Aureus.
Case reports and literature reviews have identified defective lymphocytes,
increased amounts of immunoglobulin antibodies, abnormal neutrophilic
chemotaxis (the phenomenon in which body cells, bacteria, and other
single-celled or multicellular organisms direct their movements
according to certain chemicals in their environment), and hypocomplementia
as possibilities. These problems can potentially cause severe illness.
Dermatosis is a broad term that refers to any disease of the skin,
especially one that is not accompanied by inflammation. The term
should not be confused with dermatitis, which is limited to inflammation
of the skin. A similar condition to erosive pustular dermatosis
may arise on the legs, but some authors have attributed these manifestations
to a different disease.
Erosive pustular dermatosis
clinical features
In the case of all alopecia diagnoses, examination of the entire
scalp to view the scarred and normal scalp areas is crucial to diagnosis
and treatment. The clinical presentations often provide valuable
clues in identifying the particular form of hair loss. Thereafter
confirmation of the diagnostic impression by biopsy is recommended.
In cases where preceding trauma has been identified, disease presentation
can occur immediately or take months or years to develop. The characteristic
lesion of erosive pustular dermatosis is a large, asymptomatic,
well-demarcated plaque with a superficial crust. The crust can be
easily removed to reveal flabby pustules that exude pus. In some
cases, moist erosions or crusts in the absence of pustules have
also been seen.
Untreated lesions undergo periodic pustular flares, and slowly
enlarge over years. Advanced disease culminates in cicatricial alopecia,
the extent of which cannot be appreciated until the lesion is healed
with treatment. Wounds may be colonized by staphylococcal species
and less often, by the fungus Candida. Aggravation of disease has
been reported with attempts at reparative skin grafting and treatment
of surrounding actinic keratoses (lesions of skin associated with
ultraviolet irradiation). Development of secondary carcinoma with
squamous and basal cell features can also occur if the condition
is further aggravated.
Erosive pustular dermatosis
differential diagnosis
Diagnosis of erosive pustular dermatosis involves comprehensive
knowledge of hair anatomy, recognition of clinical features and
a profound insight into the various forms of alopecia and their
patterns of manifestation. Very often some forms of alopecia show
overlapping symptoms, and it may be difficult to differentiate one
condition from another during the course of diagnosis.
The differential diagnosis of erosive pustular dermatosis is extensive
and includes the following:
- amicrobial pustulosis associated with autoimmune disease:
An eruption involving the cutaneous flexures and scalp, reported
in rare cases.
- pustular ulcerative dermatosis of the scalp:
A rare, non-crusted ulcerative dermatitis that affects malnourished
young African males.
- pyoderma gangrenosum: A chronic skin disease,
usually of the trunk, characterized by large spreading ulcers.
- pustular
psoriasis: An uncommon form of psoriasis consisting of widespread
pustules
- kerion: Fungal infection of the hair follicles accompanied
by secondary bacterial infection and marked by raised, usually
pus-filled and spongy
lesions.
- bacterial folliculitis: Superficial or deep bacterial infection
and inflammation of the hair follicles
- cicatricial pemphigoid: A group of rare
chronic autoimmune blistering diseases that predominately affect
the mucous membranes
- pemphigus vulgaris: Skin disease characterized
by groups of itching blisters
- blastomycosis-like pyoderma: A
rare skin lesion
- erosive candidiasis of the scalp
- temporal arteritis: Arterial
inflammation that occurs in older persons and that is characterized
by the presence of multinucleated
giant cells in temporal, retinal, or intra-cerebral arteries
Both clinical and pathological features should be co-related by
the dermopathologist or clinician before arriving at a conclusive
diagnosis.
Erosive pustular dermatosis
pathology
In most kinds of alopecia, scalp biopsies provide critical information
to the assessment and diagnosis of the patient. The rule of the
thumb is to take skin biopsies from advanced areas of alopecia to
evaluate the pattern of elastic tissue loss, to confirm follicular
loss, and to establish whether there is potential for re-growth
of hair or not. In cases like erosive pustular dermatosis, when
superficial pustules are present on the scalp, a combination of
two or more biopsies (a procedure that involves obtaining a tissue
specimen from the skin for microscopic analysis to establish a precise
diagnosis) for both transverse as well as vertical sectioning may
be required to reflect the complete histological picture.
In the case of erosive pustular dermatitis, histopathologic features are rather
non-specific. It has been found that extensive chronic and acute inflammation
involves the follicles, the sebaceous glands, other adnexa, and the interstitial
dermis (situated between parts or in the interspaces of the skin tissues).
Dense, chronic mixed inflammatory cells infiltrate (lymphocytes and neutrophils)
and occasionally foreign-body giant cells occupy the dermis.
The pathological findings of late stage biopsies are dermal fibrosis,
loss of the follicular units, and remnants of the arrector pili,
the tiny muscle fibers attached to each hair follicle, may be visible.
Erosive pustular dermatosis
treatment
Awareness of the existence of this condition by the dermatologist
is important for management and prognosis of the patient. The choice
of treatment depends on age, severity and extent of disease. As
a rule, the treatment approach for erosive pustular dermatosis begins
with local treatment for primary stages of disease. Systemic medication
is administered in rapidly advancing, extensive disease, or when
the condition is unresponsive to other forms of treatment. At the
moment, the available therapeutic methods offer only limited success
in slowing down the progress of this disease.
The most common treatment approach involves corticosteroids. The
use of class I and class II steroids have shown rapid improvement
of erosive pustular dermatosis. Sustained therapy is required for
long-lasting effect of treatment.
The following should be noted:
- Oral and topical antibiotics have provided transient benefits,
especially for secondary infection.
- Calcipotriol cream as a potential
alternative to steroids has been found to induce remission.
- Effective use of Zinc sulphate has been documented in the treatment
of erosive pustular dermatosis.
- Oral isotretinoin, a chemical compound
that inhibits the secretion of sebum, and dapsone (antibacterial
drug) are ineffective in the treatment
of Erosive pustular dermatosis of the scalp.
- Patients with erosive pustular
dermatosis of the scalp should see their medical practitioner
or dermatologist regularly as negligence in treatment
may lead to development of new keratoses and skin cancers in the affected
areas.
Erosive pustular dermatosis of the scalp is a rare and chronic
dermatosis of unknown etiology with non-specific histology. The
condition represents a distinct disease with a history of relapsing
and shows unsatisfactory response to common treatments, necessitating
the use of steroids.
Erosive pustular dermatosis
references
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