ARTICLE
A 37-year-old surdo-mute woman was referred to our department for the
diagnosis of chronic and relapsing inflammatory lesions of the scalp which
had been present for 7 years. She complained of intermittent itching and
sticky hair within the affected area. Her medical history included deafness
with resulting mutism following meningitis of unknown origin at the age
of 10 months and chronic rhinitis leading to hypertrophy of the turbinals.
The clinical examination revealed a circumscribed area of 20 cm length
and 8 cm width on the left fronto-parietal side of the scalp which was
characterized by sticky hairs and adherent yellow-white crusts. When crusts
and hair were removed, the scalp was erythematous, partly erosive and
showed flesh-coloured patches of scarring with consecutive alopecia surrounded
by inflamed margins. Remaining follicular openings were enlarged and were
characterized by containing 2-5 hairs per infundibulum. Staphylococcus
aureus was cultured from several swabs of the affected lesion by superficial
and deep sampling. Routine biochemical and haematological investigations
including peripheral immunophenotyping of lymphomononuclear cells, immunoglobulins
and phagocytic capacity were normal.
Tufted hair folliculitis
The biopsy specimen demonstrated subepidermal edema, infiltration with
neutrophils, eosinophils, plasma cells and granulomatous tissue formation.
The biopsy was characterized by tufts of hair consisting of a central
anagen hair surrounded by telogen hairs. The surrounding tissue was fibrotic,
early scarring events could be seen. All topical and systemic antimicrobial
and antimycotic treatments had failed before, but i.v. antistaphylococcal
antibiotic plus corticosteroid lowered the activity of the disease. However,
the psychoreactive distress led the patient to ask us for therapy. Due
to the widespread lesions and the inherent frequent relapse of this disease
we excised parts of the affected area (15 x 2 cm) in general anaesthesia.
As the elasticity of the scalp was markedly reduced as a result of the
long, chronic and granulomatous inflammation, the defect was partly closed
by skin mesh-graft from the right thigh. The postoperative course was
unremarkable and so far there has been no relapse within the skin-grafted
area. There are some remaining lesions present but they are small and
stabilized with topical treatment only. So far, the patient has expressed
no desire for surgical treatment of the remaining lesions.
Discussion
Tufted hair folliculitis is a very rare recurrent and progressive scarring
folliculitis of the scalp characterized by multiple hair tufts leading
to patches of scarring alopecia with remaining tufted hairs. Each tuft
consists of up to 10 to 15 hairs, each arising from its own follicle but
all converging towards a single orifice in the epidermis. It mainly occurs
in young men and affects the parietal and occipital region of the scalp.
Differential diagnosis in the early stages mainly includes folliculitis
decalvans, folliculitis keloidalis nuchae, kerion
celsi, perifolliculitis abscedens et suffodiens (Hoffmann), trichostasis
spinulosa, follicular lichen planus and relapsing staphylococcal folliculitis.
Dermatopathology reveals superficial and deep folliculitis involving several
follicles opening into a common ostium from which multiple hairs emerge
[1-3]. Various pathogenetic mechanisms have been discussed including nevoid
abnormalities [1, 3], recurrent infections of the follicles [2] and retention
of telogen hair in the tufts [4]. Others suggest a distinctive clinicohistological
variant of folliculitis decalvans [5]. The mechanism of tuft formation
is peculiar and consists of clustering of several adjacent follicular
units caused by a fibrosing process and retention of telogen hairs within
the involved follicules [5]. It must be emphasized that compound hairs
(2-3 anagen hairs emerging from a single infundibulum), which are also
called "follicular units" [6] occur normally in the occipital scalp and
may also be associated with folliculitis. It remains to be determined
whether tufted hairs represent a nevoid malformation [1, 3] or an acquired
phenomenon [2, 4, 7]. Therapy is difficult as
the course of the disease is chronic and recurrent. In case of S. aureus
a sufficient antibiotic treatment needs to be undertaken which in most
cases needs to be repeated over months or years. It may reduce inflammtion
but has no effect on the tufts of hair. In the case of circumscribed lesions
and insufficient improvement by antibiotic therapy the excision of the
affected area is the therapy of choice, leading to a long-term benefit.
New lasers such as erbium-YAG are probably an alternative to removing
the hair bundles but this has yet to be proved.
REFERENCES
1. Metz J, Metz G. Nävoide Bündelhaare beim Menschen. Hautarzt
1978; 29: 586-9.
2. Smith NP, Sanderson KV. Tufted folliculitis of the scalp. J R
Soc Med 1978; 71: 606-8.
3. Tong AKF, Baden HP. Tufted hair folliculitis. J Am Acad Dermatol
1989; 21: 1096-9.
4. Dalziel KL, Telfer NR, Wilson CL, Dawber RPR. Tufted folliculitis.
A bacterial disease? Am J Dermatopathol 1990; 12: 37-41.
5. Annessi G. Tufted folliculitis of the scalp: a distinctive clinicohistological
variant of folliculitis decalvans. Br J Dermatol 1998; 138: 799-805.
6. Headington JT. Transverse microscopic anatomy of the human scalp.
Arch Dermatol 1984; 120: 449-56.
7. Luz Ramos M, Munoz-Perez MA, Pons A, Ortega M, Camacho F. Acne keloidalis
nuchae and tufted hair folliculitis. Dermatology 1997; 194:
71-3.
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