ARTICLE
A 50-year-old man without previous medical history had an isolated pruriginous
palmoplantar hyperkeratosis, evolving over 4 years. Clinically, this hyperkeratosis
was symmetric and well delineated. The borders were hyperkeratotic and
lichenoid hyperkeratotic papules were seen on the dorsal area of the palms
and soles. By coalescence these lichenoid hyperkeratotic papules sometimes
had a reticulated pattern and formed yellow-grey plaques on the palmoplantar
area (Figs. 1
to 3). Nail abnormalities were observed as longitudinal
ridges with red discoloration and slight distal onycholysis (Fig.
4). No consistent associated changes of the hair, teeth or eyes
and no mucous membrane involvement were noted. A cutaneous biopsy of a
lichenoid hyperkeratotic papule was performed and showed hyperkeratosis
with focal parakeratosis, acanthosis as well as atrophy of the epidermis.
A band-like dense lymphohistiocytic infiltrate in the upper dermis with
focal degeneration of the basal cell layer was observed (Fig.
5).
Isolated palmoplantar
hyperkeratosis revealing keratosis lichenoides chronica
Comments
Keratosis lichenoides chronica (KLC) first described
by Kaposi in 1895 and called "lichen ruber verrucosus et reticularis"
is considered as a rare and unique entity with characteristic clinical
and histopathological features although some authors have commented that
KLC may represent a variant of lichen planus. No evidence of pathological
association has been clearly established yet.
The clue for the diagnosis is the presence of
lichenoid violaceous keratotic papules arranged in plaques or in a characteristic
linear or reticular pattern. KLC usually begins in adult life with a mean
age of 28 years and is asymptomatic but a few patients complain of pruritus.
KLC affects the dorsal part of the extremities and the trunk symmetrically
and, on the face, an eruption mimicking a seborrheic dermatitis or rosacea
is reported in about 75% of cases. Palmoplantar hyperkeratosis is present
in about half the cases but is rarely isolated [1]. Nails changes including
onycholysis, thickening, ridging of the nail plate, brownish discoloration,
hyperkeratosis of the nail bed and, in particular, warty hypertrophy of
the nail folds occur in one-third of patients. Additional clinical features
such as erosive gingivostomatitis, cheilitis, oral aphtoid ulcerations,
nodular infiltrate of the soft palate and epiglottis, hoarseness, genital
involvment such as chronic balanitis and phimosis and eye involvment including
blepharitis, conjunctivitis, keratoconjunctivitis and anterior uveitis
are reported [1, 2]. Recently, a mycosis fongoides simulating a typical
KLC has been reported [3].
Histopathological findings typically showed
hyperkeratosis of the ortho- and para-type, an alternating atrophic and
acanthotic malpighian cell layer and a hydropic degeneration of the basal
cell layer resulting in cleft formations and a band-like mononuclear infiltrate
in the upper dermis (Fig. 6).
Follicular and eccrine duct hyperkeratinisation with perforation into
the surrounding dermis have also been observed. Recently, Kossard et
al. described in a 70-year-old woman with a diagnosis of KLC, a lichenoid
tissue reaction which was centred on the acrosyringia and the eccrine
ducts as they entered the epidermis. For Kossard et al., the clinical
findings defining KLC, exhibit a heterogeneous group including this variant
of lichen planus which they called lichen planoporitis [4].
The clinical course of KLC is chronic, progressive and usually resistant
to most therapeutic approaches [5, 6]. Two cases of spontaneous resolution
have been observed [7].
REFERENCES
1. Masouyé I, Saurat JH. Keratosis lichenoides chronica: the centenary
of another Kaposi's disease. Dermatology 1995; 191: 188-92.
2. Ezzine-Sebai N, Fazaa B, Mokhtar I, Piérard-Franchimont C,
Piérard GE, Kamoun R. Keratosis lichenoides chronica: an unusual
case. Dermatology 1996; 192: 416-7.
3. Bahadoran P, Wechsler J, Delfau-Larue MH, Gabison G, Revuz J, Bagot
M. Mycosis fongoides presenting as keratosis lichenoides chronica. Br
J Dermatol 1998; 138: 1067-9.
4. Kossard S, Lee S. Lichen planoporitis: keratosis lichenoides chronica
revisited. J Cutaneous Pathol 1998; 25: 222-7.
5. Konstantinov KN, Søndergaard J, Izuno G, Obreshkova E. Keratosis
lichenoides chronica. J Am Acad Dermatol 1998; 38: 306-9.
6. Grunwald MH, Hallel-Halevy D, Amichai B. Keratosis lichenoides chronica:
response to calcipotriol. J Am Acad Dermatol 1997; 37: 263-4.
7. Van de Kerkhof PCM. Spontaneous resolution of keratosis lichenoides
chronica. Dermatology 1993; 187: 200-4.
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