Liken amiloidoz'un kriyocerrahiyle başarılı tedavisi
Introduction
Lichen amyloidosis (LA) is an
unusual, persistent pruritic papular
eruption, characterized by cutaneous
amyloid deposition without systemic
involvement. The clinical features
consist of discrete, intensely pruritic,
hyperkeratotic skin colored or yellowish brown papules that may coalesce into plaques, mainly located onCilt 48 · Sayý 2 · Gülhane TD Lichen amyloidosis · 113
Ultrasonography of the thyroid and
surrenal glands revealed no pathological findings.
A 4 mm punch biopsy specimen
revealed orthokeratosis, hypergranulosis, irregular acanthosis, and a perivascular lymphohistiocytic inflammatory infiltrate and melanophages
in the superficial dermis. In the papillary dermis, focal collections of
amorphous eosinophilic-staining material that stained positive with crystal
violet were present (Figure 2). A
diagnosis of LA was established and
the patient was treated with liquid
nitrogen cryosurgery using a 15 second, single freeze thaw cycle. One
month later there was prominent
regression of the papular lesions, and
a second session of cryosurgery was
carried out. At the end of the second
month, the patient was symptom
free, and a near complete resolution
of the lesions was achieved (Figure
3). A side effect of hypopigmentation
was also observed in the treatment
site. A control biopsy specimen of the
previously involved skin showed
clearance of amyloid deposits in the
superficial dermis. No recurrence
was noted during 6 months of follow-up.
Discussion
The term amyloidosis refers to
the extracellular deposition of amyloid, which is normally a soluble
autologous protein, in a characteristic
abnormal form (6). Primary cutaneous amyloidosis is characterized by
the deposition of amyloid in a previously apparently normal skin without
internal organ involvement. Primary
cutaneous amyloidosis has been classified into three types: macular,
lichen (papular), and nodular (tumefactive form) amyloidosis (7).
Unusual rare types, which include
poikiloderma-like cutaneous amyloidosis, bullous amyloidosis and vitiliginous amyloidosis have been reported in the literature (7). LA is seen
more frequently in South-east Asia,
China and some South American
countries (8). The course of LA is
unknown, but genetic factors based
on familial cases of LA, Epstein-Barr
virus and chronic friction have been
implicated in the etiology of the disease (1,9,10). According to the apoptosis theory, degenerated keratins
from apoptotic keratinocytes are
transformed into amyloid by dermal
macrophages and fibroblasts (11).
Yamagihara et al. considered that
cutaneous amyloid deposits might
also be secretory products of keratinocytes (12). The presence of amyloid K, an epidermal keratin derived
amyloid suggests that the amyloid is
derived from keratin peptides originating in the epidermis in LA (9).
The lesions of LA usually occur in
a symmetrical distribution as multiple, scaly, closely set papules, some
with a lichenified surface, mainly
located on the lower legs, especially
around the ankles, and, to a lesser
extent, the trunk and the upper
extremities. Atypical localization with
involvement of the vulva, ears and
buttocks and generalized cases have
also been described (13-15).
Histological features include
eosinophilic globular masses of
amorphous material in the papillary
dermis that stain positively for amyloid with Congo red and crystal violet
stains. Hyperkeratosis, hypergranulosis, slight degree of epidermal hyperplasia, colloid bodies, basal cell vacuolar degeneration, melanophages,
and a mild superficial and perivascular lymphocytic infiltrate in the dermis may also be observed (6,9). The
findings of excoriated, hard and
hyperkeratotic papules on the anterior aspect of the shin as well as the
deposition of amorphous amyloid
material in the papillary dermis was
consistent with a diagnosis of LA in
our case.
LA is reported in association with
several disorders including multiple
endocrine neoplasia type 2A (Sipple
syndrome), atopic dermatitis, lichen
planus, mycosis fungoides, angiolymphoid hyperplasia with eosinophilia,
Kimura's disease and Alagille syndrome (2,16-18) No associated disFigure 1. Discrete, scaly, hyperkeratotic
papules on the shin
Figure 2. Arrows indicating focal collections
of amorphous material in the papillary dermis
consistent with lichen amyloidosis (crystal violet stain, X80)
Figure 3. Clearance of lesions with postinflammatory hypopigmentation after cryosurgery114 · Haziran 2006 · Gülhane TD Sezer ve ark.
ease has been observed in our patient
despite detailed clinical and radiological examinations.
The treatment of LA is frequently
disappointing. Topical corticosteroids have no effect on papular
lesions, except for variable improvement in pruritus. Other treatment
modalities including intralesional
corticosteroid injection, topical
tacrolimus, topical Capsaicin, oral
and topical dimethylsulfoxide (DMSO), photo(chemo)therapy, retinoids, hydrocolloid dressings, dermabrasion, carbon dioxide laser ablation have been tried with mixed
results (3-5,19,20). In LA, very limited data regarding effectiveness of
cryosurgery exist. Hallel-Halevy et al.
have reported a case of LA treated
with topical corticosteroid preparation, retinoic acid 0.05% cream,
cryosurgery, and systemic antihistamines with only minor effect, however the technique and freeze time of
cryosurgery have not been described
(12). In contrast, an open spray technique of liquid nitrogen cryosurgery
with two sessions of a single 15 second, freeze thaw cycle resulted in
complete resolution of pruritus and
also clearance of amyloid deposits in
the papillary dermis. The mode of
action of cryosurgery in lichen amyloidosis should be explained by the
destructive mechanism of this treatment modality (21). Cryosurgery is
well-known to produce a destructive
effect in the skin tissue depending on
the depth of freeze, thus resulting in
destruction of amyloid deposits located in the superfical dermis in lichen
amyloidosis. The only side effect was
postinflammatory hypopigmentation
on the treatment site, which was
neglected by the patient.
In conclusion, we consider that an
open spray technique of cryosurgery
may be considered in the therapy of
localized LA resistant to other treatment options.