Professional Documents
Culture Documents
A
crodermatitis continua of Hallopeau (ACH) is a and ventral aspects of several fingers and dorsal aspect
rare, chronic disease characterized by pustular of several toes, with associated nail dystrophy (figure 1).
eruptions predominantly involving the distal pha- There were no skin lesions elsewhere. Earlier treatment
langes of the hands and feet with marked involvement of had included topical antibiotics, antimycotics, potent cor-
the nail bed [1]. Atrophic skin changes, onychodystophy ticosteroids, calcipotriol as well as methotrexate (15 mg/
and osteolysis are frequently present, causing painful and week for one year at age 21) without any success. The
disabling lesions [2]. ACH is generally considered as a patient’s past medical history was otherwise unremarkable
rare variant of pustular psoriasis, though some authors and he had no personal or family history of psoriasis.
classify it as a separate entity [3]. Treatment of ACH is Clinical and microbiological examination gave no evi-
very difficult and often disappointing. Phototherapy is one dence for bacterial or fungal infection. Histological fea-
of the safest and most effective treatments for psoriasis tures of psoriasis were found in a biopsy specimen from
[4]. The use of narrowband ultraviolet B (NB-UVB) lesional skin. X-ray examination revealed no osteolytic
phototherapy, with a peak emission wavelength at changes of the bones or joint deformities. Based on the
311 nm, has been used for the treatment of psoriasis and clinical and histopathological findings, ACH was diag-
other inflammatory disorders for more than 20 years [5]. nosed. Because of the disease progression, which led to
NB-UVB has been reported to be more effective than severe disability, the lack of response to previous treat-
broadband (BB) UVB [4] and as effective as systemic ments, rejection of systemic medication by the patient
psoralen plus UVA (PUVA) in psoriasis [5]. Topical 8- and in reference to a previous report on the successful
methoxypsoralen (8-MOP) has been reported to enhance use in psoriasis [7], our patient was given twice weekly
the therapeutic results of targeted NB-UVB [6]. Topical 8- local NB-UVB phototherapy with preceding 0.1% 8-MOP
MOP/NB-UVB has been used successfully to treat psori- over the lesional areas.
asis [7]. The photoelectrical device used in the treatment (Daavlin,
We describe a patient with ACH who responded to the Bryan, OH, USA, 4356) had a local system with a hand
treatment of topical 8-MOP/NB-UVB successfully. To and foot unit and contained 8 lamps; 4 emitting UVA
the best of our knowledge, we report the first case of suc- (Voltarc F24T12/B4HO 16121) and the other 4 emitting
cessful treatment of ACH with topical 8-MOP/NB-UVB. NB-UVB (Philips TL-01 20W/01RS). We used the device
only equipped with Philips TL-01 lamps and NB-UVB
was applied only in combination with topical 8-MOP.
Case report
doi: 10.1684/ejd.2009.0726
Discussion
The treatment of ACH is known to be difficult. Lesions of
ACH are painful and disabling and associated with skin
atrophy, onycodystrophy and osteolysis. Histologically,
the characteristic feature of ACH is a subcorneal cavity
filled with polymorphonuclear neutrophils [8]. Because
of the histopathological similarity of ACH with pustular
psoriasis, it is generally accepted as a variant of localized
pustular psoriasis [1, 9, 10]. As high numbers of T lym-
phocytes and neutrophilic granulocytes infiltrating the
skin lesions play a major pathophysiological role in
ACH, most treatment regimens aim at downregulating the
A disease-related proinflammatory cytokines [11]. Unfortu-
nately, no therapeutic guidelines exist, only anecdotal
observations have been reported in the literature and
every successfully treatment of ACH contributes to the
therapeutic armamentarium [3, 9, 12]. For the treatment
of ACH, various topical and systemic agents, as well as
combined schemes, have been used. Topical treatments
such as corticosteroids, tar, dithranol, fluorouracil, calci-
potriol and calcineurin inhibitors have been used in com-
bination with systemic drugs or alone [11]. Retinoids,
cyclosporine, methotrexate, tetracycline, dapsone, colchi-
cine, anti-tumour necrosis factor-α (TNF-α) agents and
PUVA phototherapy have been reported as systemic ther-
apies [1-3, 8-12]. Topical 8-MOP/NB-UVB therapy has
B never been used to treat ACH.
NB-UVB phototherapy is an effective treatment for psori-
Figure 1. The patient on presentation; pustular and erythema- asis [13] and has been reported to be more effective than
tosquamous psoriasiform lesions, skin atrophy, onychoptosis
BB-UVB [4] and as effective as PUVA therapy in psoria-
and nail dystrophy of toes.
sis [5]. Because of the histopathological similarity of
ACH with psoriasis, among various treatment alternatives
mined on gluteal areas of the patient was 300 mJ/cm2. we decided to give topical 8-MOP/NB-UVB phototherapy
The initial dose was 70% of the MED. If erythema did in reference to the previous report on its successful use in
not occur, a dose increment of 40% was applied. If there psoriasis [7]. In this report, topical 8-MOP/NB-UVB pho-
was erythema, a 20% dose increment was administered. totherapy was applied once a week and fluencies of UVB
When more prominent erythema occurred, the dose was delivered to each spot were four multiples of MED and
not increased, instead it was reduced by a ratio of 1:2. remained constant throughout the study [7]. We applied
After reaching 2,000 mJ/cm2 the dose remained constant. topical 8-MOP/NB-UVB phototherapy two times a
Already after the fifth session, the lesions responded to week, because the initial dose started was 70% of the
the treatment (figure 2). Ten weeks later, when the patient MED and our patient could attend the irradiation program
had received 20 sessions, almost all lesions had cleared two times a week.
(figure 2) and the cumulative dose reached was It has been shown that UVB primarily affects the T cells
in lesional skin of psoriasis [14]. However, UVB therapy
has also been shown to suppress lymphocyte production
by decreasing the production of proinflammatory cyto-
kines and by inducing the production of anti-
inflammatory cytokines within the lymph nodes [13].
UVB therapy has been proposed to have immunosuppres-
sive effects in psoriasis through induction of apoptosis in
T cells, and NB-UVB has been reported to be directly
cytotoxic to T cells in vivo, resulting in a higher depletion
of dermal and epidermal T cells in psoriatic skin [15]. It
has been shown that patients treated with NB-UVB
secreted interleukin-10 (IL-10), an anti-inflammatory
cytokine, and showed a markedly decreased production of
A B IL-1β, IL-2, IL-5 and IL-6 [13]. Treatment with recombi-
nant IL-10 has been reported to be effective in psoriasis
Figure 2. Lesions after the 20th session. and UVB irradiation was shown to induce the production