ARTICLE
Auteur(s) : Vincenzo DE
Francesco1, Eva Quinkenstein1, Laura
Mariuzzi2, Alfonsina Frattasio1, Barbara
Pillon1, Pasquale Patrone1
1Institute of Dermatology, Department of Clinical and
Experimental Pathology and Medicine, Gemona Hospital, Piazza
Rodolone 1, 33123 Gemona del Friuli (Udine), Italy
2Institute of Pathology, University of Udine, Italy
accepté le 1 Juillet 2005
Prurigo pigmentosa is a rare inflammatory skin disease of unknown
etiology, described first by Nagashima in 1971, characterized by
recurrent, symmetrical, pruritic, erythematous papules resulting in
gross reticular hyperpigmentation [1, 2]. The rash occurs mainly on
the back, the chest and the nape of the neck [3, 4]. It is observed
rather frequently in Japan, where more than 200 patients have been
reported, but only a few cases have described in non-Japanese cases
[5-9]. Vesicular or bullous forms have been rarely reported, and
only in Japanese subjects [3, 5, 10]. The differential diagnosis
includes lichen pigmentosus, pigmented contact dermatitis,
confluent and reticulated papillomatosis of Gougerot and Carteaud,
dermatitis herpetiformis and bullous lichen ruber planus [3,
6].This case report concerns a young Caucasian patient with prurigo
pigmentosa, in whom predominantly vesicular, but also bullous
manifestations appeared on an existing maculopapular eruption on
the trunk.
Case report
The patient, a 17 year-old student, came to our attention in August
2003 due to an erythematous vesiculopapular eruption on the trunk.
The patient reported the initial appearance of itchy maculopapular
lesions covering an area of about 10 × 10 cm in the left
lumbar region, in October 2002. The individual lesions were
separated by apparently normal skin. Treatment with topical
steroids and antihistamines per os had not been effective; in fact,
after a modest regression of the eruption, the maculopapulae had
spread over the trunk, with the onset of clear vesicular lesions in
a matter of weeks. The patient reported a relapsing chronic
clinical course during which the lesions assumed a prevalently
brown macular pigmentation. In June the patient underwent a skin
biopsy abroad, which resulted in “Dermatopathia Pigmentosa
Reticularis (DPR)”. Physical examination showed an erythematous
maculo-vesiculopapular eruption on the lower half of the dorsal
region, the right flank, the right inguinal area, the right
pectoral region and right armpit (( figure 1 )A). The
maculopapulae were of a few mm in size, irregular with clear
margins, and bright red in colour. Most of the lesions were
overlaid by vesicles containing clear liquid; the lesions had a
tendency to become confluent, assuming a reticular aspect (( figure 1 )B).
Serohematic crusts covering the maculopapulae were present on the
right pectoral region. The patient reported to be in good health,
was not taking any drugs and had never suffered from any
dermatological disease; moreover, the patient was active and
practised sports. The family history showed that an aunt died of
reported systemic lupus erythematosus and two cousins were celiac
sufferers, one of whom had relapsing cutaneous eruptions.
Hematochemical tests were within normal values except for
monocytes 16.2% (1.01 thousand/mm3) and potassium 5.3
mEq/l; reactive protein C, antistreptolysin O titre, C3, C4,
anti-transglutaminase antibodies were normal. Glucose tolerance
test was negative. Anti-herpes virus 1 and 2, anti-echovirus,
anti-Epstein-Barr virus, anti-Coxsackie virus, anti-Cytomegalovirus
antibodies were negative. Tests for IgG anti-skin antibodies by
means of indirect immunofluorescence proved to be negative. The
rheumatoid factor, HBV and HCV antibodies, anti-nucleus antibodies,
anti-DNA, anti-ENA and anti-ANCA antibodies were all absent; TPHA
was also negative. Urine tests were within normal values. Chest
X-ray and the ultrasonography of the abdomen were normal.
A skin biopsy specimen was taken from the right flank. The
histological examination showed subepidermal vesicles containing
rare erythrocytes and lymphocytes; the epidermis revealed
hyperkeratosis and necrotic keratinocytes. Near the vesicles, the
epidermis showed hypergranulosis, spongiosis and some dyskeratotic
cells. The papillary dermis hosted lymphohistiocytic infiltrates
that locally became dense, obscuring the dermo-epidermal junction
(( figure 3 ),
left inset). The vascular plexus was dilated and there was
erythrocyte extravasation (( figure 3 ), right
inset)?
Direct immunofluorescence for IgG as well as IgA, C3 and C4 was
negative.
During hospitalisation only topical treatment with
fluorine-containing steroids was used; after about three weeks
there was an improvement in the clinical picture and the vesicles
and evident brown maculopapulae disappeared. As the patient lived
abroad for his studies, we were unable to follow him over time.
Nevertheless, three months later he informed us by phone that there
had been a remission of the disease after a cycle of narrow-band
ultraviolet B phototherapy (311 nm). When the treatment was
interrupted, the lesions reappeared. The patient underwent several
cycles of UVB phototherapy during 18 months with partial and
temporary clinical improvement. We phoned the patient abroad and,
considered the stationary clinical condition, suggested starting a
therapy with minocycline 100 mg/day orally for 6 weeks.
Four weeks later the patient reported the almost complete
resolution of the eruption and pruritus.
Discussion
Prurigo pigmentosa (PP) is a rare inflammatory dermatosis. The
etiology and pathogenesis of PP have yet to be determined. Some
authors suggested that various contact allergens may be pathogenic
or triggering factors, but all attempts to identify an allergen
have been unsuccessful [6, 11]. Nagashima hypothesized that some
unknown environmental contaminant specific to Japan might be
involved in the pathogenesis [3]. Ketosis was considered as an
important etiological factor, it is the common denominator in some
conditions associated with PP: fasting, dieting and
insulin-dependent diabetes mellitus [4, 6, 10, 12-14]. However, the
relationship between the ketosis and the pathogenesis of the PP is
still uncertain [4]. None of these factors were present in our
patient.
PP is characterized by a rash that consists of recurrent,
symmetrical, intensely pruritic, erythematous papules. They resolve
leaving a peculiar reticular hyperpigmentation. The rash occurs
mainly on the back, the chest and the nape of the neck [3, 4].
Recurrent attacks are very common for several months [15, 16].
Vesicular or bullous forms of PP are very rare, only 12 cases have
been described between 1971 and 1999, most of them in Japanese
reports [3, 5, 10, 17].
The characteristic feature in the present case is the formation
of numerous vesicles and bullae at the beginning and throughout the
clinical course of the disease. Vesicles have been reported during
the clinical course of PP sometimes, but in literature we found a
severe formation of bullae or vesicles only in two Japanese men [3,
10].
PP is observed rather frequently among young adult Japanese
females, but only few cases have been reported in non-Japanese
patients [1, 5, 6, 18, 19]. PP is not a very widely known entity
outside Japan, so the number of new cases in Europe may be
underestimated [7, 15].
The differential diagnosis includes bullous lichen ruber planus,
pigmented contact dermatitis, confluent and reticulated
papillomatosis of Gougerot and Carteaud and dermatitis
herpetiformis [3, 6]. Direct immunofluorescence, required for
differential diagnosis, is constantly negative in PP. Clinical
features which include the seasonal predilection in spring or
summer, the distribution of lesions, coexistence of erythematous
papules and reticulate pigmentation, good response to dapsone
therapy distinguish PP from other dermatoses [11]. It is important
to know this relatively new entity in western countries for a
correct diagnosis. As regards treatment, our patient had
experienced an improvement with the application of a topical
fluorine-containing steroid. Usually topical corticosteroids and
systemic antihistamines have only a modest effect; systemic
corticosteroids and dapsone (diaminophenylsulphone), a drug that is
not available in Italy, produce improvement but cannot prevent
recurrences. Systemic minocycline has been reported to be effective
in the treatment of PP [5, 13, 20]. Both dapsone and minocycline
have the capacity to inhibit the release of proinflammatory and
chemotactic factors [5, 15, 17, 21]. Also macrolide antibiotics can
be considered as an alternative treatment for PP [22].
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