Correspondence 1391
Discussion
Figure 1 (a) Lamellar horizontal splitting of the right distal
thumbnail plates, and (b) desquamation and scaling of the tip of
the right thumbnail
CORRESPONDENCE
Unilateral idiopathic atrophoderma of Pasini and Pierini
Case report
A 17-year-old girl presented with a 5-year history of asymp-
tomatic unilateral eruption which started on the thigh but
quickly spread to the abdomen, buttock, and back on the left
side only. Neither earlier inflammation nor any preceding
eruption was observed. The patient’s general health was
excellent. The patient’s had no history of any infections
associated with these lesions or any cutaneous or systemic
diseases.
© 2006 The International Society of Dermatology
Lamellar onychoschizia is one of two types of nail brittleness
characterized by lamellar exfoliation in the distal portion of
the nail plate. Triangular pieces may easily be detached from
the free margin.1 Nail flexibility is mainly determined by the
water content in the nail plate, which is normally 18%. When
the water content is reduced to < 16%, the nails tend to be
brittle.2 Occupational contact with solvents and solutions can
lead to progressive dehydration of the nail plate and plays an
important role in the development of nail brittleness in
shoemakers and carpenters.3
China, as an original tea producing country, harvests the
largest amount of tea for the world tea market. The process of
picking tea is still repetitive manual work. The authors’ report
thumbnail fragility in a tea-picker who pinched the tea leaves
off with the right index fingernails every year from March–
September. Possibly, lamellar dystrophy is a result of frequent
wetting and drying of the right dominant thumbnails during
picking the leaves. Moreover, it is speculated that catechine,
a biological acid in the tea, could modify the keratin content
of the nail plate or break the amino acid chains, causing brit-
tleness of the thumbnail. Causes related to other skin disease,
systemic disease or drugs were easily excluded from this case.
Cheng Tan, MD
Department of Dermatology, First Affiliated Hospital of
Nanjing University of TCM, Nanjing, China
Wen-Yuan Zhu, MD
Department of Dermatology, First Affiliated Hospital of
Nanjing Medical University, Nanjing, China
References
1 Wallis MS, Bowen WR, Guin JD. Pathogenesis of
onychoschizia (lamellar dystrophy). J Am Acad Dermatol
1991; 24: 44–48.
2 Iorizzo M, Pazzaglia M, Piraccini BM, et al. Brittle nails.
J Cosmetic Dermatol 2004; 3: 138–144.
3 Lubach D, Cohrs W, Wurzinger R. Incidence of brittle nails.
Dermatologica 1986; 172: 144–147.
Physical examination revealed numerous skin lesions com-
posed of 20–50-mm diameter violaceous patches on the left
thigh, left buttock, left side of the back, and left abdominal
area. The lesions were depressed below the level of the sur-
rounding skin and coalesced to form large pigmented areas
(Fig. 1). Their distinct margin and 1–4-mm depression gave
them a cliff-drop appearance. Most of the lesions on the back
showed a band-like pattern (Fig. 2). There was no inflamma-
tion, induration, nor sclerosis.
Laboratory studies including a complete blood count, liver
function test, urinalysis, and electrolytes were within normal
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1392 Correspondence

Figure 1 Unilateral idiopathic atrophoderma of Pasini & Pierini Figure 2 Depressed violaceous macules in linear distribution on
(IAPP) involving the trunk – posterior aspect the left side of the body – lateral aspect

limits. Serum antibodies for Borrelia burgdorferi were nega-

tive. Complete ophthalmologic, odontologic, and radiologic
surveys revealed no abnormalities.
A skin biopsy taken from the atrophic pigmented lesion on
the back showed an indurated dermis with hyalinized and
swollen collagen bundles (Fig. 3a). In some areas the collagen
bundles were tightly packed (Fig. 3b). The elastic fibers
mostly in the upper dermis were fragmentized and the changes
were better observed by orcein staining.

Discussion
Idiopathic atrophoderma of Pasini & Pierini is an uncommon
dermatosis of unknown etiology. In 1923, Pasini1 presented a
case of pigmentary atrophoderma both clinically and histo-
logically different from localized scleroderma, or any other
known atrophy, which the author called progressive idio- Figure 3 (a) Changes in deeper layers of the dermis show
pathic atrophoderma. In 1936, Pierini & Vivoli2 described a hyalinized and swollen collagen bundles (H&E, ×40) and
similar entity occurring in a 19-year-old woman. Pierini sub- (b) tightly packed collagen bundles in deeper layers of the
sequently observed more than 50 cases. Pierini’s reports and dermis (H&E, ×400)

International Journal of Dermatology 2006, 45, 1391–1393

1369 –1370 © 2006 The International Society of Dermatology

numerous reports by other authors are found in the Argentinian
literature.3 Idiopathic atrophoderma of Pasini & Pierini has
been reported under a variety of names, including atrophic
scleroderma d’emble, dyschromic and atrophic variant of
sclerodrema, morphea plana atrophica, and atypical lilac-
colored and nonindurated scleroderma.4–6 The disorder was
first introduced into the American dermatologic literature in
1958 by Canizares et al.,3 who proposed the term idiopathic
atrophoderma of Pasini & Pierini (IAPP).
This IAPP usually affects people in the second and third
decades of life, but can occur in infancy and old age,3,5 and the
female to male ratio is 2 : 1.7 It is characterized by single or
multiple pigmented, depressed plaques. The affected areas are
atrophic, often exhibiting a cliff-drop border of 2–8 mm
forming an inverted plateau. The lesions are usually round
or ovoid, varying in size from a few millimeters to several
centimeters, and often orientated along the skin cleavage lines
and possibly coalescing to form larger, irregular-shaped plaques
with convex borders.3 The lesions are usually asymptomatic
and do not show inflammation and sclerosis.7 First they are
localized and then they slowly spread to other areas over a
period of months or years before stabilizing. The course of the
disorder is benign. The histologic changes in early lesions are
slight and nonspecific, consisting of mild homogenization of
the collagen bundles and a scattered chronic inflammatory
infiltrate. Older lesions may show in the deeper layers of the
dermis collagen bundles that are not only thickened but also
appear tightly packed.8
The back, especially the lumbo-sacral region, is most com-
monly involved,5,7 and other sites frequently include the chest,
abdomen, flanks, thighs, and upper extremities. No occur-
rences have been recorded on the face, and the hands and feet
are also rarely affected.3 The distribution of the lesions is
often bilateral and symmetrical although unilateral involve-
ment has been reported.2,3,7,9,10 Several bands of typical
depressed areas confined to the left side of the body and
extending from the axilla to the foot have been described in a
French patient.9 Ironado et al.10 reported a patient with IAPP
involving only the right side of the body with multiple
ungrouped depressed areas extending from the right axilla to
the right ankle; the patient’s case differed in that the lesions
were ungrouped instead of in bands. Of particular interest
was the segmental zosteriform distribution in some of the
patients with IAPP.2,3
However, in this reported case, all the lesions occurred on the
left side of the body where the thigh, buttock, and abdominal
area manifested the classic presentation of IAPP. Most of the
lesions on the back showed a band-like configuration; how-
ever, they did not follow the pattern of Blaschko’s lines. Because
linear lesions are often a feature of localized scleroderma,
the latter can be regarded as an important differential diagnosis
of IAPP. Linear scleroderma showed a similar configuration,
atrophy, and hyperpigmentation. The absence of inflamma-
Correspondence
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© 2006 The International Society of Dermatology
Correspondence 1393
tion, sclerosis, or induration leads to a diagnosis IAPP. The
skin lesions of linear atrophoderma of Moulin resemble those
of IAPP; however, they do follow Blaschko’s lines. Some pig-
mented unilateral lesions of IAPP may clinically resemble
hypermelanosis of Ito, pigmented post-herpes zoster scars,
especially on the back, and Goltz syndrome.
Conclusion
Unilateral and widespread involvement, as well as linear dis-
tribution of the lesions of IAPP, has rarely been reported.
It is controversial as to whether IAPP represents a variant
of scleroderma or is a separate entity.3,5 Our patient had no
signs of scleroderma preceding the onset of the skin lesions.
Considering the onset, course, and the clinical and histological
findings in this case, we support the concept that IAPP is a
rare form of dermal atrophy and may represent a separate
entity of unclear cause.
Ljubka Miteva, MD, PhD
Medical University, Sofia, Bulgaria
Miroslava Kadurina, MD, PhD
Military Medical Academy, Sofia, Bulgaria
References
1 Pasini A. Atrophoderma idiopathica progressiva. G Ital
Dermatol 1923; 58: 785– 809.
2 Pierini LE, Vivoli D. Atrophodrmia idiopathica
progressiva. (Pasini). G Ital Dermatol 1936; 77: 403–
409.
3 Canizares O, Sachs PM, Jaimovich L, et al. Idiopathic
atrophoderma of Pasini and Pierini. Arch Dermatol 1958;
74: 42– 60.
4 Jablonska S, Szczepanski A. Atrophoderma Pasini-Pierini.
In: Scleroderma and Pseudoscleroderma. Warsaw: Polish
Medical Pubishers, 1975: 521–536.
5 Pullara TJ, Lober CW, Fenske NA. Idiopathic
atrophoderma of Pasini and Pierini. Int J Dermatol 1984;
23: 643– 645.
6 Gougerot H. Sclerodermies atypiques: la forme lilacee non
induree en plaques ou un bandes. Bull Soc Fr Dermatol
Syphiligr 1932; 39: 1667–1669.
7 Buechner SA, Rufli T. Atrophoderma of Pasini and Pierini.
Clinical and histopathological findings and antibodies to
Borrelia burgdorferi in thirty-four patients. J Am Acad
Dermatol 1994; 30: 441– 446.
8 Lever WF, Schaumburg-Lever G. Atrophoderma of Pasini
and Pierini. In: Lever WF, Schaumburg-Lever G.
Histopathology of the Skin, 5th edn, Philadelphia: JP
Lippincott, 1975: 444– 445.
9 Bourgeois-Spinasse J, Grupper H. Atrophodermie de
Pasini-Pierini en bands unilaterale. Bull Soc Fr Dermatol
Syphiligr 1969; 76: 494.
10 Iriondo M, Bloom RF, Neldner KH. Unilateral
atrophoderma of Pasini and Pierini. Cutis 1987; 39:
69 –70.
International
International Journal
Journal of
of Dermatology
Dermatology 2006,
2006, 45,
45, 1369
1391–1393
–1370