ARTICLE
Deeply depressed lesions suggest lipoatrophic or lipodystrophic disease.
Recently, we had an adult female patient with deeply depressed lesions
on the abdominal wall that were preceded by slight pain and erythema.
There was a marked decrease of the subcutaneous fatty tissue with minimal
inflammatory cell infiltration. We found three similar cases in the literature
[1-3]. We herein report and discuss our case along with these other cases.
Case report
A 46-year-old female came to us with a well-defined, deeply-depressed
palm-sized lesion on the right lateral to lower abdominal wall. Slightly
brownish pigmentation was noted on the surface of the lesion (Fig.
1). Regional lymph node swelling was not noted. She told us that
the depressed lesion had developed after a slightly painful sensation
of a few days duration on the right side of the navel at the age of 43.
There was no history of trauma or injection in this area.
A year later, she again noticed slight pain and erythema on the right
lateral abdomen, without any general symptoms.The erythematous area had
gradually become depressed, leaving slight brownish pigmentation, and
expanded over a few months. A biopsy was performed from the depressed
area, where virtually no subcutaneous fatty tissue was noted. Microscopical
examination revealed a marked decrease of subcutaneous fatty tissue, and
normal collagen bundles with minimal inflammatory cell infiltration. Elastica
staining did not show any abnormalities in the dermis. The results of
laboratory examinations including complete blood cell counts, biochemical
analysis of serum, and the levels of amylase in serum and urine, C-reactive
protein, and antistreptolysin O titer were all normal or within normal
limits. The levels of serum complement components C3 and C4 were 67.9
mg/dl (80-200 mg/dl) and 9.7 mg/dl (15-60 mg/dl) respectively. During
a follow-up period of three years, the depressed lesion gradually became
indistinct, but a new depressed lesion developed on the lower abdominal
wall one week after the onset of pain.
Discussion
This case is characterized by well-defined, deeply depressed lesions
on the abdominal walls of an adult female, which were preceded by slight
pain and erythema of a few days duration, without any history of trauma
or injection. The depressed lesion showed a marked decrease of the subcutaneous
fatty tissue with minimal cell infiltration. The most important differential
diagnosis may be lipodystrophia centrifugalis abdominalis infantilis (LCAI)
[4], which is characterized by depressed lesions in the abdominal wall
of infants or children.
Regarding 86 cases of LCAI that we collected, the depressed lesions
developed by the age of 8 years in all cases, mostly in the groin or axillary
area. Although the depressed areas were occasionally surrounded by slight
erythema, the patients never complained of pain or other subjective symptoms
in these areas [5]. In a search of the literature, we found three similar
cases, which had been reported under the name of lipodystrophia centrifugalis
(abdominalis infantilis) [1-3]. All of those patients had pain or paresthesia
with erythema before the development of the depression on the trunk, and
these patients were all adults (Table
1). Our patient and these three patients are distinct from those
with LCAI in respect of (1) the presence of preceding pain or paresthesia
and (2) the development of the lesions on the trunk of adults. Other differential
diagnoses include atrophoderma of Pasini-Pierini [6] and several localized
lipoatrophic or lipodystrophic diseases such as atrophic connective tissue
disease panniculitis [7], lipoatrophia annularis [8], lipoatrophia semicircularis
[9], and panatrophy of Gowers [10]. Atrophoderma of Pasini-Pierini may
reveal erythematous changes of the skin, but histological examination
shows the homogenization of collagen bundles, suggesting a variant of
morphea. Atrophic connective tissue disease panniculitis develops in patients
with connective tissue diseases. Although our patient showed slight decreases
of C3 and C4 levels in serum, no other symptoms or laboratory findings
suggestive of connective tissue diseases were noted. Lipoatrophia annularis
and lipoatrophia semicircularis show circular atrophy exclusively on the
extremities or ankles. Pantrophy of Gowers shows sharply defined atrophy
of the skin, subcutaneous fat and also, in rare cases, of the muscle and
bone. However, pain does not precede any of these disorders.
CONCLUSION
In conclusion, our case and three similar cases in the literature do
not fit any of the so far described lipoatrophic diseases and might be
a new clinical entity.
Accepted on 14/6/00
REFERENCES
1.Yoshizumi Y, Sakakibara S. So-called lipodystrophia centrifugalis
abdominalis infantilis. Jpn J Dermatol 1984; 94: 623 (in Japanese).
2. Rowland Payne CME, Harper JI, Branfoot AC, et al. Lipodystrophia
centrifugalis. Br J Dermatol 1985; 113 (suppl 29): 100-1.
3. Endo N, Akasaka T, Mori Y, et al. Lipodystrophia centrifugalis
abdominalis-Adult case. Jpn J Clin Dermatol 1991; 45: 27-30 (in
Japanese).
4. Imamura S, Yamada M, Ikeda T. Lipodystrophia centrifugalis
abdominalis infantilis. Arch Dermatol 1971; 104: 291-8.
5. Imamura S, Yamda M, Yamamoto K. Lipodystrophia centrifugalis
abdominalis infantilis. A follow-up study. J Am Acad Dermatol 1984;
11: 203-9
6. Buechner SA, Rufli TR. Atrophoderma of Pasini and Pierini.
Clinical and histopathologic findings and antibodies to borrelia burgdorferi
in thirty-four patients. J Am Acad Dermatol 1994; 30: 441-6.
7. Peters MS, Winkelmann RK. Localized lipoatrophy (atrophic
connective tissue disease panniculitis). Arch Dermatol 1980; 116:
1363-8.
8. Ferreira-Marques J. Lipoatrophia annularis. Arch Dermatol
Syphilis 1953; 195: 479-91.
9. Geschwandtner WR, Munzberger H. Lipoatrophia semicircularis.
Hautarzt 1974; 25: 222-7.
10. Magee KR, Critchley M. Gower's local panatrophy. Neurology
1957; 7: 307-12.
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