|Year : 2019 | Volume
| Issue : 1 | Page : 60-63
Zosteriform idiopathic atrophoderma of Pasini and Pierini
Ellahi Medicare Clinic, Srinagar, Jammu and Kashmir, India
|Date of Web Publication||14-Dec-2018|
Dr. Tasleem Arif
New Colony Soura, Near Water Supply Control Room, Srinagar - 190 011, Jammu and Kashmir
Source of Support: None, Conflict of Interest: None
Idiopathic atrophoderma of Pasini and Pierini (IAPP) is a rare disease of unknown etiology characterized by well-defined atrophic plaques with a “cliff-drop” border that show no signs of inflammation, sclerosis, and induration. The trunk is most commonly affected site. It usually affects the body in a bilaterally symmetrical distribution, although asymmetric involvement has also been reported. Very few cases occurring in a zosteriform distribution have been reported. In this article, the author reports a rare case of IAPP in an 18-year-old male where the lesions are distributed in a zosteriform distribution on the trunk.
Keywords: Atrophoderma, cliff-drop border, idiopathic atrophoderma of Pasini and Pierini, zosteriform
|How to cite this article:|
Arif T. Zosteriform idiopathic atrophoderma of Pasini and Pierini. Indian J Paediatr Dermatol 2019;20:60-3
| Introduction|| |
Idiopathic atrophoderma of Pasini and Pierini (IAPP) is a rare disease of unknown etiology characterized clinically by the appearance of well-defined atrophic plaques with a “cliff-drop” border. These plaques in contrast to morphea do not show signs of inflammation, sclerosis, and induration. The most commonly affected site is the trunk especially the back and abdomen. Proximal extremities can also get affected. Most commonly, the plaques are present in a bilaterally symmetrical distribution, although lesions involving the body in an asymmetrical distribution have also been reported. The plaques are generally round or ovoid in shape with their long axis parallel to the lines of cleavage. Unilateral involvement has been described less commonly. Very few cases where the lesions are present in a zosteriform distribution have been reported. Herein, the author reports a rare case of zosteriform IAPP in an 18-year-old male involving trunk.
| Case Report|| |
An 18-year-old male presented with a 1½-year history of gradually progressive skin lesions involving the left side of the chest and abdomen. There was no history of any previous skin lesions at that particular site. The patient did not report any preceding tick bite, redness, induration, or a history of trauma. There was no itching or pain associated with these skin lesions. The patient reported that initially there were multiple lesions which in due course of time coalesced with one another to form a single larger skin lesion.
Clinical examination revealed a large, 20 cm × 6 cm, linear, light brownish colored, atrophic plaque distributed in a zosteriform pattern, extending from the left anterior chest below the nipple to the midline in the epigastric region, and not crossing the midline [Figure 1]. The affected skin of the plaque was depressed compared to the surrounding normal skin, giving the appearance of an “inverted plateau.” The plaque was depressed and had sharply defined, abrupt borders [black arrows in [Figure 1] giving a typical “cliff-drop” and “footprint in snow” appearance. The depression at the borders ranged from 2 to 4 mm. At some places, the plaque had arcuate borders [red arrows in [Figure 1] as if smaller plaques have coalesced with one another to form a single large plaque, confirming the patient's history. The surface of the plaque was smooth without epidermal atrophy, hyperkeratosis, telangiectasia, or signs of inflammation. There was no erythematous or lilac ring seen around the plaque. The follicular orifices along with hair shafts emanating from them were intact. Palpation revealed no edema, thickening, induration, or sclerosis. No “hernia” phenomenon was appreciated on palpation ruling out the macular atrophies (anetodermas). The rest of the systemic examination was unremarkable.
|Figure 1: There is a linear, light-brownish colored, atrophic plaque distributed in a zosteriform pattern. The plaque is depressed with sharply defined, abrupt borders (black arrows) giving a typical “cliff-drop” appearance. At some places, the plaque has wavy arcuate borders (red arrows)|
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Routine laboratory tests were within normal limits. Antinuclear antibody testing was negative. Enzyme-linked immunosorbent assay for Borrelia burgdorferi was negative. Histopathology revealed normal thickness of the epidermis, increased pigmentation of the basal layer, and dermis filled with homogenized collagen bundles. Sweat glands and pilosebaceous units were intact [Figure 2]. There was scanty inflammation. Based on the history, a suggestive clinical examination and further supported by histopathological findings, he was diagnosed as a case of zosteriform IAPP.
|Figure 2: The epidermal thickness is normal with increased pigmentation of the basal layer and dermis filled with homogenized collagen bundles. Sweat gland and pilosebaceous units are intact. There is scanty mononuclear infiltrate in the upper dermis (H and E, ×10)|
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| Discussion|| |
IAPP has been reported as early as 1902–1904, when Brocq et al. termed it “atrophic scleroderma d'emblee” and Thibierge described a “dyschromic and atrophic variation of scleroderma.”, In 1923, Pasini described a case report of a 21-year-old female with a 4-year history of brownish gray, “cyanotic” lesions on the trunk that was published in an Italian dermatology journal. These lesions were round or oval and depressed below the surrounding tissue. There was no inflammation or sclerosis. These patches were smooth, flat, and depressed 1–2 mm below the surrounding skin. He termed this entity as “atrofodermia idiopatica progressiva” designating it as a separate entity from that of morphea or scleroderma. It has been translated in English as “progressive idiopathic atrophoderma.” In 1936, in Argentina, Pierini and Vivoli described similar lesions in a 19-year-old female. She had bluish, nonindurated patches of 3 years' duration that were present on the right side of the chest and back in a somewhat zosteriform distribution. These skin lesions remained completely unchanged for 10 years. Subsequently, Pierini observed over 50 such cases. His cases and several reports by other researchers can be seen in the Argentine literature. Pierni classified this disorder as a primary idiopathic form of atrophy and an atrophoscleroderma, which was secondary to localized scleroderma. Since then, it has been described in the literature by various names such as “atrophoscleroderma superficialis circumscripta,” “morphea with an unusual degree of atrophy,” “morphea plana atrophica,” and “atypical lilac-colored and nonindurated scleroderma.” It was Canizares et al., in 1958, who first introduced this entity into the American dermatologic literature. He reviewed the findings made by Pierini and proposed the term “IAPP.” Canizares et al. believed that the disease was unique and differed sufficiently from morphea to classify it as a distinct entity, and that the two authors deserve the credit for making the greatest contribution to the knowledge of this entity. Yokoyama et al., in 2000, showed that the skin glycosaminoglycans extracted from the lesions of patients with IAPP were different from those seen in typical lesions of morphea thus supporting the view that IAPP is different from morphea.
IAPP is a distinctive form of dermal atrophy which is characterized by well defined, slightly depressed blue-gray-brown plaques arising within the areas of normal-looking skin. The lesions lack inflammation. They are asymptomatic, nonindurated, and lack the characteristic “lilac ring” border of morphea. Their spread is very slow spanning over to many years without showing a tendency to undergo a spontaneous resolution. It most commonly affects young women. Lesions predominantly involve the trunk especially the upper back and lumbosacral region. In most of the cases, lesions show a bilateral symmetric distribution; however, unilateral cases have also been reported. Rarely, zosteriform distribution of the lesions has been described thus prompting me to present this case.,,
IAPP usually affects the individuals during the second or third decades of life. However, it can occur in infancy and old age. It predominantly affects females with female-to-male ratio of 2:1. I could search four cases of congenital IAPP.
The etiology of IAPP remains unknown. Some authors have proposed that genetic factors may be involved in its pathogenesis. This is supported by a hypothesis where the disease occurred in two brothers. It has also been supported by a case where IAPP and discoid lupus erythematosus were seen in a patient with hereditary C2 complement deficiency which has a known association with a particular human leukocyte antigen haplotype. A neurogenic cause has been suggested by some authors in view of the apparent zosteriform distribution of lesions in few cases., Cells with aberrant T-cell phenotype have been incriminated as a cause of collagen fibre destruction. Few cases of IAPP along the blaschko lines including the one reported by Arif et al. raise the possibility of a developmental abnormality such as somatic mosaicism in such cases. Some authors have suggested the role of spirochete B. burgdorferi in the pathogenesis of some cases of IAPP. Buechner and Rufi investigated sera of 26 patients with typical lesions of IAPP. About 53% of patients had immunoglobulin G anti–B burgdorferi antibodies while only 14% of the control participants demonstrated these antibodies. However, no immunoglobulin M antibodies were found. Immunological factors and abnormal metabolism of dermatan sulfate are the other factors that have been implicated by some authors in its pathogenesis.,
Histopathological changes in IAPP are not diagnostic and may be mild and can be easily missed unless an elliptical biopsy involving both the affected and normal skin is taken perpendicular to the edge of the lesion and deep enough to include the subcutaneous tissue. On H and E stain, IAPP exhibits mostly a normal epidermis with increased pigmentation in the basal layer. Dermis shows a mild perivascular mononuclear cell infiltrate with melanophages in the upper dermis. There is edema, homogenization, and clumping of collagen in the deeper dermis. The appendageal structures and subcutaneous tissue are usually intact. The elastic tissue stain of lesional skin is essentially normal., However, Saleh et al., in their study, involving 17 skin biopsies from typical IAPP lesions showed that the elastic fiber changes on Verhoeff-van Gieson and/or orcein-Giemsa stains were present in the upper one-third of the dermis. There was a reduction with the fragmentation of elastic fibers in the dermis, and it was graded as moderate to severe in 35.5% of their specimens. The sweat glands and the pilosebaceous units are not affected, unlike morphea. The direct immunofluorescence may be negative or nonspecific. Franck et al. using magnetic resonance imaging demonstrated that the skin depression in IAPP lesions is solely because of dermal atrophy. On the contrary, Abe et al. using B-mode ultrasound in one patient showed that this clinical depression was because of atrophy involving both dermis as well as subcutaneous layers.
IAPP needs to be differentiated from other related disorders such as morphea, linear atrophoderma of Moulin (LAM), and anetoderma. The absence of signs of inflammation, sclerosis, induration, and “lilac ring” border in the lesions of IAPP differentiates it from morphea. The sclerosis which is prominent in morphea is absent or minimal in IAPP. Elastic tissue changes which have been described in IAPP are not seen in morphea. IAPP can be distinguished from LAM by the latter's earlier onset, nonprogressive course, and distribution of lesions along Blaschko lines, as well as absence of dermal changes. IAPP is considered mostly a dermal atrophy, while as in LAM, the depression in the lesions is because of atrophy in subcutaneous fat., Cases of IAPP with significant elastic fiber reduction need to be differentiated from anetodermas. The lesions of anetodermas are characterized by wrinkled macules or patches that protrude and herniate into the skin upon palpation (sac-like protrusions).
Currently, there is no effective treatment available for IAPP. However, some treatments have been tried which include psoralen plus ultraviolet A, potassium benzoic acid, and oral antibiotics in some patients where B. burgdorferi antibodies were elevated. A report of a 35-year-old female with elevated B. burgdorferi antibody (IgM) titer and IAPP showed clinical improvement with doxycycline (200 mg/day) for 6 weeks. A retrospective study involving 25 patients who were treated with either oral penicillin (2 million IU/day) or oral tetracycline (500 mg three times/day) for 2–3 weeks showed clinical improvement in 20 patients with no new active lesions. Oral hydroxychloroquine has been suggested by Carter et al. as a therapeutic option for chronic refractory IAPP. Q-switched alexandrite laser was effective in treating hyperpigmentation associated with IAPP; however, it did not help with atrophy.
IAPP tends to be progressive, with new lesions developing and enlarging variably over 10–20 years. Ultimately, a stage is reached at which no further progression occurs, even though the existing lesions do not involute which differentiates it from morphea. No definite progression to systemic sclerosis has been observed. However, Bisaccia et al., in 1982, reported a case of a 42-year-old female with typical lesions of IAPP on her back who developed systemic sclerosis with sclerodactyly, Raynaud's phenomenon, and lung fibrosis.
Declaration of patient consent
The author certifies that he has obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]