Congenital idiopathic atrophoderma of Pasini and Pierini
- Author(s): Handler, Marc Zachary;
- Alshaiji, Jasem M;
- Shiman, Michael I;
- Elgart, George W;
- Schachner, Lawrence A
- et al.
Published Web Locationhttps://doi.org/10.5070/D38pj8x3bz
Congenital idiopathic atrophoderma of Pasini and PieriniUniversity of Miami, Miami, Florida
Marc Zachary Handler MD, Jasem M Alshaiji MD, Michael I Shiman MD, George W Elgart MD, Lawrence A Schachner MD
Dermatology Online Journal 18 (4): 4
Idiopathic atrophoderma of Pasini and Pierini is a disorder of dermal atrophy. There is a female predominance and almost never does the condition present at birth. Histopathological examination reveals attenuated dermis. We report a case of a healthy male born with a plaque of idiopathic atrophoderma of Pasini and Pierini.
Idiopathic Atrophoderma of Pasini and Pierini (IAPP) is a rare disorder of dermal atrophy. Virtually all cases present after birth and have a strong female predominance. This case is unique in that it describes a male with an atrophic, ovoid plaque present at birth.
We report a case of a Caucasian male who was born without complication to a healthy mother. At the time of birth, a large atrophic plaque was noted on his lower back. Growth continued appropriately and developmental milestones were met. The patient presented to dermatology clinic at the age of 5 months with a well-defined 9.0 cm x 2.5 cm gray, atrophic plaque with prominent vasculature on his lower right back. A biopsy was performed at the peripheral margin and allowed for comparison of normal and affected tissue. The tissue demonstrated a thin dermis with normal appearing sub cutis which was relatively high-up, adjacent to the epidermis, suggestive of dermal atrophy. There was a normal amount and distribution of elastic tissue fibers and PAS did not reveal an infectious organism (Figures 1 and 2). The patient underwent an MRI of the lumbar region which did not reveal involvement of deeper structures. Throughout his follow up visits with dermatology the lesion has grown to 9.0 cm x 3.0 cm (Figure 3) and the child has remained in good health.
|Figure 2||Figure 3|
Idiopathic atrophoderma of Pasini and Pierini is a rare, disorder of dermal atrophy described by Pasini in 1923 and Pierini & Vivoli in 1936 [1, 2]. The typical presentation is an ovoid, mildly depressed, hyperpigmented lesion of the trunk [3, 4, 5]. There exists a female to male predominance between 2 and 6 fold [3, 5, 6, 7]. Onset is common between 20 and 40 years of age [3, 5]. The lesions are often bilateral and color may range from blue to grey to brown. Review of the literature reveals a single congenital case in a Korean female . Our case is the first time congenital IAPP has been reported in a male.
Etiology of IAPP is unknown. There is an association between lesion development and a positive ANA . An association between exposure to Borrelia burgdorferi has been identified in a small number of patients as well [5, 7].
Debate exists on whether IAPP is distinct from morphea. Some authors have suggested that IAPP is a partially developed lesion of morphea [5, 7]. Idiopathic atrophoderma of Pasini and Pierini does have a similar appearance to morphea with atrophy of the dermis and both have the ability to persist though life and be progressive in diameter. Histology demonstrates similarities between regressing morphea plaques, abortive morphea plaques and IAPP .
Despite these similarities there are distinct differences. Idiopathic atrophoderma of Pasini and Pierini does not demonstrate the erythematous, lilac-ring border of morphea . It is not uncommon for patients with IAPP to develop morphea at separate sites . Histology for IAPP is non-specific and unlike morphea does not demonstrate sclerosis at presentation [4, 9]. Histological examination of the elastic tissue is often normal and thinning of the dermis in conjunction with the clinical picture is used for diagnosis of IAPP [4, 7].
We believe IAPP to be a distinct entity that may rarely present congenitally in males and females. Although a benign condition, clinical improvement has been seen in treatment with hydroxychloroquine and the Q-switched alexandrite laser [6, 10].
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