Translate this page into:
Dermoscopy of perforating periumbilical pseudoxanthoma elasticum
Corresponding author: Dr. Kewal Krishan, Department of Dermatology, Venereology & Leprosy, Government Medical College, Srinagar, Jammu & Kashmir, India. kewalkrishan33@gmail.com
-
Received: ,
Accepted: ,
How to cite this article: Bashir Y, Rather SP, Bhat YJ, Krishan K, Binti Ismail I, Sultan Y. Dermoscopy of perforating periumbilical pseudoxanthoma elasticum. Indian J Dermatol Venereol Leprol. doi: 10.25259/IJDVL_1259_2025
Dear Editor,
Perforating periumbilical pseudoxanthoma elasticum (PPPXE) is a rare disorder. It represents a localised variant of pseudoxanthoma elasticum (PXE). It usually affects obese multiparous women and typically presents as a yellowish abdominal plaque involving the periumbilical area with areas of atrophy and keratotic plugs. Histopathology demonstrates calcified elastic fibres, amorphous calcium deposits, epidermal perforation, and inflammatory infiltrate. Dermoscopy has been rarely described in this disorder. It shows yellow structureless areas, curved/semilunar brown lines, and keratotic plugs.1
We report a series of three cases of PPXE with dermoscopic and histopathological features.
Our first patient was a 56-year-old normotensive, nondiabetic, and hypothyroid woman. She presented with an asymptomatic, well-defined yellowish plaque with atrophic scarring and multiple erythematous keratotic papules with brownish black central crusts for 1 year [Figure 1a]. There was no history of intermittent claudication, chest pain, or significant gastrointestinal symptoms. Baseline investigations were unremarkable. Lipid profile revealed hypertriglyceridaemia and hypercholesterolaemia. Echocardiography and electrocardiography were normal. Ophthalmology consultation showed a normal fundoscopic examination. Dermoscopy showed the presence of keratotic plugs, telangiectasias, brown arcuate lines, and white structureless areas [Figures 1b and c].
Our 2nd patient was a 48-year-old woman, hypertensive, nondiabetic, and euthyroid. She presented with a yellowish plaque below the umbilicus for 2 years. Multiple hyperpigmented crusted papules involved the central part of the plaque with areas of atrophy and scarring peripherally [Figure 2a]. Baseline investigations, echocardiography, electrocardiography, and ophthalmological examination were normal. Dermoscopic features noted were diffuse erythema, plugging, and pigmentation in a nonspecific pattern [Figures 2b and c].
The 3rd case was a hypertensive, diabetic, and euthyroid 50-year-old woman who presented with yellowish plaque with areas of atrophic scarring and overlying brownish crusted papules above the umbilicus for 1.5 years [Figure 3a]. Ophthalmological and other routine investigations were normal. Dermoscopy showed the presence of keratotic plugs, scarring, and pigmentation in a linear and arcuate pattern [Figures 3b and c].
Histopathological examination of the skin lesion showed calcification of elastic fibres in the dermis, amorphous calcium deposits, inflammatory infiltrate, and perforation of epidermis by elastic fibres [Figures 4a and b].
PXE is associated with flexural lesions and retinal involvement, or localised involvement of skin without systemic involvement.2-5 The classification is still controversial, as the term perforating calcific elastosis (PCE) was coined to emphasise the lack of systemic manifestations.6 Some studies consider PPPXE to be an acquired entity due to cutaneous trauma secondary to obesity, multiparity, abdominal surgeries, and ascites. Others suggest PPPXE to be a limited cutaneous expression of hereditary PXE because of concomitant cardiovascular and ophthalmological complications. However, literature suggests PPPXE to be a “nosocomial bridge” between hereditary and acquired types of PXE.7
Our patients did not show any flexural lesions or systemic involvement. Two of our patients had hypertension, however, angioid streaks were not found in any of them. There should be a strong suspicion of an underlying systemic disorder if PPPXE occurs in the absence of contributory risk factors. The risk factors include cutaneous trauma to the abdominal wall secondary to multiparity and obesity.
Dermoscopy may become an essential diagnostic tool for PPPXE. In our cases, we found the presence of keratotic plugs, arcuate and curvilinear hyperpigmented lines, white areas, and orange yellow pigmentation [Table 1]. Keratotic plugs seen on dermoscopy correspond to extrusion of elastotic material, whereas yellowish pigmentation represents degeneration of elastic fibres. Dermoscopic features with histopathological correlation is reported in Table 1. Dermoscopic features of other perforating disorders are provided in Table 2.
| Dermoscopic feature | Histopathological correlation |
|---|---|
| Arcuate hyperpigmented lines | Altered elastic fibres or calcific deposits |
| Keratotic plug | Trans-epidermal elimination of elastic fibers, perforation of epidermis |
| Yellowish hue | Degeneration of elastic fibres |
| Erythema | Inflammatory infiltrate |
| Disorder | Dermoscopic features |
|---|---|
| Kyrle’s disease | Central keratotic plug surrounded by a whitish rim and a peripheral erythematous halo |
| Perforating folliculitis | Three concentric zones- keratotic plug, surrounding white collar (white collar sign), and surrounding pink area with vessels. |
| Elastosis perforans serpiginosa | Milky red background, comedo- like structures, grey structureless areas, white collarette. |
| Reactive perforating collagenosis | Three zone pattern- central yellow brown structureless area (crusted lesion), white rim of keratinous debris (epidermal invagination), outer dotted or radial vessels (dermal inflammation) |
| Acquired perforating dermatosis (APD) | Central crateriform keratotic plug, white rim/collarette, surrounding erythema, vascular structures may be seen |
Since none of our patients had flexural lesions or involvement of retinal epithelium, it favours PPPXE as a distinct entity. However, two of our three patients were hypertensive, which may be an indicator of an underlying systemic disorder.
Ethical approval
Institutional Review Board approval is not required.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
Use of artificial intelligence (AI)-assisted technology for manuscript preparation
The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.
References
- Periumbilical perforating pseudoxanthoma elasticum: A rare case report. Dermatol Pract Concept. 2018;8:75-7.
- [CrossRef] [PubMed] [Google Scholar]
- Perforating pseudoxanthoma elasticum Its distinction from elastosis perforans serpiginosa. Arch Pathol Lab Med. 1976;100:544-6.
- [PubMed] [Google Scholar]
- Periumbilical hyperpigmented plaque periumbilical perforating pseudoxanthoma elasticum (PPPXE) Arch Dermatol. 1990;126:1639-42.
- [CrossRef] [PubMed] [Google Scholar]
- Perforating pseudoxanthoma elasticum associated with chronic renal failure and hemodialysis. Arch Dermatol. 1985;121:1321-2.
- [CrossRef] [PubMed] [Google Scholar]
- Periumbilical pseudoxanthoma elasticum with systemic manifestations. South Med J. 1991;84:788-9.
- [CrossRef] [PubMed] [Google Scholar]
- Periumbilical perforating pseudoxanthoma elasticum. J Am Acad Dermatol. 1988;19:384-8.
- [CrossRef] [PubMed] [Google Scholar]
- Localized acquired cutaneous pseudoxanthoma elasticum. J Am Acad Dermatol. 1979;1:523-30.
- [CrossRef] [PubMed] [Google Scholar]