Fibroelastolytic papulosis: histopathologic confirmation of disease spectrum variants in a single case

Fibroelastolytic papulosis is a rare, acquired fibroelastolytic disorder that presents clinically as white‐to‐yellow papules and plaques most commonly occurring on the neck of elderly patients. The term fibroelastolytic papulosis encompasses two closely related conditions previously described as pseudoxanthoma elasticum‐like papillary dermal elastolysis (PDE) and white fibrous papulosis of the neck (WFPN). Here we present a case of a 78‐year‐old white female with a several‐year history of numerous, asymptomatic 2–3 mm yellowish, non‐follicular papules distributed symmetrically over the posterior neck, axillae, arm and antecubital fossae. Histopathologic examination revealed thickened and clumped elastotic fibers admixed with thick, sclerotic appearing collagen bundles in the mid and deep reticular dermis. Rare melanophages, loss of vertically oriented elastic fibers and scattered elastotic globes were noted in the papillary dermis. Based on the shared clinicopathologic features showed in this case, strong consideration should be made for the additional inclusion of papillary dermal elastosis as existing along the disease continuum of fibroelastolytic papulosis. This occurrence of fibroelastolytic papulosis shows unique histopathologic findings of pseudoxanthoma elasticum‐like PDE, papillary dermal elastosis and WFPN, further supporting the theory that these entities exist as variants along the fibroelastolytic papulosis spectrum.     

Papillary dermal elastosis

There are numerous acquired disorders of elastic tissue that are distinguished by a combination of clinical appearance, location, gender, age of onset, and characteristic histopathologic findings. We present a case of a 36-year-old man with multiple confluent, hypopigmented papules that coalesced into plaques with prominent follicular ostia over the dorsal aspects of the forearms, shoulders, upper chest, and upper back. Histologically there was selective loss of papillary dermal elastic fibers. The clinical and histopathologic findings in this case are consistent with an acquired disorder of elastic tissue which we believe represents the second reported case of papillary dermal elastosis.     

"Lumpy-Bumpy" Elastic Fibers in the Skin and Lungs of a Patient with a Penicillamine-Induced Elastosis Perforans Serpiginosa

Penicillamine-induced cutaneous elastosis perforans serpiginosa associated with a large air-cyst in the right lung is described in a 29-year-old female patient with Wilson disease. Identical light and electron-microscopic changes were present in both dermal and pulmonary elastic tissue, suggesting a disseminated drug-induced cutaneo-visceral elastosis. Lung cysts have not been previously reported in association with long term penicillamine treatment. The electron-microscopic morphology of the elastic fibers was found to be "specific" enough to allow separation of penicillamine-induced elastosis perforans serpiginosa from other forms of this disease.     

Pseudoxanthoma elasticum-like papillary dermal elastolysis in non-exposed skin

Pseudoxanthoma elasticum-like papillary dermal elastolysis is an acquired elastic tissue disorder clinically similar to pseudoxanthoma elasticum in the absence of systemic involvement. Histopathologically, special staining of elastic fibers demonstrates a total or partial band-like loss of elastic fibers in the papillary dermis. Although ultraviolet radiation seems to be one of the main etiological factors in this entity, we report a case of pseudoxanthoma elasticum-like papillary dermal elastolysis on the neck of a woman who wore hijab.     

Morphological study of the relationship between solar elastosis and the development of wrinkles on the forehead and lateral canthus

OBJECTIVE To identify whether there is a relationship between solar elastosis and the development of wrinkles in human skin. DESIGN Wrinkle depth was measured on the forehead and lateral canthus of human cadavers using image analysis. The thickness of the dermis was measured in skin sections obtained around wrinkles and stained with Elastica-van Gieson. SETTING Gross Anatomy Section, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan. SUBJECTS Fifty-eight male and female cadavers (age range at death, 29-93 years). MAIN OUTCOME MEASURES The ratio of solar elastosis dermal thickness to full dermal thickness (elastosis ratio) was calculated and compared between the deepest point of a wrinkle (wrinkle point) and a point within 1 mm where no wrinkle existed (nonwrinkle point). The relationship between elastosis ratios and wrinkle depths was investigated. RESULTS Advanced solar elastosis was present at nonwrinkle points but was present a little bit at wrinkle points. On the forehead, a positive correlation between elastosis ratios and wrinkle depths was observed at nonwrinkle points but not at wrinkle points. On the lateral canthus, a positive correlation between elastosis ratios and wrinkle depths was observed at nonwrinkle points, as well as at wrinkle points until the wrinkle became deeper than one-half of the original dermal thickness (0.6 mm). Solar elastosis on the lateral canthus ceased developing at this point, but the wrinkle developed further. CONCLUSIONS Solar elastosis tends to commence with the development of a wrinkle until the wrinkle becomes deeper than 0.6 mm. This tendency is less evident at wrinkle points than at nonwrinkle points.     

Elastosis perforans serpiginosa with widespread arterial lesions: a case report.

A case is presented of elastosis perforans serpiginosa (EPS) with unilateral dermal lesions, widespread arterial lesions with aortic rupture, and elastosis of the endocardium and bronchiolar walls. Other chronic skin disorders with lesions resembling EPS are discussed; and the arterial lesions compared with some arterial diseases. The findings support a concept of the disease as a focal affection of elastic tissue, not only in the skin, but also in arteries and other organs.     

Actinic elastosis in black skin

Summary In 9 otherwise dermatologically normal South African Black volunteers (1–72 years old), sunlight-exposed and non-exposed skin has been examined by light- and electronmicroscopy with special references to age-dependent and actinic alteration of dermal structures. Two 72 year old Blacks exhibited typical dermal elastosis: in 1 case to a marked extent and already detectable by lightmicroscopy, in the other case only to a mild degree. In contrast, only age-related elastic fibers were revealed in the lower dermis of sunlight-exposed skin and in all dermal layers of unexposed skin. These observations are contradictory to the general view that actinic elastosis does not occur in Blacks. The elastotic material is obviously a de-novo synthesized and secreted pathological product of chronically UV-altered fibroblasts.Reported in part at the XV. International Congress of Dermatology, Mexico City, October 1977, by M. Dogliotti, W. Ch. Marsch, E. Schober and F. Nürnberger     

Linear focal elastosis (elastotic striae)

Three patients are described who had a distinctive clinical and histologic presentation of dermal elastosis. Sunlight apparently played little role in the development of this elastotic change. To the best of our knowledge, this is the first report of this form of elastosis.     

Penicillamine-induced elastosis perforans serpiginosa with abnormal "lumpy-bumpy" elastic fibers in lesional and non-lesional skin

Four types of elastosis perforans serpiginosa (EPS) have been described in literature: 1) idiopathic EPS, 2) reactive perforating elastosis associated with connective tissue disorders, 3) in some instances of pseudoxanthoma elasticum (PXE), disease-specific calcified elastic tissue is extruded, producing a clinical picture indistinguishable from other types, may also be seen in patients undergoing hemodialysis and 4) EPS induced by long-term treatment with D-penicillamine is observed in patients suffering from Wilson's disease. Long term D-penicillamine therapy causes an alteration in the dermal elastic tissue. D-penicillamine induced EPS has a distinctive histopathologic feature - serrated appearance of elastic fibers due to perpendicular budding from their surface giving a "lumpy-bumpy" look. D-penicillamine induced elastic fiber alteration may not always manifest clinically as EPS. We report a case of D-penicillamine induced widespread alteration in skin elastic tissue with distinct histopathologic features.     

Acquired disorders of elastic tissue: part I. Increased elastic tissue and solar elastotic syndromes

Elastic fibers in the extracellular matrix are an integral component of dermal connective tissue. The resilience and elasticity required for normal structure and function of the skin may be attributed to the network of elastic tissue. Advances in our understanding of elastic tissue physiology provide a foundation for studying the pathogenesis of elastic tissue disorders. Many acquired disorders are nevertheless poorly understood due to the paucity of reported cases. Several acquired disorders in which accumulation or elastotic degeneration of dermal elastic fibers produces prominent clinical and histopathologic features have recently been described. They include elastoderma, linear focal elastosis, and late-onset focal dermal elastosis and must be differentiated from better-known disorders, among them acquired pseudoxanthoma elasticum, elastosis perforans serpiginosa, and Favré-Racouchot syndrome. Learning objective At the conclusion of this learning activity, participants should understand the similarities and differences between acquired disorders of elastic tissue that are characterized by an increase in elastic tissue, as well as the spectrum of solar elastotic dermatoses.     

Pseudoxanthoma-like late-onset focal dermal elastosis

A 73-year-old woman presented with a 10-year history of asymptomatic coalescent flat pale yellow lesions forming a cobblestone pattern that resembled pseudoxanthoma elasticum over her neck, upper trunk and axillae. Skin biopsies from both the neck and axilla showed focal upper dermal elastosis with increase in structurally normal elastic fibres. There was no evidence of either pseudoxanthoma elasticum or solar elastosis in either biopsy. This under-recognized presentation has previously been identified as a distinct clinicopathological entity and termed late-onset focal dermal elastosis.     

Late-onset focal dermal elastosis: an uncommon mimicker of pseudoxanthoma elasticum

Late-onset focal dermal elastosis is a rare disorder that presents clinically with the development of small white-to-yellow papules simulating pseudoxanthoma elasticum (PXE) in otherwise healthy adults in the seventh through ninth decades. It is characterized histopathologically by foci of increased normal-appearing elastic tissue in the reticular dermis. The disorder lacks any of the systemic complications of PXE and clinically resembles several other elastic tissue disorders that mimic PXE. We report two cases of late-onset focal dermal elastosis. The first is of a 75-year-old female who presented with symmetrically distributed, 2-5 mm white-to-yellow, discrete and coalescing, non-follicular papules on the posterolateral neck, anterior chest and axillae. The second case involves a 39-year-old female who presented with asymptomatic flesh-colored lesions on the posterior neck, back, antecubital and popliteal fossae, thighs, forearms and wrists. Skin biopsies in each case revealed aggregates of elastic fibers in the reticular dermis without calcification. The differential diagnosis of clinical and histopathologic imitators of PXE is discussed.     

Linear focal elastosis

A case of linear focal elastosis is reported. A 19-year old male presented with asymptomatic, palpable yellow lines over back along with striae distensae over axilla. Light microscopic examination demonstrated dermal thickening but no change in the epidermis. The elastic tissue stain revealed fragmented elastic fibres throughout the dermis.     

Non-invasive evaluation of dermal elastosis by in vivo multiphoton tomography with autofluorescence lifetime measurements

The non-invasive differentiation of dermal elastic fibres from solar elastosis in vivo is of great interest in dermatologic research, especially for efficacy testing of anti-ageing products. To date, no studies on multiphoton excited fluorescence lifetime characteristics of human elastic fibres and solar elastosis are reported. The goal of the present work was the identification of differential criteria for elastic fibres and solar elastosis by the analysis of fluorescence decay curves acquired by time-correlated single photon counting in vivo multiphoton tomography. For this purpose, fluorescence lifetime measurements (FLIM) were performed with 47 volunteers of different age groups at sun-protected and sun-exposed localizations. Bi-exponential curve fitting was applied to the FLIM data, and characteristic differences between age groups and localizations were found in both relevant fit parameters describing the decay slope. The FLIM analyses have shown that dermal autofluorescence has different lifetimes depending on age and in part on localization.     

Papular elastosis

I report two typical cases of a cutaneous disorder caused by sunlight, which is common in Phoenix, Arizona, "The Valley of the Sun." It currently masquerades under the misnomer, adult colloid milium. It generally presents clinically as asymptomatic, shiny, smooth, firm papules, 1 to 10 mm in diameter, involving chronically sun-damaged skin of adults. The papules are generally multiple, but may be solitary, with various colorations. Microscopically, the papules all show severe elastosis involving most of the papillary cutis. It is most important to biopsy these lesions because clinically they may mimic a variety of skin lesions, some of which would require further investigations and therapy. This is a distinct clinical form of severe solar elastosis, which I propose to term papular elastosis.     

Relationship between food intake and cutaneous solar elastosis adjacent to basal cell carcinoma

Background/Objective Studies suggest that diet may influence in skin ageing and skin appearance. However, the effect of diet in the elastotic changes of dermis, which is the main histological sign of ageing, has not been studied previously. The objective was to investigate if the dietary habits influence the dermal elastosis observed in BCCs. Materials and methods The 136 patients with facial BCCs, who underwent surgery, were interviewed to assess the consumption of fruit, vegetables, fat, red meat, coffee and tea. We reviewed 136 specimens of BCC to identify the presence of solar elastosis. We also analysed clinical variables such as gender, age, phototype and smoking. Results Severe solar elastosis was found in 22 patients (16%), middle reticular dermis in 37 (27 %) and 77 patients (57%) had abscence or light elastosis. Fat consumption was reported by most of participants from our sample, while fruit and tea consumption was less common. Intakes of fat, vegetables and coffee were not associated with the grade of elastosis whereas Vitamin E and C‐rich fruits and tea were correlated with less risk of elastosis. Smokers showed higher grades of elastosis than non‐smokers. Conclusion Our study provides evidence that the presence of dermal elastosis and cutaneous ageing may be influenced by the type of food intake: Vitamin E and C‐rich fruit and tea are positively associated with less elastosis.     

HISTOLOGICAL EVALUATION OF THE EFFECT OF 0.05% TRETINOIN IN THE TREATMENT OF PHOTO DAMAGED SKIN

In a randomised double-blind vehicle controlled trial of 0.05% tretinoin cream in the treatment of photodamaged skin, the histological results of paired biopsies from 28 individuals who applied tretinoin for 26 weeks are compared with 28 paired biopsies from a control group applying vehicle alone. There was a significant increase in epidermal thickness in the tretinoin-treated group (P.001). Epidermal atrophy was reversed in ten patients applying tretinoin cream. Baseline biopsies obtained from participants in Melbourne, Victoria (38° latitude) showed significantly less elastosis than those in Sydney (34° latitude) and Newcastle in N.S.W., although the two groups did not show significant differences in age or sex, and the differences could not be correlated with skin type. Tretinoin cream had no effect on the degree of solar elastosis after 26 weeks application. Tretinoin cream appears effective in reversing epidermal atrophy and clinically diminishes fine wrinkling, mottled hyperpigmentation and skin roughness. Tretinoin cream may not offer a solution to the gross solar damage seen in the Australian population who have marked solar elastosis as a principal, clinical and histologie finding. However it is possible that dermal repair and reversal of solar elastosis may require topical application of tretinoin cream for a longer period than the six months used in this trial.     

In vitro reconstitution of skin: fibroblasts facilitate keratinocyte growth and differentiation on acellular reticular dermis.

Extensive full-thickness burns require replacement of both epidermis and dermis. We have described a method in which allogeneic dermis from engrafted cryopreserved cadaver skin was combined with cultured autologous keratinocytes. In the present study we combined human keratinocytes and fibroblasts, and acellular human dermis in vitro and transplanted this "reconstituted skin" into athymic mice. Both human papillary dermis in which the basement membrane zone has been retained and human reticular dermis that has been repopulated with human dermal fibroblasts are good substrates for keratinocyte attachment, stratification, growth, and differentiation. Both of these dermal preparations can be lyophilized and stored at room temperature without losing their ability to support keratinocyte growth. In contrast, human papillary dermis that has been treated with trypsin lacks laminin and collagen type IV in the BMZ and supports keratinocyte attachment and differentiation less well.     

Generalized elastosis perforans serpiginosa in Down''s syndrome

Elastosis perforans serpiginosa is a rare disorder of epidermal perforation characterized by the extrusion of dermal elastic tissue through the epidermis. Its aetiology is unknown, but there is histological and biochemical evidence of an abnormality of elastic tissue. Three forms of elastosis perforans serpiginosa exist. It may be either idiopathic, iatrogenic, or, in approximately one quarter of cases, associated with certain genetically determined disorders of connective tissue. Cutaneous lesions appear between the ages of 6 and 20 years, and persist for 6 months to 5 years. They may be confined to one anatomic area, or less frequently are disseminated. We review the case of a 28-year-old woman with recent onset of unusually extensive elastosis perforans serpiginosa with co-existing Down's syndrome and (secondary) sclerosing cholangitis.     

Erythema ab igne with features resembling keratosis lichenoides chronica

Erythema ab igne (EAI) is an asymptomatic dermatosis that develops in response to chronic exposure to low‐grade heat. Characteristic findings on histopathology include epidermal atrophy, dermal elastosis, atypical histiocytes, and melanin and hemosiderin deposition. Reactive endothelial changes and prominent vascular proliferation are variable. Keratosis lichenoides chronica (KLC) is a rare lichenoid hyperkeratotic dermatosis. Acanthosis with parakeratosis and a lichenoid interface dermatitis with lymphocytes, histiocytes, and plasma cells are characteristic findings of KLC. Although its etiology remains unclear, KLC has been reported to occur in response to heat. Herein, we report a case of EAI with features resembling KLC.