Acral Persistent Papular Mucinosis: A New Entity

Rongioletti, Franco; Rebora, Alfredo

doi:10.1001/archderm.1986.01660230027002

Cited by 54 publications

(16 citation statements)

References 13 publications

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“…Among nonhistiocytic disorders, acral persistent papular mucinosis differs from HPMH in that it is not hereditary, children are not affected, and the lesions show mucinous degeneration with a scant histiocytic infiltrate. 54 Multiple dermatofibromas are larger clinically, and multiple lesions are uncommon over the upper limbs or hands, but inheritance and mucin production in HPMH are essential diagnostic features.…”

Section: Differential Diagnosismentioning

confidence: 99%

Unusual variants of non-Langerhans cell histiocytoses

Caputo¹,

Marzano²,

Passoni³

et al. 2007

Journal of the American Academy of Dermatology

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Histiocytic syndromes represent a large, heterogeneous group of diseases resulting from proliferation of histiocytes. In addition to the classic variants, the subset of non-Langerhans cell histiocytoses comprises rare entities that have more recently been described. These last include both forms that affect only the skin or the skin and mucous membranes, and usually show a benign clinical behavior, and forms involving also internal organs, which may follow an aggressive course. The goal of this review is to outline the clinical, histologic, and ultrastructural features and the course, prognosis, and management of these unusual histiocytic syndromes.

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Section: Differential Diagnosismentioning

confidence: 99%

Unusual variants of non-Langerhans cell histiocytoses

Caputo¹,

Marzano²,

Passoni³

et al. 2007

Journal of the American Academy of Dermatology

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“…In 1986, Rongioletti and Rebora 18 described a new form of cutaneous mucinosis: acral persistent papular mucinosis. Since then, several cases of this entity have been reported.…”

Section: Discussionmentioning

confidence: 99%

Atypical acral persistent papular mucinosis

Abalde¹,

Ginarte²,

Fernández‐Redondo³

et al. 1999

Int J Dermatology

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A 70‐year‐old woman presented with a 10‐year history of cutaneous lesions on her calves that worsened with solar exposure. Her past medical history was irrelevant. Physical examination revealed 10–15 erythematous, firm nodules with a diameter of 2–5 mm, which were slightly painful on palpation. The lesions were located on both calves, although some elements extended to the posterior side of the thighs ( Fig. 1). General physical examination did not show any abnormalities. A biopsy specimen from one of the elements showed a normal epidermis and, in the upper dermis, a slight inflammatory infiltrate of mononuclear cells, with the presence of splits between collagen fibers ( Fig. 2). Alcian blue stain at pH 2.5 revealed the existence of mucinous material in these spaces ( Fig. 3). An electron microscopic study did not show any abnormalities. 1 Clinical appearance of the lesions 2 Histopathologic picture (hematoxylin and eosin, ×40) 3 Alcian blue stain showing the presence of mucin in the dermis (Alcian blue, ×100) The patient was diagnosed as having papular mucinosis. The following studies were normal or negative: complete cell blood count, serum biochemistry, serum protein electrophoresis, serum immunoelectrophoresis, serologic test for human immunodeficiency virus (HIV), hepatitis B and C viruses, and syphilis, antinuclear antibodies, and thyroid hormone levels. No treatment was carried out.

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“…In der Alcianblaufärbung waren in diesen Bereichen deutliche Die akrale persistierende papulöse Muzinose (APPM) wurde als eigenständiges Krankheitsbild innerhalb der dermalen Muzinosen 1986 von Rongioletti et al definiert [19]. In der Alcianblaufärbung waren in diesen Bereichen deutliche Die akrale persistierende papulöse Muzinose (APPM) wurde als eigenständiges Krankheitsbild innerhalb der dermalen Muzinosen 1986 von Rongioletti et al definiert [19].…”

Section: Histologie (Abb 3)unclassified

“…Ob die APPM ein eigenes Krankheitsbild darstellt, wie Rongioletti meinte [17][18][19][20], oder eine klinische Variante der diskreten papulösen Form des Lichen myxoedematosus (DPLM) [3,15], wurde bisher noch nicht endgültig geklärt.…”

Section: Histologie (Abb 3)unclassified

“…Autoren [7,12,17,[19][20] definierten Kriterien des Krankheitsbildes weitgehend auf. Auffällig sind die Ausbreitung der Effloreszenzen über die gesamte Unterarmlänge bis hin zu den Ellenbogen und eine leichte Tendenz zur Aggregation.Demgegenüber ist der Lichen myxoedematosus (LM) durch asymptomatische und meist hautfarbene, jedoch typischerweise zur Aggregation neigende Papeln oder Plaques vor allem an Armen, Rumpf und Oberschenkeln charakterisiert.…”

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Akraler papulöser Lichen myxoedematosus

Gartner¹,

Schoppelrey²,

Agathos³

et al. 1998

Der Hautarzt

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In 1986 Rongioletti introduced the term acral persistent papular mucinosis for persistent papules on the dorsum of the hands and distal forearms. Skin specimens reveal mucin deposits which resemble those in lichen myxedematosus (LM). Laboratory results are usually unremarkable. Our 54-year-old female patient showed a 2 1/2 year history of skin lesions in the typical acral distribution pattern. This entity appears to be an acral papular subtype of LM. In the classification of LM, acral papular lichen myxedematosus should replace the discrete papular form. This latter form has been well known for many years, but is not sufficiently definable and is of secondary clinical importance.

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Acral Persistent Papular Mucinosis: A New Entity

Cited by 54 publications

References 13 publications

Unusual variants of non-Langerhans cell histiocytoses

Unusual variants of non-Langerhans cell histiocytoses

Atypical acral persistent papular mucinosis

Akraler papulöser Lichen myxoedematosus

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