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Background
This dermal mucinosis presents as flesh-colored papules on the back of the hands and occasionally forearms. The disorder occurs predominately in women and is progressive. Some investigators believe it is a localized variant of papular mucinosis or scleromyxedema.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION INCIDENCE Rare
DISEASE ASSOCIATIONS CHARACTERIZATION IgA MONOCLONAL GAMMOPATHY Acral persistent papular mucinosis and IgA monoclonal gammopathy: report of a case.
Borradori L, Aractingi S, Blanc F, Verola O, Dubertret L. Clinique des Maladies Cutanees, CHU Saint-Louis, Paris, France.
Dermatology 1992;185(2):134-6 Abstract quote
The case of a 60-year-old man with acral persistent papular mucinosis (APPM), thought to represent a new distinctive form of dermal mucinosis not associated with systemic diseases, is reported.
The patient had a 4-year history of multiple small papular lesions on the distal forearms, wrists and back of the hands. Histologically, mucin deposits in the upper and mid dermis sparing a superficial subepidermal grenz zone were observed. In contrast to previously described cases, a monoclonal IgA of kappa light chain isotype was detected.
Our findings challenge the view that absence of paraproteinemia is a peculiar characteristic of APPM and raise once more the question of its relationship to the discrete papular form of lichen myxedematosus.
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION
- Acral persistent papular mucinosis.
Harris JE, Purcell SM, Griffin TD.
Lehigh Valley Hospital Dermatology Residency Program, Allentown, Pennsylvania, USA.
J Am Acad Dermatol. 2004 Dec;51(6):982-8. Abstract quote
Acral persistent papular mucinosis is a rare subtype of localized lichen myxedematosus. For half a century, this disease has endured a controversial and constantly evolving classification.
We describe a patient who presented with discrete, flesh-colored papules on the hands, wrists, and forearms in a distribution consistent with acral persistent papular mucinosis. Histology was also constant with this disease, showing a well-circumscribed deposition of mucin in the upper and mid dermis that spared a small grenz zone.
The changing nomenclature and diagnostic requirements of acral persistent papular mucinosis that have allowed it to remain a topic of debate are examined through a comprehensive review of the literature. All reported cases are reviewed.VARIANTS FAMILIAL Acral persistent papular mucinosis in two sisters.
Menni S, Cavicchini S, Brezzi A, Gianotti R, Caputo R.
Institute of Dermatological Science, University of Milan, IRCCS Ospedale Maggiore, Italy.
Clin Exp Dermatol 1995 Sep;20(5):431-3 Abstract quote
We describe two young sisters with an asymptomatic papular eruption on the forearms, the clinical, histopathological and ultrastructural features of which were consistent with acral persistent papular mucinosis.
Familial occurrence of this uncommon disease is exceptional.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL A skin biopsy shows dermal mucinosis similar to papular mucinosis but there is less mucin and fibroblasts.
SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHERCHARACTERIZATION SPECIAL STAINS IMMUNOPEROXIDASE ELECTRON MICROSCOPY Ultrastructural signs of altered intracellular metabolism in acral persistent papular mucinosis.
Aho HJ, Forsten Y, Hopsu-Havu VK.
Department of Pathology, University of Turku, Finland.
J Cutan Pathol 1991 Oct;18(5):347-52 Abstract quote
An asymptomatic 24-year-old woman presented with multiple discrete papules on the extensor surfaces of the hands and wrists.
Light microscopy revealed focal increase in the amount of dermal fibroblasts as well as deposition of hyaluronidase-labile mucoid substance. The collagen and elastin were decreased. The changes were consistent with acral persistent papular mucinosis (APPM). In electron microscopy, the intercellular glycosaminoglycans showed small ruthenium red-positive granules and thin filaments indicating normal morphology. The fibroblastic cells, however, were conspicuously altered. Endoplasmic reticulum was dilated, cytoplasm contained large amounts of osmiophilic, concentric lysosomal structures, and there was distinct fibrous lamina in the nuclear membrane.
It was concluded that the primary event in APPM probably affects the intracellular metabolism of the dermal fibroblast. The accumulation of lysosomal structures may be a distinct feature of APPM differentiating it from the other reactive cutaneous mucinoses, or it may only reflect nonspecific degeneration in a long-standing lesion.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES Dermal mucinosis Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.
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Last Updated June 9, 2005
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