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Background
This dermal mucinosis is a variant of a connective tissue nevus characterized by small papules that are grouped or linear on the extremities or trunk. They occur at birth or early adolescence. Under the microscope, there is thickened papillary dermis secondary to abundant mucin. There may be an increase in fibroblasts and the epidermis may show mild hyperplasia.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS INCIDENCE/
PREVALENCEAGE SEX GEOGRAPHY EPIDEMIOLOGIC ASSOCIATIONS
DISEASE ASSOCIATIONS CHARACTERIZATION
PATHOGENESIS CHARACTERIZATION
LABORATORY/
RADIOLOGIC/
OTHER TESTSCHARACTERIZATION RADIOLOGIC LABORATORY MARKERS
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION GENERAL
- Mucinous nevus.
Yokogawa M, Kamakura T, Ishiguro H, Ikeda M, Kodama H.
Department of Dermatology, Kochi Medical School, Nankoku, Kochi, Japan.
J Dermatol. 2005 Jan;32(1):30-3. Abstract quote
A 15-year-old boy first noticed multiple firm papules on his right upper chest two years before presenting to our clinic. These papules were densely distributed and showed epidermal nevus-like linear arrangement at some sites. The number, size, and distribution of these papules remained unchanged for one year of our observation. The papules flattened leaving scars and did not recur within a year after one shaving abrasion using a scalpel.
Histopathologically, epidermis of the papule displayed acanthosis with elongated rete ridges. Accumulation of mucin was apparent in the papillary and subpapillary dermis. In mucinous nevus, the origin of cells with nevoid proliferation is obscure. In contrast with common collagenous connective tissue nevus, it is hard to define the localized persistent mucin accumulation as a nevoid manifestation.
The present case of mucinous nevus might be caused by significantly stimulated glycosaminoglycan synthesis in a kind of epidermal nevus without extreme hyperkeratosis.
- A case of congenital mucinous nevus: a connective tissue nevus of the proteoglycan type.
Chang SE, Kang SK, Kim ES, Lee MW, Choi JH, Sung KJ, Moon KC, Koh JK.
Department of Dermatology, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea.
Pediatr Dermatol. 2003 May-Jun;20(3):229-31. Abstract quote
Mucinous nevus is a very rare entity and can be classified as both a cutaneous mucinosis (CM) and a connective tissue nevus (CTN).
We describe the clinicopathologic features of an unusual case of mucinous nevus in a 14-year-old Korean boy who presented with zosteriform plaques of congenital onset.
- Mucinous nevus: report of two cases and review of the literature.
Suhr KB, Ro YW, Kim KH, Lee JH, Song KY, Park JK.
Department of Dermatology, Chungnam National University School of Medicine, Taejon, Korea.
J Am Acad Dermatol. 1997 Aug;37(2 Pt 2):312-3. Abstract quote
We report two cases of nervus mucinosis with papulonodules distributed dermatomally on the right T12 and L1 areas.
Case 1 is a 61-year-old man who exhibited nodular lesions on the right abdomen in a zosteriform arrangement. Alcian blue staining showed the presence of mucin in the papillary dermis. Case 2 is a 17-year-old man who had a 10-year history of match-head to bean-sized skin lesions, multiple papules, and nodules distributed dermatomally on the right lower back.
- Mucinous eccrine nevus.
Romer JC, Taira JW.
Department of Dermatology, University of Oklahoma Health Sciences Center, Oklahoma City 73104.
Cutis. 1994 May;53(5):259-61 Abstract quote.
We report a solitary lesion with histologic characteristics of an organoid nevus occurring on the lower extremity of a forty-seven-year-old woman.
This lesion is thought to represent a variant of eccrine nevus composed of an intradermal proliferation of hyperplastic eccrine sweat glands with associated mucinous change.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL
- Mucinous eccrine nevus.
Park HS, Lee UH, Choi JC, Chun DK.
Department of Dermatology, Sanggye Paik Hospital, Inje University College of Medicine, Seoul, Korea.
J Dermatol. 2004 Aug;31(8):687-9. Abstract quote
A 46-year-old man with an erythematous, tender, swollen patch on the dorsum of his left fourth toe visited us.
The biopsied tissue showed a proliferation of normally structured eccrine glands and ducts surrounded with abundant materials confirmed as mucin by toluidine blue stain. Mucinous eccrine nevus is an extremely rare entity, and only two cases have been reported in the literature.
We describe a case of mucinous eccrine nevus which was late in onset and presented as a swollen patch.
SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHERCHARACTERIZATION SPECIAL STAINS IMMUNOPEROXIDASE ELECTRON MICROSCOPY
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES BLUE NEVUS
- Sclerosing 'mucinous' blue naevus.
Rongioletti F, Innocenzi D.
Section of Dermatology and Centre of Dermatopathology, DISEM, University of Genoa, Genoa, Italy.
Br J Dermatol. 2003 Jun;148(6):1250-2. Abstract quote
Abundant mucin deposition is an unusual finding in melanocytic naevi.
We describe the first example, to our knowledge, of a sclerosing blue naevus with an abundant mucinous stroma in two unrelated patients.
This uncommon variant of blue naevus should be differentiated from desmoplastic-neurotropic melanoma, in which the presence of mucin stromal deposition is a more typical finding.DERMAL MUCINOSIS
- Cutaneous mucinoses: microscopic criteria for diagnosis.
Rongioletti F, Rebora A.
Department of Epidemiological and Metabolic Diseases, Section of Dermatology, University of Genoa, Via le Benedetto XV, 7-16132 Genoa, Italy.
Am J Dermatopathol. 2001 Jun;23(3):257-67. Abstract quote
The clinical aspects and the histologic features of cutaneous mucinoses have been reviewed and their classification updated.
Cutaneous mucinoses are divided into distinctive (primary) cutaneous mucinoses in which the mucin deposit is the main histologic feature resulting in clinically distinctive lesions, and disorders associated with histologic mucin deposition as an additional finding (secondary mucinoses). The former are further divided into degenerative-inflammatory mucinoses, which may be either dermal or follicular, and into neoplastic-hamartomatous mucinoses.
Histopathologic diagnosis is particularly difficult for dermal mucinoses and requires clinicopathologic correlation. Three histologic clues, namely the pattern of mucin distribution (diffuse or focal), the level of mucin deposit in the dermis and some additional findings may help diagnosis. Follicular mucinoses have the easiest pattern to recognize histologically, but the distinction between Pinkus' follicular mucinosis and follicular mucinosis with mycosis fungoides is very difficult.
Lastly, neoplastic-hamartomatous cutaneous mucinoses include mucinous nevus, a benign hamartoma, and myxoma, which is a benign tumor to be differentiated from reactive cutaneous focal mucinosis.LYMPHEDEMA
- Scalp skin lesion in Turner syndrome: more than lymphoedema?
Debeer A, Steenkiste E, Devriendt K, Morren M.
aNeonatal Intensive Care Unit, Department of Pediatrics bDepartment of Pathology cCenter for Human Genetics dDepartment of Dermatology, University Hospital Leuven, Leuven, Belgium.
Clin Dysmorphol. 2005 Jul;14(3):149-50. Abstract quote
We present a case of a newborn with Turner syndrome (TS) and a scalp skin lesion resembling cutis verticis gyrata (CVG).
Several reports on CVG in TS describe the lesions and correlate it with lymphoedema frequently seen in foetuses and infants with Turner syndrome.
Histological examination in this case, however, shows a congenital mucinous nevus. Possibly an abnormal amount of proteoglycans in the skin of patients with TS can clarify the occurrence of this type of skin lesion.MELANOCYTIC NEVUS
- Mucinous naevocellular naevi.
Michal M.
Sikl's Department of Pathological Anatomy, Medical Faculty, Charles University, Pilsen, Czech Republic.
Dermatology. 1994;188(3):232-5. Abstract quote
We report 4 cases of a naevocellular naevus variant containing abundant amounts of mucinous matrix. The mucinous matrix was Alcian blue (pH 2.5) and Halle's colloidal iron positive and mucicarmine and PAS negative.
When the blocks of tumours were cut down, regions typical of common intradermal naevi were found in all 4 cases. The differential diagnosis with mucinous carcinoma of the skin and myxoid malignant melanoma is discussed.
PROGNOSIS CHARACTERIZATION
TREATMENT CHARACTERIZATION GENERAL 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 15, 2005
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