Clinical Challenge: Solitary Lesion on Hand

Cutaneous focal mucinosis is a skin condition characterized by mucin accumulation primarily in the dermis. Johnson and Helwig first described mucinosis as a solitary lesion in 1966.1 To differentiate these patients from those with multiple lesions, Kuo et al suggested the...

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Cutaneous focal mucinosis is a skin condition characterized by mucin accumulation primarily in the dermis. Johnson and Helwig first described mucinosis as a solitary lesion in 1966.¹ To differentiate these patients from those with multiple lesions, Kuo et al suggested the name solitary cutaneous focal mucinosis.² Cutaneous focal mucinosis are classified as either primary or secondary, with mucin deposition in the latter associated with an underlying condition.^3,4

Solitary lesions in cutaneous focal mucinosis typically present as flesh-colored to white or red, asymptomatic, dome-shaped papules. Lesions appear most commonly on the arms but have been reported on legs, head, neck, and trunk.^3,4 There is a slight male predilection with age of presentation ranging from 29 to 60 years. Clinical differential diagnosis is broad and includes dermatofibroma, neurofibroma, basal cell carcinoma, epidermoid inclusion cysts, seborrheic keratoses, and others.^3-5

As noted, multiple cutaneous focal mucinoses are associated with systemic diseases. These include scleroderma, scleromyxedema, Birt-Hogg-Dube syndrome, thyroid disease, and systemic lupus erythematous.^5-7 A detailed family history and laboratory evaluation are merited in these patients, including thyroid stimulating hormone (TSH), thyroid anti-bodies, serum protein electrophoresis, antinuclear antibody, dsDNA, Smith antibody, and scleroderma 70 antibody.^5-7

Because of the variable morphologic presentation, biopsy of cutaneous focal mucinosis is necessary to establish the diagnosis.^3-5 Hematoxylin and eosin-staining will demonstrate unencapsulated mucin in the upper dermis associated with scattered fibroblasts. Mucin specific stains are useful to confirm the diagnosis.³ Simple local excision of the lesion is the treatment of choice and has a low recurrence rate.

Alexandra Stroia, DO,  is a PGY-1 Transitional Year Resident at Geisinger Community Medical Center, in Scranton, Pennsylvania. Stephen Schleicher, MD, is director of the DermDox Dermatology Centers, associate professor of medicine at Geisinger Commonwealth Medical College, and clinical instructor of dermatology at Arcadia University and Kings College.

References

1. Johnson WC, Helwig EB. Cutaneous focal mucinosis. A clinicopathological and histochemical study. Arch Dermatol. 1966;93(1):13-20. doi:10.1001/archderm.1966.0160019001900

2. Kuo KL, Lee LY, Kuo TT. Solitary cutaneous focal mucinosis: a clinicopathological study of 11 cases of soft fibroma-like cutaneous mucinous lesions. J Dermatol. 2017;44(3):335-338. doi:10.1111/1346-8138.13523

3. Rongioletti F, Rebora A. Les mucinoses cutanées [Cutaneous mucinosis]. Ann Dermatol Venereol. 1993;120(1):75-87.

4. Gutierrez N, Erickson C, Calame A, Cohen PR. Solitary cutaneous focal mucinosis. Cureus. 2021;13(10):e18618. doi:10.7759/cureus.18618

5. Cohen PR, Erickson CP, Calame A. Case report and review of solitary cutaneous focal mucinosis: a unique primary cutaneous mucinosis unrelated to mucinosis-associated systemic diseases. Dermatol Online J. 2020;26(8):13030/qt33m815p3. doi:10.5070/D3268049883

6. Jakubovic HR, Salama SS, Rosenthal D. Multiple cutaneous focal mucinoses with hypothyroidism. Ann Intern Med. 1982;96(1):56-58. doi:10.7326/0003-4819-96-1-56

7. Baxi KD, Chaudhary RG, Rathod SP, Jagati A. Monoclonal gammopathy of undetermined significance-associated scleromyxoedema. Indian Dermatol Online J. 2019;10(1):54-57. doi:10.4103/idoj.IDOJ_138_18