Rubbery plaques on the shoulders, upper back, and chest

Case #1Keloids are benign dermal fibroproliferative tumors that usually develop in areas of trauma (eg, after injury or surgery). Less commonly, keloids may develop as part of systemic disease, infection, or genetic syndromes. Development of spontaneous keloids, de novo, is...

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Case #1

Keloids are benign dermal fibroproliferative tumors that usually develop in areas of trauma (eg, after injury or surgery). Less commonly, keloids may develop as part of systemic disease, infection, or genetic syndromes. Development of spontaneous keloids, de novo, is rare.¹

Keloids occur more commonly in the African American population than in the white population, with an estimated 2:1 to 19:1 ratio, respectively.² Both sexes are affected equally, and age of onset is usually between 10 and 30 years. There is likely a genetic component, as keloids have a familial predilection.

Keloids present as rubbery or firm, pink or red papules or plaques. Early lesions typically appear more inflamed and red, whereas older lesions are paler and may appear mildly hyperpigmented. Lesions can be symmetric with regular margins, or they can present as irregular claw-like protrusions. The lesions are often pruritic or tender. The chest, back, shoulders, and earlobes are the most common areas of involvement.

Unlike hypertrophic scars, keloids extend beyond the borders of the original wound, do not regress spontaneously, and tend to recur after excision.³ Keloids are limited to dermal invasion, except for the rare case of keloid of the cornea.

When injury or surgical involvement precedes the development of keloids, clinical diagnosis is made based on the typical overgrowth of scar tissue beyond the boundary of the original wound.

When a diagnosis of spontaneous keloidosis is suspected, a skin biopsy is appropriate to confirm the diagnosis. A malignant tumor that is treated like a keloid can yield disastrous or even fatal results. Single keloids can mimic benign lesions such as dermatofibromas or prurigo nodularis. Malignant mimickers to rule out include amelanotic melanoma, Merkel cell carcinoma, carcinoma en cuirasse,⁴ and cutaneous lymphoma. Dermatologic diseases that may be associated with keloid formation include acne vulgaris, dissecting cellulitis of the scalp, hidradenitis suppurativa, and acne conglobata. Keloidal scleroderma can look like multiple keloids⁵; however, the former diagnosis requires a thorough systemic workup and close observation to detect onset of systemic sarcoidosis.

On histopathologic examination, keloids reveal a collection of atypical fibroblasts with excessive deposition of extracellular matrix components, especially collagen, fibronectin, elastin, and proteoglycans. Thick, hyalinized collagen bundles are usually seen in the deep dermal portion of the lesion.

For patients with a known history of keloid formation, prevention is crucial. Unnecessary surgical procedures, such as skin piercings, should be avoided. Occlusive dressings and compression therapy can be used on new areas of trauma to minimize the likelihood of keloid formation.

Numerous treatment options exist, although success is variable. Traditional treatment is injections of cortisone, such as triamcinolone, directly into the lesion. Concentrations from 10 to 40 mg/mL are appropriate, depending on lesion thickness and location.

Surgical excision alone is not recommended, as the risk of keloid recurrence is high. In the appropriate scenario, surgical treatment may be attempted, in combination with early postoperative cortisone injections, radiation treatment, or pressure dressings. Interferon therapy has not been proved effective, and cryotherapy is likely equally ineffective, with significant risk of scarring and pigment alteration.⁶ One older study found topical tretinoin 0.05% cream to be effective at improving the size, thickness, and itching of the lesions.² Other treatments with variable efficacies include pulsed dye laser, 5-fluorouracil, and calcium channel blockers.⁴

For the patient in this case, results of a skin biopsy confirmed the diagnosis of keloids. Treatment options were discussed, and the patient chose to undergo intralesional triamcinolone injections at monthly intervals. After 3 months of treatment, there was significant flattening of most lesions, and the patient reported a significant improvement of itching.

Case #2

Sarcoidosis is a multiorgan disease characterized by the presence of noncaseating granulomas. Skin involvement is referred to as cutaneous sarcoidosis and occurs in 25% to 30% of cases of systemic sarcoidosis.⁴ Skin involvement may occur prior to the development of systemic disease, simultaneously, or even years after systemic disease appeared. Approximately 30% of patients with cutaneous sarcoidosis will progress to multiorgan involvement within 1 year.⁷ Lung disease and hilar lymphadenopathy is the most frequent component of sarcoidosis. Alternately, the skin may be the only organ involved.

Women, as well as people of African-American and Scandinavian descent, are at higher risk for the development of sarcoidosis. The disease tends occur most commonly in the third and fourth decades of life.⁴

Although the exact etiology of sarcoidosis is unknown, it is believed to involve genetic factors as well as environmental exposures, infectious agents, and antigen-immune responses. Traditionally, mycobacteria have been implicated as a possible etiologic agent; more recently Propionibacterium acnes, herpesvirus, and Epstein-Barr virus have been considered. Some noninfectious exposures that have been considered as triggers include injectable dermal fillers,⁸ contact allergens, and metals. Cutaneous disease often presents within scars, areas of trauma, or areas where foreign material is deposited.

Cutaneous sarcoidosis is classified as specific, referring to cases with biopsy-proven noncaseating granulomas, or nonspecific, which is mainly reactive processes that do not exhibit granulomas. Erythema nodosum is the most common nonspecific finding in sarcoidosis.

Cutaneous sarcoidosis is often referred to as the great mimicker because its presentation can vary greatly, and it often resembles many common and rare dermatologic conditions.⁹ Some of the forms of presentation of specific lesions include papular, plaque, photodistributed, psoriasiform, tumoral, mucosal, atrophic, ulcerative, alopecia, and scar-like.⁹ The papular type usually presents on the face, specifically around the eyes and on the nasolabial folds, with red-brown, flat-topped papules and plaques. Lupus pernio is a variant, and presents with brown to violaceous, smooth, shiny plaques on the head and neck. These lesions are often most disfiguring and are most strongly associated with systemic disease.

A skin biopsy is necessary to diagnose cutaneous sarcoidosis. Sarcoid lesions are characterized as “naked,” noncaseating granulomas with a sparse lymphocytic infiltrate at the margins of granulomas. The granulomas are generally circumscribed and composed of epithelioid cells, some with small foci of necrosis in the center.

The histologic differential diagnosis is broad; therefore, a definitive diagnosis requires further workup. Sarcoid skin lesions often present with the same histologic pattern as necrobiosis lipoidica lesions.¹⁰ Granulomatous sarcoidosis may also mimic granulomatous secondary syphilis and granulomatous T-cell lymphoma.

In a patient diagnosed with cutaneous sarcoidosis, the first step in the workup to exclude systemic involvement should be a chest radiograph, looking for hilar adenopathy and lung involvement. Ophthalmologic evaluation, measurement of serum angiotensin-converting enzyme (ACE) level, electrocardiograph, and other baseline laboratory testing should be considered.

Although numerous treatment options exist, there are very few published reputable trials to provide clear guidelines. Many lesions resolve spontaneously; therefore, observation alone may be sufficient. Often, however, patients request treatment to improve cosmetic appearance. In addition, disfiguring and symptomatic lesions should be treated.

Haimovic and colleagues⁹ recommend intralesional and topical corticosteroid treatment as a first-line treatment option for localized and mild disease. The usual dose for topical therapy includes potent topical steroids, such as clobetasol or halobetasol, applied with or without occlusion twice weekly. Triamcinolone, 3 to 10 mg/mL, can be injected intralesionally every 3 to 4 weeks until lesions clear.

Systemic corticosteroids are helpful. However, the high dosage necessary makes them unsafe for long-term use; therefore, they should be reserved for rapidly progressing cases. Topical tacrolimus and phototherapy may be beneficial for thinner lesions. More recent literature encourages the use of oral doxycycline or minocycline,¹¹ with a maximum response noted at 3 months. The antimalarial agents, chloroquine and hydroquinone, and infliximab infusions,¹² are reserved for more serious cases. One case report involved successful treatment with methotrexate after failure to respond to intralesional and systemic steroids.¹³

The patient in this case was referred for baseline laboratory testing, serum ACE level measurement, electrocardiographic testing, and ophthalmologic evaluation; results of all tests were within normal limits. After discussing treatment options, the patient chose to observe the lesions and not actively treat them unless the cutaneous signs worsened.

Esther Stern, NP, is a family nurse practitioner at Advanced Dermatology & Skin Surgery, P.C., in Lakewood, N.J.

References

1. Sage R, Kwon J. Extensive spontaneous keloidosis: a case report. J Am Acad Dermatol. 2010;62(suppl 1):AB32.
2. Halder RM. The role of retinoids in the management of cutaneous conditions in blacks. J Am Acad Dermatol. 1998;39(2 Pt 3):S98-S103.
3. Berman B, Bieley HC. Keloids. J Am Acad Dermatol. 1995;33:117-123.
4. James WD, Berger TG, Elston DM. Andrews’ Diseases of the Skin: Clinical Dermatology. 11th ed. Philadelphia, PA: Saunders-Elsevier; 2011.
5. Heath CR, David JN, Taylor SC. Nodular scleroderma presenting as multiple spontaneous keloidal scars. J Am Acad Dermatol. 2012;66:e245-e246.
6. Shaffer JJ, Taylor SC, Cook-Bolden F. Keloidal scars: a review with a critical look at therapeutic options. J Am Acad Dermatol. 2002;46(2 suppl):S63-S97.
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8. Santos-Alarcón S, Sanchis-Sánchez C, Benavente-Villegas F-C, Mateu-Puchades A, Camarasa-Lillo N. Cutaneous sarcoidosis during interferon and ribavirin therapy in a patient with hepatitis C virus infection located at silicone injection sites. J Am Acad Dermatol. 2016;74:AB15.
9. Haimovic A, Sanchez M, Judson MA, Prystowsky S. Sarcoidosis: a comprehensive review and update for the dermatologist: part I. Cutaneous disease. J Am Acad Dermatol. 2012;66:699.e1-699.e18.
10. Nappi F, Laurain D, Jalalat S, Saco M, Fenske N. Cutaneous sarcoid with necrobiosis lipoidica-like histology. J Am Acad Dermatol. 2016;74(suppl 1):AB97.
11. Steen T, English JC. Oral minocycline in treatment of cutaneous sarcoidosis. JAMA Dermatol. 2013;149:758-760.
12. Ahmed A, Morar N. Infliximab treatment for refractory cutaneous sarcoidosis: experience from a tertiary referral center. J Am Acad Dermatol. 2013;68(suppl 1):AB53.
13. Ringger B, Sluzevich J. Recalcitrant cutaneous sarcoidosis successfully treated with methotrexate: a case report. J Am Acad Dermatol. 2012;66(suppl 1):AB60.