Verrucous and macular sarcoidosis
Published Web Locationhttps://doi.org/10.5070/D33c68904v
Verrucous and macular sarcoidosisThe Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York
Taylor DeFelice MD MPH, Max Fischer MD, Hideko Kamino MD, David Cohen MD MPH, Jo-Ann Latkowski MD
Dermatology Online Journal 18 (12): 10
We present a case of cutaneous sarcoidosis in a 77-year-old woman without systemic disease, who presented with two subtypes of lesions: erythematous and hyperpigmented macules on the arms and anterior upper legs and erythematous, hyperkeratotic nodules, some crateriform, on the lower legs and feet. Whereas cutaneous sarcoidosis is present in up to one-third of cases and may present with a wide variety of lesions, verrucous sarcoidosis is uncommon. Diagnosis is by clinical and histopathologic exclusion of other entities, which include other causes of granulomatous inflammation such as infections and foreign-body reactions. Management options include topical, intralesional, or systemic glucocorticoids, antimalarials, tetracycline derivatives, and methotrexate.
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A 77-year-old woman presented to the New York University Dermatologic Associates in May, 2010, for evaluation of multiple erythematous, hyperkeratotic nodules on the lower legs and feet as well as erythematous and hyperpigmented macules on the arms and thighs. The lesions had been present for approximately three years and were increasing in number, although some individual lesions had decreased in size. They were not exudative but were sometimes painful. She denied any systemic symptoms, such as fevers, shortness of breath, cough, joint or muscle pains, nausea, vomiting, diarrhea, night sweats, or weight loss.
Past medical history included Sjögren syndrome and a basal-cell carcinoma. She was taking no medications and had no known allergies. Three punch biopsies were taken of lesions on the left thigh, right posterior lower leg, and right medial malleolar region.
Erythematous and hyperpigmented macules were present on the arms and anterior aspect of the upper legs. On the right lower leg and feet were erythematous, hyperkeratotic nodules, some crateriform.
Bacterial, mycobacterial, and fungal cultures of a portion of the biopsy specimen from the right posterior lower leg demonstrated no growth. A chest computed tomography scan was normal.
Within the reticular dermis and superficial subcutaneous tissue, there are nodular aggregates of epithelioid histiocytes and multinucleated giant cells with surrounding lymphocytes. There is no evidence of foreign material under examination with polarized light. Periodic acid-Schiff, Ziehl-Neelsen, and Gram stains fail to reveal microorganisms.
Sarcoidosis is a multisystem granulomatous disease that most often affects the lungs and intrathoracic lymph nodes. Cutaneous involvement occurs in up to one-third of cases and not infrequently may be the only organ affected . Skin lesions are divided into non-specific inflammatory types, the most common of which is erythema nodosum, and specific lesions, which histopathologically demonstrate non-caseating granulomas and are more often associated with chronic disease . Specific sarcoidal skin manifestations often present a diagnostic challenge because lesions are heterogeneous; cutaneous sarcoidosis is another great imitator . Lesions may present as macules, papules, plaques, nodules, ulcers, infiltrated scars, subcutaneous lesions, erythroderma, and scarring alopecia; they may be skin-colored, yellow-translucent, hyper- or hypopigmented, or erythematous-to-violaceous in color [2, 3]. Additionally, epidermal changes may be absent or include atrophy, hyperkeratosis, or telangiectasia . Subtypes of cutaneous sarcoidosis may present in the same patient, as was the case in our patient .
Verrucous sarcoidosis is a rare variant, which presents with lesions that resemble warts or severe psoriasis . Papules, papulonodules, or plaques are typically well-demarcated, exophytic, and hyperkeratotic. Verrucous lesions are reported most often in African-American patients on the legs. They may occur either alone or in conjunction with ulcerative, papillomatous, or typical papular lesions [5-12]. The majority of patients with this subtype have had pulmonary involvement, except one adolescent with exclusively skin involvement . The differential diagnosis includes prurigo nodularis and hypertrophic lichen planus as well as deep fungal or atypical mycobacterial infections [4, 5]. Biopsy specimens of verrucous sarcoidosis exhibit acanthosis and hyperkeratosis as well as the typical dermal non-caseating epithelioid granulomas, sometimes with central fibrinoid necrosis, that is surrounded by an inflammatory cell infiltrate .
Diagnosis of cutaneous sarcoidosis is established by clinical and histopathologic exclusion of other etiologies. Performance of polarized light microscopy for foreign-body reactions and stains and tissue cultures for fungal and mycobacterial infections are necessary when granulomatous inflammation is demonstrated in a biopsy specimen . Patients with exclusively cutaneous manifestations need close follow up evaluations because systemic disease may develop eventually .
Treatment of cutaneous lesions is generally initiated to prevent disfigurement and impairment of function . For limited disease, potent topical glucocorticoids or a series of intralesional injections of triamcinolone may be effective; oral glucocorticoids are reserved for recalcitrant or widespread involvement [2, 3, 4]. Use of tetracycline derivatives, antimalarials, and methotrexate is well-supported in the literature and some reports suggest the efficacy of infliximab, adalimumab, thalidomide, isotretinoin, and laser modalities in refractory cases [2, 3, 4]. Relapse after discontinuation of therapy is common. For verrucous sarcoidosis, improvement has been noted most commonly after treatment with topical and systemic glucocorticoids; verrucous sarcoidosis has a propensity to leave residual scars upon resolution [5, 6, 7, 11].
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