Primary Capsule-Deficient Cutaneous Cryptococcosis in a Sporotrichoid Pattern in an Immunocompetent Host

Primary Capsule-Deficient Cutaneous Cryptococcosis in a Sporotrichoid Pattern in an Immunocompetent Host Nathan Andrew Merl Jackson, DO; Daniel B. Herring, MD Practice Points Cryptococcus neoformans is an encapsulated yeast that is ubiquitous in the environment and is especially abundant in soil enriched with pigeon droppings. Immunocompetent hosts often are asymptomatic or have only mild pulmonary disease, while disseminated disease affects the lungs, central nervous system, bones, and skin in immunocompromised hosts. Diagnostic tests include india ink or mucicarmine staining to highlight characteristic capsules or the latex agglutination test to measure circulating capsular antigen. copy not Cryptococcosis is an opportunistic yeast infectionDo ryptococcosis is an opportunistic yeast infection caused by Cryptococcus neoformans that remains caused by Cryptococcus neoformans that remains the most common systemic fungal infection in Cthe most common systemic fungal infection in immunosuppressed patients and often pres- immunosuppressed patients and often presents with ents with signs of meningitis. Primary cutaneous signs of meningitis. Cutaneous cryptococcosis occurs cryptococcosis (PCC) is a more rare clinical iden- in 10% to 20% of systemic Cryptococcus infections and tity that is characterized by skin lesions confined usually is secondary to hematogenous dissemination in to 1 body region, often presenting as a whitlow or patients with an underlying disease, particularly human phlegmon with positive cultureCUTIS for C neoformans immunodeficiency virus. Primary cutaneous crypto- and no evidence of simultaneous dissemination. coccosis (PCC) is a more rare clinical identity that We report a rare case of PCC in a 73-year-old man is characterized by skin lesions confined to 1 body with intact cell-mediated immunity. region, often presenting as a whitlow or phlegmon Cutis. 2015;96:E26-E29. with positive culture for C neoformans and no evidence of simultaneous dissemination. We report a rare case of PCC in a 73-year-old man with intact cell-mediated immunity. Case Report A 73-year-old man who was a beef farmer presented on primary care referral with multiple red nodules and ulcers on the right third and fourth digits and Dr. Jackson is from West Virginia School of Osteopathic Medicine, distal forearm following abrasion to the region. The Lewisburg. Dr. Herring is from McCagh, Roberts & Herring patient reported that the lesions had started as pain- Dermatology, Cumberland, Maryland. The authors report no conflict of interest. ful nodules that would open and drain. He had been Correspondence: Nathan Andrew Merl Jackson, DO, 11007 Blan taking oral ciprofloxacin and oral ketoconazole for Avon Rd, Midlothian, MD 21543 ([email protected]). 3 days as prescribed by his primary care physician but E26 CUTIS® WWW.CUTIS.COM Copyright Cutis 2015. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. Primary Cutaneous Cryptococcosis had not begun to see results. He denied any travel did not reveal dark red capsules characteristic of or exposure to roses, fish tanks, or any sick contacts. Cryptococcus (Figure 4). The pathology report indi- A review of systems was negative for fever, night cated that the findings may represent sporotrichosis sweats, malaise, headache, or any other systemic in the appropriate clinical setting, but GMS staining symptoms. Physical examination revealed multiple could not definitively classify Sporothrix schenckii or 2- to 6-mm nodules and ulcers distributed in a sporo- rule out other fungal infections without tissue cul- trichoid pattern on the right hand (Figure 1) and arm ture. Before culture results could be obtained, the (Figure 2). Lymphadenopathy was absent and the patient returned 2 weeks later for suture removal at rest of the examination revealed no abnormalities. which point the prior medications were stopped and Initially, 4 punch biopsies of the right hand itraconazole 200 mg once daily was initiated. and arm were obtained and sent for Gram stain- Upon receiving the culture results, a diagnosis of ing, tissue culture (bacterial and fungal), and his- primary capsule-deficient cutaneous cryptococcosis topathologic review. A presumptive diagnosis of was made. The lesions showed clinical improvement sporotrichosis was made, with change of treatment at 1-month follow-up, and treatment with itracon- pending culture. On routine hematoxylin and eosin azole was continued with monthly liver function staining, marked acute and chronic granuloma- tests. After 5 months of continued improvement, the tous inflammation with microabscesses was noted. Acid-fast bacilli staining was negative. Follow-up Gomori methenamine-silver (GMS) staining showed numerous fungal spores with narrow base budding (Figure 3). Subsequent mucicarmine staining copy not Do Figure 3. Numerous fungal spores with narrow base budding on Gomori methenamine-silver staining (original magnification 100). CUTIS Figure 1. Ulceration of the right fourth digit and palm. Figure 4. Mucicarmine staining showed an absence of Figure 2. Multiple nodules and ulcers distributed in a characteristic red Cryptococcus neoformans capsules sporotrichoid pattern on the right arm. (original magnification 100). WWW.CUTIS.COM VOLUME 96, JULY 2015 E27 Copyright Cutis 2015. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. Primary Cutaneous Cryptococcosis itraconazole dose was decreased to 100 mg once daily characteristically measures 3 to 20 μm in diam- for 1 month. The patient was free of lesions and any eter and stains well with periodic acid–Schiff stain sequelae at 6-month and 1-year follow-up. and GMS.4,5 Therefore, the lack of capsule broad- ened our early differential to include Histoplasma Comment capsulatum, S schenckii, Paracoccidioides brasiliensis, Cryptococcosis is caused by C neoformans, an oppor- and even Blastomyces dermatitidis. tunistic, basidiomycetous, yeastlike fungus1 that Neuville et al1 proposed the following criteria presents as a yeast in both the environment and tis- for the diagnosis of PCC: the absence of dissemina- sue and normally is associated with immunocompro- tion and predominantly a solitary skin lesion on mised host infection, especially in individuals with unclothed areas presenting as a whitlow or phleg- human immunodeficiency virus. The most common mon, a history of skin injury or damage leading to route of infection is through the lungs as respiratory direct inoculation, participation in outdoor activi- droplets followed by hematogenous dissemination to ties, exposure to bird droppings, and isolation of the central nervous system and skin, with meningitis C neoformans serotype D. Other factors that strongly being the most common clinical manifestation and support PCC diagnosis over squamous cell carci- Cryptococcus being the most common cause of fun- noma (based on a review of the literature) are rural gal meningitis worldwide.2 Cutaneous involvement residential environment, older age, equal prevalence after hematogenous spread (secondary cutaneous among men and women, and lack of underlying cryptococcosis) is reported in 10% to 20% of systemic disease. Presence of these factors seem to favor PCC Cryptococcus cases, while PCC is limited to rare cases over squamous cell carcinoma, as some still consider in which trauma or abrasions to the affected site are the existence of PCC in general to be controversial notable risk factors.2,3 because skin manifestations represent a sentinel Cryptococcus can produce a myriad of skin mani- finding indicativecopy of disseminated disease.1,3 festations including but not limited to nodules, The fungus can be found worldwide as an ubiq- ulcers, plaques, pustules, vesicobullous lesions, and uitous saprophyte of soil, especially if the soil is draining sinuses. Neuville et al1 found that cellulitis, enriched with pigeon droppings. A link between cutaneous ulcers, and whitlows were the most com- C neoformansnot and pigeons has been suggested, with mon presenting clinical features in PCC. Whitlows dried avian excreta allowing the yeast to abundantly also have been reported as a rare presentation in grow because of its high nitrogen content.5,6 Other secondary cutaneous cryptococcosis yielding to the possible sites include decaying wood, fruits, veg- much more prevalent presentation of umbilicatedDo etables, and dust.1 There are 4 main serotypes and papules resembling molluscum contagiosum.1 This 3 varieties of C neoformans: C neoformans var grubii polymorphic identity can therefore mimic not only (serotype A; worldwide distribution), C neoformans other dermatoses and neoplasms but other infections var gattii (serotypes B and C; more circumscribed such as bacterial cellulitis, herpes simplex virus, and diffusion and distribution including subtropical molluscum contagiosum, especially in disseminated regions of Australia, Central Africa, South Asia, cryptococcosis, making microscopic assessment cru- and California), and C neoformans var neoformans cial for the diagnostic confirmationCUTIS of cutaneous (serotype D; worldwide distribution).3,7 A litera- cryptococcosis. The differential diagnosis includes ture review indicated that known cases of sero- sporotrichosis and Mycobacterium marinum due to type D (global incidence, 9%) tended to produce the lymphatic distribution of the lesions as well as cutaneous lesions without systemic involvement.7

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