A 13-year-old girl, without any medical history, presented with a fever of 39.5^o, headache and vomiting evolving one week before her hospitalization in pediatric department. The patient was stable hemodynamically, lethargic, and exhibited neck stiffness. Dermatological examination found two painless and non-pruritic ulcerated nodules with hemorrhagic crusts evolving for 10 days on the face (Fig. 1a and 1b). The dermoscopy revealed a central ulceration, fine whitish scales and polymorphic vascular structures (Fig. 1c). The biological tests were normal.

Figure 1: (a and b) Central ulcerated nodules with hemorrhagic crusts on the face. (c) Dermoscopy revealing a central ulceration, fine whitish scales and polymorphic vascular structures made of linear vessels and telangiectasias. (d) Histopathology showing an ulcerated epidermis and a mucoid infiltrate in the dermis with spherical spores extending into the hypodermis.

WHAT IS YOUR DIAGNOSIS?

A skin biopsy was performed revealing an ulcerated epidermis, a mucoid infiltrate in the dermis, spherical spores of variable size with a thick capsule creating a clear halo and extending into the hypodermis (Fig. 1d). This histological appearance was consistent with cutaneous cryptococcosis.

The patient refused to stay for the study of cerebrospinal fluid and Human immunodeficiency virus HIV serology and came back a week later with a complicated meningoencephalitis leading to her death few hours later. The skin biopsy results allowed us to retain the diagnosis of cutaneous cryptococcosis probably associated with a disseminated form which was responsible for the meningoencephalitis.

Cryptococcosis is a fungal infection caused by Cryptococcus neoformans, an encapsulated germ found in pigeons and other birds. Humans are infected by the respiratory route, by inhalation. It has been initially described in Acquired Immunodeficiency Syndrome.

AIDS patients [1], but has been reported in many immunocompetent individuals as well. Adults are predominately affected, as there are fewer than 100 reported cases in children [2].

The most frequently reported form is meningoencephalitis, the cutaneous form is rare (5 to 15% of all cryptococcosis cases) [3] and often remains a sentinel for disseminated disease; however, primary skin lesions do occur in immunocompetent individuals [4]. However, primary cutaneous cryptococcosis is a diagnosis of exclusion.

Cutaneous manifestations predominantly occur on the head and neck (78%), the clinical features are various [3], the most frequent form remains the molluscum contagiosum like lesion presenting an ulcerated papule in its center as found in our case, especially in patients with HIV.

Dermoscopy of cutaneous cryptococcosis has been reported only once by Slawinska and al showing the presence of white structureless areas with linear irregular and branched vessels surrounded with yellowish halo [5].

The diagnosis of cryptococcosis is based on the detection of a yeast with a specific polysaccharide capsule (glucuronoxylomannan) of the genus Cryptococcus on direct examination. Culture on Sabouraud is essential for the identification of the species. Histopathological diagnosis should also be performed isolating pathogenic fungi in the tissue.

The current guidelines of the Infectious Diseases Society of America IDSA recommend amphotericin B based combination therapy with flucytocine as primary induction therapy followed by oral fluconazole consolidation therapy [6].

Finally, it should be borne in mind that the primary cutaneous form of cryptococcosis is rare and should be systematically investigated for an internal focus before considering treatment options.

REFERENCES

1. Wafa A, Hicham H, Yassir A, Zoubida TM, Mohamed A, Mohamed A, et al. Une cryptococcose disséminée compliquant un traitement par prednisone et azathioprime d’un pemphigus vulgaire. Pan African Med J. 2011;10:35.

2. Lenz D, Held J, Goerke S, Wagner D, Tintelnot K, Henneke P, et al. Primary Cutaneous Cryptococcosis in an Eight-year-old Immunocompetent Child:How to Treat?Klin Padiatr. 2015;227:41-4.

3. Noguchi H, Matsumoto T, Kimura U, Hiruma M, Kusuhara M, Ihn H. Cutaneous Cryptococcosis. Med Mycol J. 2019;60:101-7.

4. Moreno Castillo JL, Del Negro G, Heins-Vaccari E, de Melo NT. Primary cutaneous cryptococcosis. Mycopathologia. 1986;96:25-8.

5. Sławińska M, Hlebowicz M, Iżycka-Świeszewska E, Sikorska M, Sokołowska-Wojdyło M, Smiatacz T, et al. Dermoscopic observations in disseminated cryptococcosis with cutaneous involvement. J Eur Acad Dermatol Venereol. 2018;32:e223-4.

6. Probst C, Pongratz G, Capellino S, Szeimies RM, Schölmerich J, Fleck M, et al. Cryptococcosis mimicking cutaneous cellulitis in a patient suffering from rheumatoid arthritis:a case report. BMC Infectious Diseases. 2010;10:239.