engleski

A patient with acute onset of generalized comedones and cysts

A 64-year-old male patient presented with widespread comedones, cysts, eczematous and indurated lesions, particularly affecting the face and posterior neck, as well as the thighs and upper trunk. According to patient history, these lesions had an acute onset, appeared 6 months prior to our examination and were accompanied by severe pruritus. At the same time, the patient noticed absence of sweating while playing tennis, apart from the palms of hands and soles of feet. He was seen by several dermatologists and prescribed local corticosteroid treatment with no effect. He also visited a beautician for comedone extraction. The lesions persistently worsened. Physical examination demonstrated erythematous and indurated skin, leonine facies, widespread comedones and white papules 5 mm in size. The scalp and beard showed alopecic lesions. Initially, a punch biopsy was preformed which revealed a cutaneous keratocyst. As laboratory tests showed increased LDH, tumor markers, ultrasound of the abdomen, inguinal and axillary regions were subsequently performed, however no abnormalities were found. Two excisional biopsies were then performed which showed nonspecific findings and were sent for further immunohistochemistry analysis. PET-CT scan revealed metabolically active diffuse thickening of the skin and subcutaneous tissues of the head, body and extremities of uncertain etiology ; hypermetabolic lymph nodes in the bilateral inguinal region, a nonspecific finding. Further follow up ultrasound and/or ultrasound guided fine needle aspiration biopsy was recommended. Based on clinical and laboratory findings, the diagnosis of folliculotropic mycosis fungicides (MF) was made. Folliculotropic MF is found in approximately 10% of MF patients. As the clinical manifestations are variable and include follicle-based patches, plaques, infiltrated plaques, tumors, and prurigo nodularis-like lesions, as well as keratosis pilaris-like lesions and acneiform lesions such as comedones and cysts, the diagnosis may be difficult. One study reported a mean time of 2.8 (range 0.3 to 10) years between the onset of the eruption and the biopsyproven diagnosis of folliculotropic MF. It was previously considered an aggressive variant of MF, however newer studies suggested this may not always be the case.

folliculotropic mycosis fungicides ; immunohistochemistry ; comedone ; lesions

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