Search

A rare case of neurosyphilis with oral condyloma lata and fingernail damage is reported. A 49-year-old male presented with a two-month history of copper-red macules on the palms of both hands and verrucous plaques on the tongue tip for 1 month, and dark shadows on the left eye for 1 week. Dermatological examination revealed uneven and partially thickened nail surfaces of the left thumb and right index finger. The palms were scattered with copper-red macules in the size of mung beans, surrounded by collar-like desquamation. Flat-topped verrucous plaques could be seen on the mucosa of the tongue tip. Hearing was slightly less in the left ear than in the right ear. Corrected visual acuity was 1.0 in the right eye and 0.8 in the left eye. Laboratory tests showed positive for both serum Treponema pallidum particle agglutination (TPPA) (>1∶1 280) and Tolulized red unheated serum test (TRUST) (1∶64). Moreover, the cerebrospinal fluid (CSF) was positive for TRUST(1∶1), TPPA(1∶640) and venereal disease research laboratory (VDRL) (1∶2). The presence of Treponema Pallidum (T. Pallium) in oral verrucous plaque was confirmed by metagenomics next-generation sequencing (mNGS) technology. The diagnosis was neurosyphilis, secondary syphilis (oral flat condyloma). After 2 weeks of treatment with aqua penicillin at a dose of 4 million U q4h, almost all clinical manifestations were improved. Serum TRUST titer was decreased to 1∶8 after 3 months. The follow-up is still ongoing.

Objective To report a case of hemophagocytic lymphohistiocytosis syndrome (HLH) secondary to systemic hydroa vacciniforme lymphoproliferative disorder (systemic HVLPD) and review relevant literature, in order to improve understanding of the disease. Methods Clinical data of the case, laboratory examination, histopathology, immunohistochemistry, TCR gene rearrangement, and whole exon gene sequencing were collected for analyses. Results A 19 years old man was admitted to the hospital due to generalized papules, blisters, necrotic scabs and fever for 7 months. The clinical manifestations included hydroa vacciniforme-like rashes, lymphadenopathy, splenomegaly, pancytopenia, hypertriglyceridemia, decreased NK cell activity, high levels of sIL-2r, positive for VCA-IgA and EA-IgA, and elevated EBV DNA load. The whole exon sequencing did not show pathogenic gene mutations. Histopathological changes in the skin lesions included liquefaction degeneration of epidermal and follicular basal cells, pigmentary incontinence, lymphocytic infiltrates of the epidermis and follicular epithelium, perivascular and perifollicular infiltrates of lymphocytes and eosinophils in the dermis, extravasation of erythrocytes, as well as focal infiltrates of atypical lymphoid cells around appendages and small blood vessels. Pathologic mitosis was visible. Immunohistochemistry showed CD3 (+), CD5 (+), CD20 (individual+), CD30 (small patch+), CD79a (individual+), CD4 (+), CD7 (+), CD8 (+), TIA-1 (+), CD56 (scattered lymphocyte+), ≈50% of Ki-67(+), and in situ hybridization EBER (+). TCRβ gene monoclonal rearrangements were detected. According to the results of clinical and auxiliary examination, patient was diagnosed with HLH secondary to systemic HVLPD. After 8 months of follow-up, the patient passed away while waiting for transplantation of allogeneic hematopoietic stem cells. Conclusions Systemic HVLPD is rare and requires vigilance for the development of secondary hemophagocytic syndrome. The awareness of hemophagocytic syndrome should be improved to avoid possible misdiagnosis and delayed treatment.