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Fabry disease is a X-linked lysosomal storage disorder due to alpha galactosidase A deficiency leading to abnormal accumulation of glycosphingolipids in different parts of body. This case report introduces a 35-year-old man with diffuse keratotic erythematous papules. Histopathological evaluation of the skin biopsy suggested the diagnosis of angiokeratoma. With attention to his nephropathy and the same skin lesions and renal involvement in his brother, which led him to receive renal transplantation, the diagnosis of Fabry disease was suggested. 

Background and Aim: Cicatricial alopecia refers to lesions that result in permanent and irreversible hair loss and are associated with destruction of hair follicles. In this alopecias the hair follicle is replaced with connective tissue. After remission of initial infection or inflammation, hair regrowth is unlikely.

Methods: In this retrospective cross-sectional study, 222 patients with cicatricial alopecia, admitted to Farshchian Hospital, Hamadan, Iran were investigated. Hospital records of patients with cicatricial alopecia were reviewed, and their demographic as well as clinical data were extracted and entered into prepared data gathering forms.

Results: One-hundred and ten patients (49.5%) had discoid lupus erythematosus, 71 patients (31.9%) had lichen planopilaris, 23 patients (10.4%) had pseudopelade of Brocq, 11 patients (4.9%) had keratosis pilaris spinulosa decalvans and 3 patients (1.3%) had central centrifugal cicatricial alopecia. Each of alopecia mucinosa and dissecting cellulitis/folliculitis were observed in 2 patients (0.9%). The correlation between of illness, extent of disease, gender, location, occupation, and clinical features of the disease were studied.

Conclusion: Cicatricial alopecia usually results in irreversible hair loss, but early diagnosis and treatment can prevent the progression of the lesions and its complications. The diagnosis of this disease is possible with a clinicopathologic study.

Background and Aim: Direct immunofluorescence (DIF) is represented as a gold standard method in diagnosis of autoimmune blistering dermatoses. Normal saline, liquid nitrogen and michel’ solution are a widely accepted media for preserving biopsy samples of skin or mucosa before DIF examination. Occasionally clinicians put the biopsy specimen taken for DIF in formalin 10%, occasionally clinicians ordered DIF retrogradely and only a paraffin-embedded biopsy specimen exposed to formalin 10% is available. To determine the diagnostic value of DIF when it was performed on biopsy samples of skin or mucosa exposed to formalin 10% in comparison to the same biopsy samples exposed to normal saline.
 

Methods: In 74 patients (38 immunobullous and 23 chronic dermatitis), which the latter served as the normal controls, 2 perilesional punch biopsy of skin or mucosa were done, one put in formalin 10% and fixed in paraffin and one put in normal saline, and DIF was done on both samples. 
 

Results: DIF sensitivity and specificity was with IgG 31.5% and 100% in pemphigus and 15.36% and 93.44% in BP, with C3 39.47% and 100% in pemphigus and 7.69% and 91.80% in BP, respectively.
 

Conclusion: DIF on specimens exposed to formalin 10% in comparison to specimens exposed to normal saline is less sensitive but approximately as specific as it is in the diagnosis of pemphigus and BP patients and especially can be useful in pemphigus patients when only a formalin exposed samples is available.