PubMed · 5548477
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J L Verbov, P F Borrie. 1971. Diffuse cutaneous mastocytosis.. https://pubmed.ncbi.nlm.nih.gov/5548477/
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Congenital poikiloderma is an uncommon hereditary disorder. It has been reported in association with various syndrome. No case has previously been reported from this environment. We report a case of congenital poikiloderma in a two and a half year old female Nigerian associated with unusual generalised hypopigmentation and acral blisters at birth. The child subsequently developed macular hyperpigmentation on an erythematous background and atrophy of the skin. Although she had some features which were suggestive of Rothmund-Thomson syndrome (RTS), the presence of hypopigmentation at birth, along with acral blistering, was noted to be peculiar to this child. We, therefore, feel that this case presents a distinct variant of congenital poikiloderma that has not been described previously.
Blistering is not a feature normally associated with mycosis fungoides (MF). We present a case of MF in which histopathological vesicle formation was such a prominent feature that diagnosis was delayed. The patient's disease ran an aggressive course and death occurred within one year of presentation. Tumour involvement of the tongue with MF was an unusual late feature.
A 10-year-old Japanese male with multiple blister formation and palpable purpura in the course of anaphylactoid purpura is described. Histologically, the lesions showed leukocytoclastic vasculitis in the upper dermis with subepidermal clefts. Blister fluid showed matrix metalloproteinase (MMP) 2 and MMP-9 gelantinolytic activities using zymography. These enzymatic reactions, especially that involving MMP-9 derived from polymorphonuclear leukocytes, might play an important role in the pathophysiology of this condition.