PMC8580718_01
Multiple flesh coloured plaques on the (a): lower trunk thighs and (b): vulva.
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Physical examination revealed abdominal distention with positive fluid thrill, multiple skin coloured nodules with background erythema on the lower abdomen, left thigh and labia majora and erythematous nodules with central hyperpigmentation and scales on the lower abdomen (Figure 1a and b).
Dermoscopy showing multiple curvilinear erythematous lines, barely perceptible polymorphic vessels, whitish streaks, distorted pigment network and yellowish white scales.
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Dermoscopy showed multiple curvilinear erythematous areas, barely perceptible polymorphic vessels, whitish streaks, distorted pigment network, and fine white scales (Figure 2a-c).
Histopathology (H&E) shows discohesive nests and sheets of malignant small round blue cells having hyperchromatic to vesicular nuclei, prominent nucleoli and scant to moderate pale eosinophilic cytoplasm.
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Histopathology of skin tissue obtained from the Right labia majora showed dermis infiltrated by sheet of hyperchromatic tumour cell in a prominent myxoid stroma (Figure 3a (x100)) and typical strap cells with eosinophilic cytoplasm (thin arrows) (Figure 3b (x400)).
(a and b): (x200) The strap cells positive for desmin. (a): The typical strap cells (arrows). (b): Perivascular cuffing (arrows) with invasion of the vessel highlighted with the thicker arrow. (c): (x100) S-100 positivity only in the nerve twigs. (d): (x100) AE1/AE3 positivity in the overlying epithelium which serves as internal positive control and positivity in the prominent vascular channels in the section.
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Immunohistochemistry of the lesional tumour cells was positive for Desmin, illustrating the myogenic origin of the strap cells and negative to cytokeratin and S100 (Figure 4a-d).